Human Genetics - 1996

469 articles | Last updated: 2025-12-03 14:12:56
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Interstitial deletion of chromosome 6q: Precise definition of the breakpoints by microdissection, DNA amplification, and reverse painting
Extreme variant of the short arm of chromosome 15
Allelic association between a Ser-9-Gly polymorphism in the dopamine D3 receptor gene and schizophrenia
Localization of the humanRGR opsin gene to chromosome 10q23
Novel interleukin-1 receptor antagonist exon polymorphisms and their use in allele-specific mRNA assessment
Hereditary ceruloplasmin deficiency with hemosiderosis
Extensive polymorphism of ABO blood group gene: three major lineages of the alleles for the common ABO phenotypes
Localization of the human vascular endothelial growth factor gene,VEGF, at Chromosome 6p12
Evidence of genetic heterogeneity of Leber's congenital amaurosis (LCA) and mapping of LCA1 to chromosome 17p13
Familial double pericentric inversion of chromosome 5 with some features of cri-du-chat syndrome
A PCR-based test suitable for screening for fragile X syndrome among mentally retarded males
Allele-specific quantification of TNFA transcripts in rheumatoid arthritis
Hb Costa Rica or α2β277(EF1)His→Arg: The first example of a somatic cell mutation in a globin gene
Announcements
Genetic mapping studies of 40 loci and 23 cosmids in chromosome 11p13-11g13, and exclusion of μ-calpain as the multiple endocrine neoplasia type 1 gene
EagI andNotI linking clones from human chromosomes 11 and Xp
Accelerated loss of telomeric repeats may not explain accelerated replicative decline of Werner syndrome cells
Distribution and frequency of a polymorphicAlu insertion at the plasminogen activator locus in humans
Isolation of the human chromosome 22q telomere and its application to detection of cryptic chromosomal abnormalities
Routine screening for microdeletions by FISH in 77 patients suspected of having Prader-Willi or Angelman syndromes using YAC clone 273A2 (D15S10)
Six new polymorphic microsatellite markers used for the integration of genetic and physical maps of human chromosome 7
H-Y antigens
A novel 5′-upstream mutation in the factor X11 gene is associated with a Taql restriction site in an Alu repeat in factor X11-deficient patients
A family segregating a Friedreich ataxia phenotype that is not linked to the FRDA locus
Geographic distribution and origin of CFTR mutations in Germany
The effect of Y-chromosome alpha-satellite array length on the rate of sex chromosome disomy in human sperm
Allelic loss of the short arm of chromosome 4 in neuroblastoma suggests a novel tumour suppressor gene locus
Detection of a germline mutation and somatic homozygous loss of the von Hippel-Lindau tumor-suppressor gene in a family with a de novo mutation
Characterization of the human p57KIP2 gene: Alternative splicing, insertion/deletion polymorphisms in VNTR sequences in the coding region, and mutational analysis
Reevaluation of the exact CAG repeat length in hereditary cerebellar ataxias using highly denaturing conditions and long PCR
Duplication of thePMP22 gene in 17p partial trisomy patients with Charcot-Marie-Tooth type-1A neuropathy
A novel sequence polymorphism in the promoter region of the human B2-bradykinin receptor gene
New allele variants of the immunoglobulin switch (Sα) regions
A newXmnI polymorphism in the regulatory region of the corticotropin releasing hormone gene
Erratum
A novel point mutation in congenital erythropoietic porphyria in two members of Japanese family Japanese
Assignment of the human ST2 gene to chromosome 2 at q11.2
Tetrasomy 18p de novo: Identification by FISH with conventional and microdissection probes and analysis of parental origin and formation by short sequence repeat typing
Differential underacetylation of histones H2A, H3 and H4 on the inactive X chromosome in human female cells
Attenuated familial adenomatous polyposis due to a mutation in the 3′ part of the APC gene. A clue for understanding the function of the APC protein
The human lanosterol synthase gene maps to chromosome 21q22.3
Analysis of the neurofibromatosis type 2 gene in different human tumors of neuroectodermal origin
A novel emerin mutation in a Japanese patient with Emery-Dreifuss muscular dystrophy
Malignant familial hypertrophic cardiomyopathy in a family with a 453Arg→Cys mutation in the β-myosin heavy chain gene: Coexistence of sudden death and end-stage heart failure
Genotypes with the apolipoprotein ε4 allele are predictors of coronary heart disease mortality in a longitudinal study of elderly Finnish men Finnish men
A new point mutation affecting the fourth transmembrane domain of PMP22 results in severe, de novo Charcot-Marie-Tooth disease
Generation of sequence-tagged sites from Xp22.3 by isolating commonAlu-PCR products of radiation hybrids retaining overlapping human X chromosome fragments
A novel polymorphism in the coding region of CYBB, the human gp91-phox gene
Cartographic study: Breakpoints in 1574 families carrying human reciprocal translocations
Mapping of the variegate porphyria (VP) gene: Contradictory evidence for linkage between VP and microsatellite markers at chromosome 14q32
SRY-negative true hermaphrodites and an XX male in two generations of the same family
Systematic screening for mutations in the human serotonin-2A (5-HT2A) receptor gene: Identification of two naturally occurring receptor variants and association analysis in schizophrenia
Screening for mutations in the neurofibromatosis type 2 (NF2) gene in sporadic meningiomas
Distribution of mosaicism in human placentae
Fanconi anaemia in Italy: High prevalence of complementation group A in two geographic clusters
An additional allelic variant of the CYP2D6 gene causing impaired metabolism of sparteine
Genetic deficiencies of the glycogen phosphorylase system
Coarctation of the aorta and renal hypoplasia in a boy with Turner/Noonan surface anomalies and a 46,XY karyotype: A clinical model for the possible impairment of a putative lymphogenic gene(s) for Tu
Human chromosome 1 localization of the gene for a prostaglandin F2α, receptor negative regulatory protein
Autosomal dominant cerebellar ataxia type I in Martinique (French West Indies): Genetic analysis of three unrelated SCA2 families
Canavan Foundation Postdoctoral Fellowship
The contribution of polymorphism in the alcohol dehydrogenase β subunit to alcohol sensitivity in a Japanese population
Polymorphisms of ethanol-oxidizing enzymes in alcoholics with inactive ALDH2
Identification and characterization of three genes and two pseudogenes on chromosome 13
Allelic association of microsatellites of 6p in Italian hemochromatosis patients
Scanning the first part of the neurofibromatosis type 1 gene by RNA-SSCP: Identification of three novel mutations and of two new polymorphisms
Abnormal gonadal differentiation in two subjects with ambiguous genitalia, Mullerian structures, and normally developed testes: Evidence for a defect in gonadal ridge development
Proportion of the GSTM 10/0 genotype in some Slavic populations and its correlation with cystic fibrosis and some multifactorial diseases Slavic populations
A novel postzygotic nonsense mutation in SRY in familial XY gonadal dysgenesis
Congenital alacrima in a patient with G (Opitz Frias) syndrome
A polymorphism but no mutations in the GADD45 gene in breast cancers
Familial non-syndromic conotruncal defects are not associated with a 22q11 microdeletion
Trisomy of human chromosome 18: Molecular studies on parental origin and cell stage of nondisjunction
Psychosis and genes with trinucleotide repeat polymorphism
Diagnosis of DiGeorge syndrome in nuclei released from archival autoptic heart specimens using fluorescence in situ hybridization
Genetic analysis of twenty-two patients with Cockayne syndrome
A point mutation in exon 3 (His 107→Tyr) in two unrelated Japanese patients with carbonic anhydrase II deficiency with central nervous system involvement Japanese
SSCP analysis of the tyrosine kinase domain of the insulin receptor gene: Polymorphisms detected in South African black and white subjects South African black
An unusual dicentric Y chromosome with a functional centromere with no detectable alpha-satellite
Identification of three new microsatellite markers in the spinocerebellar ataxia type 2 (SCA2) region and 1.2 Mb physical map
Novel trinucleotide deletion in Fabry's disease
Activation status of the X chromosome in human micronucleated lymphocytes
Acid alpha-glucosidase deficiency: Identification and expression of a missense mutation (S529V) in a Japanese adult phenotype
Recurrent and unexpected segregation of the FMR1 CGG repeat in a family with fragile X syndrome
Ablepharon macrostomia syndrome with associated cutis laxa: Possible localization to 18q
Identification of a novel mutation in CFTR gene exon 8 (L375F) in a CUAVD phenotype
Announcements
Association of CTG repeats and the 1-kbAlu insertion/deletion polymorphism at the myotonin protein kinase gene in the Japanese population suggests a common Eurasian origin of the myotonic dystrophy mu Japanese population; Eurasian origin
Sex difference in the regulation of plasma high density lipoprotein cholesterol by genetic and environmental factors
Translocation breakpoints in three patients with campomelic dysplasia and autosomal sex reversal map more than 130 kb from SOX9
Identification of four novel splice site mutations in the ornithine transcarbamylase gene
CpG dinucleotides in the hMSH2 and hMLHI genes are hotspots for HNPCC mutations
The murine homolog of the human breast and ovarian cancer susceptibility geneBrca1 maps to mouse chromosome 11D
Population study of the G1691A mutation (R506% FV Leiden) in the human factor V gene that is associated with resistance to activated protein C
Mutations of theBtk gene in 12 unrelated families with X-linked agammaglobulinemia in Japan
Mapping the locus for familial hypertrophic cardiomyopathy to chromosome 11 in a family with a case of apical hypertrophic cardiomyopathy of the Japanese type Japanese
A novel (TA)n polymorphism in the hexokinase II gene: Application to noninsulin-dependent diabetes mellitus in the Pima Indians
A solitary human H3 histone gene on chromosome 1
No evidence for ‘skewed’ inactivation of the X-chromosome as cause of Leber's hereditary optic neuropathy in female carriers
Alternative splicing of dystrophin exon 4 in normal human muscle
The enigma of common fragile sites
Genomic structure and PCR-SSCP analysis of the human CD40 ligand gene: Its application to prenatal screening for X-linked hyper-IgM syndrome
A missense mutation in the NF2 gene results in moderate and mild clinical phenotypes of neurofibromatosis type 2
Expression and regulation of the dystrophin Purkinje promoter in human skeletal muscle, heart, and brain
HbF-Lesvos: An HbF variant due to a novel Gγ mutation (:Gγ 75 ATA→ACA) detected in a Greek family Greek family
A common region of loss of heterozygosity in Wilms' tumor and embryonal rhabdomyosarcoma distal to the D11S988 locus on chromosome 11p15.5
Increased incidence of hyperhaploid 24,XY spermatozoa detected by three-colour FISH in a 46,XY/47,XXY male
Severe congenital limb deficiencies, vertebral hypersegmentation, absent thymus and mirror polydactyly: A defect expression of a developmental control gene?
Clonality of tuberous sclerosis harmatomas shown by non-random X-chromosome inactivation
X chromosome inactivation in 30 girls with Rett syndrome: Analysis using the probe
A missense mutation (Thr-6Pro) in the lysosomal acid lipase (LAL) gene is present with a high frequency in three different ethnic populations: Impact on serum lipoprotein concentrations ethnic populations
Programs, databases, and expert systems for human geneticists — a survey
Identification of mutations in the ALD-gene of 20 families with adrenoleukodystrophy/adrenomyeloneuropathy
Familial adenomatous polyposis: A submicroscopic deletion at the APC locus in a family with mentally normal patients
Arylsulfatase A pseudodeficiency in Chinese Chinese
Non-invasive early prenatal molecular diagnosis using retrieved transcervical trophoblast cells
Cockayne syndrome complementation group B associated with xeroderma pigmentosum phenotype
Mutations in the iduronate-2-sulfatase gene in five Norwegians with Hunter syndrome
A new highly polymorphic marker in the 5' untranslated region of HLA-F shows strong allelic association with haemochromatosis
Screening for known mutations in the LDL receptor gene causing familial hypercholesterolemia
Fatal mitochondrial myopathy, lactic acidosis, and complex I deficiency associated with a heteroplasmic A→G mutation at position 3251 in the mitochondrial tRNALeu(UUR) gene
Genetic epidemiology of congenital muscular dystrophy in a sample from north-east Italy
Deletions of the long arm of chromosome 10 in progression of follicular thyroid tumors
Geographic differences in the allele frequencies of the human Y-linked tetranucleotide polymorphism DYS19
Deletions in the SMN gene in infantile and adult spinal muscular atrophy patients from the same family
Validation of the determination of ΔF508 mutations of the cystic fibrosis gene in over 11000 mouthwashes
A (G-to-A) mutation in the initiation codon of the proteolipid protein gene causing a relatively mild form of Pelizaeus-Merzbacher disease in a Dutch family Dutch family
Characterisation of a human serine hydroxymethyltransferase pseudogene and its localisation to 1p32.3–33
A new base substitution in the 5′ regulatory region of the humanAγ globin gene is linked with the βs gene
No mutations found byRET mutation scanning in sporadic and hereditary neuroblastoma
Interleukin-1 receptor antagonist allele (ILIRN*2) associated with nephropathy in diabetes mellitus
A novel mechanism for the origin of supernumerary marker chromosomes
Localization of the gene encoding the human L-glutamate transporter (GLT-1) to 11p11.2–p13 by fluorescence in situ hybridization
A novel missense mutation in the thyroid hormone receptor β gene in a kindred with resistance to thyroid hormone
Fanconi anaemia complementation groups in Germany and The Netherlands
Molecular studies of the aetiology of trisomy 8 in spontaneous abortions and the liveborn population
Angelman syndrome in an inbred family
Two different mutations are responsible for Krabbe disease in the Druze and Moslem Arab populations in Israel
Cystic fibrosis in Argentina: The frequency of the ΔF508 mutation
Substitution of glycine-661 by serine in the α1(I) and α2(1) chains of type I collagen results in different clinical and biochemical phenotypes
The gene for X-linked hypophosphataemic rickets maps to a 200–300 kb region in Xp22.1, and is located on a single YAC containing a putative vitamin D response element (VDRE)
Objective analysis of centromere separation
Relative frequency of mutations causing ornithine transcarbamylase deficiency in 78 families
Deletion of a Gly-Pro-Pro repeat in the proα2(I) chain of procollagen I in a family with dominant osteogenesis imperfecta type IV
Inheritance of the S113L mutation within an inbred family with carnitine palmitoyltransferase enzyme deficiency
The heritability of human longevity: A population-based study of 2872 Danish twin pairs born 1870–1900
X-chromosome methylation ratios as indicators of chromosomal activity: Evidence of intraindividual divergencies among tissues of different embryonal origin
A deletion of five nucleotides in the LICAM gene in a Japanese family with X-linked hydrocephalus Japanese
Chromosomal localization of the human and mouse histidine decarboxylase genes by in situ hybridization. Exclusion of the HDC gene from the Prader-Willi syndrome region
Most cases of medium-chain acyl-CoA dehydrogenase deficiency escape detection in France
An analysis of Xq deletions
High resolution multicolor fluorescence in situ hybridization using cyanine and fluorescein dyes: Rapid chromosome identification by directly fluorescently labeled alphoid DNA probes
Integration of four genes, a pseudogene, thirty-one STSs, and a highly polymorphic STRP into the 7–10 Mb YAC contig of 5q34–q35
Localization and ordering of acid alpha-glucosidase (GAA) and thymidine kinase (TK1) by fluorescence in situ hybridization
Construction of a YAC contig and an STS map spanning 3.6 megabase pairs in Xp22.1
Molecular re-investigation of patients with Huntington's disease in Wessex reveals a family with dentatorubral and pallidoluysian atrophy
Towards fully automated genotyping: use of an X linked recessive spastic paraplegia family to test alternative analysis methods
Telomere elongation observed in immortalized human fibroblasts by treatment with 60Co gamma rays or 4-nitroquinoline 1-oxide
Congenital bilateral absence of the vas deferens (CBAVD) and cystic fibrosis transmembrane regulator (CFTR): correlation between genotype and phenotype
Mutation analysis of the RET proto-oncogene in Dutch families with MEN 2A, MEN 2B and FMTC: two novel mutations and one de novo mutation for MEN 2A
Direct detection of disomy in human sperm by the PRINS technique
Molecular genetic analysis of the 17p11.2 region in patients with hereditary neuropathy with liability to pressure palsies (HNPP)
A novel mutation in exon 17 of the ?-subunit of rod phosphodiesterase in two RP sisters of a consanguineous family
Ullrich-Turner syndrome is not caused by haploinsufficiency of RPS4X
Identification of three novel mutations in the PIG-A gene in paroxysmal nocturnal haemoglobinuria (PNH) patients
Mean corpuscular hemoglobin is not increased in Fmr1 knockout mice
Hereditary tyrosinemia type 1: novel missense, nonsense and splice consensus mutations in the human fumarylacetoacetate hydrolase gene; variability of the genotype-phenotype relationship
A clinical and molecular study of mosaicism for trisomy 17
Pseudohypoparathyroidism type Ia: two new heterozygous frameshift mutations in exons 5 and 10 of the Gs? gene
A novel site of DNA amplification on chromosome 1p32-33 in a rhabdomyosarcoma revealed by comparative genomic hybridization
Mutational analysis of the human cyclin-dependent kinase inhibitor p27kip1 in primary breast carcinomas
Precise chromosomal locations of the genes for dentatorubral-pallidoluysian atrophy (DRPLA), von Willebrand factor (F8vWF) and parathyroid hormone-like hormone (PTHLH) in human chromosome 12p by delet
Apparent higher frequency of phenylketonuria in the Mexican state of Jalisco
Linkage disequilibrium and extended haplotypes in the HLA-A to D6S105 region: implications for mapping the hemochromatosis gene (HFE)
Characterisation of a short interspersed repeat (Mermaid) that has family members on human chromosome 21 and elsewhere in the human genome
Identification of three neurofibromatosis type 2 (NF2) gene mutations in vestibular schwannomas
Editorial help
Announcements
DMA polymorphisms in adhesion molecule genes ? a new risk factor for early atherosclerosis
Xp-duplications with and without sex reversal
Isolation and characterization of the human mismatch repair gene hMSH2 promoter region
A highly polymorphic CA/GT repeat in intron 3 of the human urokinase receptor gene (PLAUR)
Cloning of contiguous genomic fragments from human chromosome 21 harbouring three trefoil peptide genes
Characterisation of a human serine hydroxymethyltransferase pseudogene and its localisation to 1p32.3-33
A deletion of five nucleotides in the L1CAM gene in a Japanese family with X-linked hydrocephalus Japanese family
Localization and ordering of acid alpha-glucosidase (GAA) and thymidine kinase (TK1) by fluorescence in situ hybridization
Scanning the first part of the neurofibromatosis type 1 gene by RNA-SSCP: identification of three novel mutations and of two new polymorphisms
Coarctation of the aorta and renal hypoplasia in a boy with Turner/Noonan surface anomalies and a 46,XY karyotype: a clinical model for the possible impairment of a putative lymphogenic gene(s) for Tu
Attenuated familial adenomatous polyposis due to a mutation in the 3′ part of the APC gene. A clue for understanding the function of the APC protein
Cartographic study: breakpoints in 1574 families carrying human reciprocal translocations
Genotypes with the apolipoprotein Â4 allele are predictors of coronary heart disease mortality in a longitudinal study of elderly Finnish men
A new point mutation affecting the fourth transmembrane domain of PMP22 results in severe, de novo Charcot-Marie-Tooth disease
A novel emerin mutation in a Japanese patient with Emery-Dreifuss muscular dystrophy
Eag I and Not I linking clones from human chromosomes 11 and Xp
Accelerated loss of telomeric repeats may not explain accelerated replicative decline of Werner syndrome cells
Familial double pericentric inversion of chromosome 5 with some features of cri-du-chat syndrome
Allele-specific quantification of TNFA transcripts in rheumatoid arthritis
The effect of Y-chromosome alpha-satellite array length on the rate of sex chromosome disomy in human sperm
A novel 5′-upstream mutation in the factor XII gene is associated with a Taq I restriction site in an Alu repeat in factor XII-deficient patients
Detection of a de novo R1066H mutation in an Italian patient affected by cystic fibrosis
Physical linkage and orientation of the human complement C8α and C8β genes on chromosome 1p32
The apolipoprotein B Q3405E polymorphism has no effect on its low-density-lipoprotein receptor binding affinity
The high mobility group I-C gene (HMGI-C): polymorphism and genetic localization
The heritability of human longevity: a population-based study of 2872 Danish twin pairs born 1870-1900
Validation of the determination of ΔF508 mutations of the cystic fibrosis gene in over 11000 mouthwashes
High resolution multicolor fluorescence in situ hybridization using cyanine and fluorescein dyes: rapid chromosome identification by directly fluorescently labeled alphoid DNA probes
Generation of sequence-tagged sites from Xp22.3 by isolating common Alu -PCR products of radiation hybrids retaining overlapping human X chromosome fragments
Characterization of the human p57 KIP2 gene: alternative splicing, insertion/deletion polymorphisms in VNTR sequences in the coding region, and mutational analysis
An additional allelic variant of the CYP2D6 gene causing impaired metabolism of sparteine
New allele variants of the immunoglobulin switch (Sα) regions
A new Xmn I polymorphism in the regulatory region of the corticotropin releasing hormone gene
The human mitochondrial citrate transporter gene (SLC20A3) maps to chromosome band 22q11 within a region implicated in DiGeorge syndrome, velo-cardio-facial syndrome and schizophrenia
Multiple products in the protein truncation test due to alternative splicing in the adenomatous polyposis coli (APC) gene
Point mutations in Italian patients with classic, non-classic, and cryptic forms of steroid 21-hydroxylase deficiency
Exclusion of the phosphoinositide-specific phospholipase C?3 ( PLCB3 ) gene as a candidate for multiple endocrine neoplasia type 1
Human chromosome 1 localization of the gene for a prostaglandin F 2α receptor negative regulatory protein
X-linked myotubular myopathy: refinement of the gene to a 280-kb region with new and highly informative microsatellite markers
Polymorphisms of apolipoproteins A-IV and E in a Turkish population living in Germany
Fluorescence-assisted mismatch analysis (FAMA) for exhaustive screening of the α-galactosidase A gene and detection of carriers in Fabry disease
Two novel gene mutations (Glu174→Lys, Phe383→Tyr) causing the "hepatic" form of carnitine palmitoyltransferase II deficiency
Frequent DNA variant in exon 2a of the survival motor neuron gene (SMN): a further possibility for distinguishing the two copies of the gene
A novel mechanism for the origin of supernumerary marker chromosomes
Genetic analysis of twenty-two patients with Cockayne syndrome
SSCP analysis of the tyrosine kinase domain of the insulin receptor gene: polymorphisms detected in South African black and white subjects South African black and white subjects
Mapping the locus for familial hypertrophic cardiomyopathy to chromosome 11 in a family with a case of apical hypertrophic cardiomyopathy of the Japanese type
Allelic association of microsatellites of 6p in Italian hemochromatosis patients
Abnormal gonadal differentiation in two subjects with ambiguous genitalia, Mullerian structures, and normally developed testes: evidence for a defect in gonadal ridge development
Complex mutational events at the HumD21S11 locus
The G1691A mutation of the coagulation factor V gene (factor V Leiden) is rare in Chinese: an analysis of 618 individuals Chinese
Mutations in the VHL tumor suppressor gene and associated lesions in families with von Hippel-Lindau disease from central Europe
Fragile site and interstitial telomere repeat sequences at the fusion point of a de novo (Y;13) translocation
Evidence of somatic mutations in osteoarthritis
Genetic deficiencies of the glycogen phosphorylase system
Screening for mutations in the neurofibromatosis type 2 (NF2) gene in sporadic meningiomas
Analysis of the neurofibromatosis type 2 gene in different human tumors of neuroectodermal origin
Autosomal dominant cerebellar ataxia type I in Martinique (French West Indies): genetic analysis of three unrelated SCA2 families
Interstitial deletion of chromosome 6q: precise definition of the breakpoints by microdissection, DNA amplification, and reverse painting
Extensive polymorphism of ABO blood group gene: three major lineages of the alleles for the common ABO phenotypes
Six new polymorphic microsatellite markers used for the integration of genetic and physical maps of human chromosome 7
Fine mapping of the 1q21 breakpoint of the papillary renal cell carcinoma-associated (X;1) translocation
The gene encoding the p60 subunit of chromatin assembly factor I ( CAF1P60 ) maps to human chromosome 21q22.2, a region associated with some of the major features of Down syndrome
Assignment of the NTRK4 (trkE) gene to chromosome 6p21
Beta-thalassaemia in indigenous Belgian families: identification of a novel mutation
Detection of a novel nonsense mutation and an intragenic polymorphism in the PKD1 gene of a Cypriot family with autosomal dominant polycystic kidney disease Cypriot
Hypertriglyceridemia and the apolipoprotein CIII gene locus: lack of association with the variant insulin response element in Italian school children
Isolation and characterization of the full-length 3′ untranslated region of the human von Hippel-Lindau tumor suppressor gene
Refinement of the dominant optic atrophy locus (OPA1) to a 1.4-cM interval on chromosome 3q28-3q29, within a 3-Mb YAC contig
Frequencies of cystic fibrosis mutations in the Maine population: high proportion of unknown alleles in individuals of French-Canadian ancestry French-Canadian ancestry
Revised transthyretin Ile 122 allele frequency in African-Americans
Anesthesiologic problems in Williams syndrome: the CACNL2A locus is not involved
Molecular cytogenetic characterization of breakpoints involving pericentric inversions of human chromosome 9
Molecular genetics of familial hypercholesterolemia in Israel
The origin of the most common mutation of adenine phosphoribosyltransferase among Japanese goes back to a prehistoric era
Absence of association between the Gly 40 →Ser mutation in the human glucagon receptor and Japanese patients with non-insulin-dependent diabetes mellitus or impaired glucose tolerance Japanese
Identification of WASP mutations, mutation hotspots and genotype-phenotype disparities in 24 patients with the Wiskott-Aldrich syndrome
DNA analysis of the immunoglobulin IGHG loci in a Mandenka population from eastern Senegal: correlation with Gm haplotypes and hypotheses for the evolution of the Ig CH region
Wiskott-Aldrich syndrome: no strict genotype-phenotype correlations but clustering of missense mutations in the amino-terminal part of the WASP gene product
Mutational and functional analysis of the neurofibromatosis type 1 ( NF1 ) gene
Turner syndrome: a study of chromosomal mosaicism
Phenotypic variability in two families with novel splice-site and frameshift NF2 mutations
Genetic basis of Creutzfeldt-Jakob disease in the United Kingdom: a systematic analysis of predisposing mutations and allelic variation in the PRNP gene
Prevalence of α-thalassemias in northern Thailand
Molecular analysis of the cystinuria disease gene: identification of four new mutations, one large deletion, and one polymorphism
Analysis of the Bgl I restriction fragment length polymorphism in the human factor VIII gene using "virtual PCR" - a novel approach employing the polymerase chain reaction in the absence of sequence i
Recessive Schwartz-Jampel syndrome (SJS): confirmation of linkage to chromosome 1p, evidence of genetic homogeneity and reduction of the SJS locus to a 3-cM interval
Polymorphisms in the human apolipoprotein-J/clusterin gene: ethnic variation and distribution in Alzheimer's disease ethnic variation
Hin dIII DNA polymorphism in the lipoprotein lipase gene and plasma lipid phenotypes and carotid artery atherosclerosis
Transglutaminase activity is related to CAG repeat length in patients with Huntington's disease
Evidence for an association between markers on chromosome 19q and non-syndromic cleft lip with or without cleft palate in two groups of multiplex families
Mutations in the gene encoding 21-hydroxylase detected by solid-phase minisequencing
Identification of a common 6-pyruvoyl-tetrahydropterin synthase mutation at codon 87 in Chinese phenylketonuria caused by tetrahydrobiopterin synthesis deficiency
Microsatellite variation and the differentiation of modern humans
An asymptomatic germline missense base substitution in the hypoxanthine phosphoribosyltransferase (HPRT) gene that reduces the amount of enzyme in humans
Mutational spectrum in the neurofibromatosis type 2 gene in sporadic and familial schwannomas
Human desmin gene: cDNA sequence, regional localization and exclusion of the locus in a familial desmin-related myopathy
Age-dependent penetrance and mapping of the locus for juvenile and early-onset open-angle glaucoma on chromosome 1q (GLC1A) in a French family
Human Gene Mutation Database
Molecular studies in three patients with isodicentric Y chromosome
PCR-based detection of mosaicism in Turner syndrome patients
The contribution of polymorphism in the alcohol dehydrogenase β subunit to alcohol sensitivity in a Japanese population
A point mutation in exon 3 (His 107→Tyr) in two unrelated Japanese patients with carbonic anhydrase II deficiency with central nervous system involvement
Novel interleukin-1 receptor antagonist exon polymorphisms and their use in allele-specific mRNA assessment
Routine screening for microdeletions by FISH in 77 patients suspected of having Prader-Willi or Angelman syndromes using YAC clone 273A2 (D15S10)
Identification and characterization of NF1 -related loci on human chromosomes 22, 14 and 2
Geographic homogeneity and non-equilibrium patterns of mtDNA sequences in Tuscany, Italy
Localization of the gene encoding the human L-glutamate transporter (GLT-1) to 11p11.2-p13 by fluorescence in situ hybridization
Integration of four genes, a pseudogene, thirty-one STSs, and a highly polymorphic STRP into the 7-10 Mb YAC contig of 5q34-q35
Identification and characterization of three genes and two pseudogenes on chromosome 13
No evidence for 'skewed' inactivation of the X-chromosome as cause of Leber's hereditary optic neuropathy in female carriers
Assignment of the human ST2 gene to chromosome 2 at q11.2
Reevaluation of the exact CAG repeat length in hereditary cerebellar ataxias using highly denaturing conditions and long PCR
The human lanosterol synthase gene maps to chromosome 21q22.3
A novel sequence polymorphism in the promoter region of the human B2-bradykinin receptor gene
Localization of the human RGR opsin gene to chromosome 10q23
Genetic mapping studies of 40 loci and 23 cosmids in chromosome 11p13-11q13, and exclusion of Ì-calpain as the multiple endocrine neoplasia type 1 gene
Hereditary ceruloplasmin deficiency with hemosiderosis
Distribution and frequency of a polymorphic Alu insertion at the plasminogen activator locus in humans
Localization of the human vascular endothelial growth factor gene, VEGF , at Chromosome 6p12
A family segregating a Friedreich ataxia phenotype that is not linked to the FRDA locus
Wilson disease mutations associated with uncommon haplotypes in mediterranean patients
Relationship of (CAG)nC configuration to repeat instability of the Machado-Joseph disease gene
A molecular anatomical analysis of mosaic trisomy 16
CFTR haplotypic variability for normal and mutant genes in cystic fibrosis families from southern France
Identification of NF2 germ-line mutations and comparison with neurofibromatosis 2 phenotypes
Latent chromosomal instability in cancer patients
Interleukin-1 receptor antagonist allele (IL1RN*2) associated with nephropathy in diabetes mellitus
Untangling genetic influences on smoking, body mass index and longevity: a multivariate study of 2464 Danish twins followed for 28 years Danish
Short direct repeats at the breakpoints of a novel large deletion in the CFTR gene suggest a likely slipped mispairing mechanism
Are men carrying the apolipoprotein ε4- or ε2 allele less fertile than ε3ε3 genotypes?
The apolipoprotein E polymorphism in Greenland Inuit in its global perspective Greenland Inuit
Sporadic cardiac myxomas and tumors from patients with Carney complex are not associated with activating mutations of the Gsα gene
Possible genetic heterogeneity in the Saethre-Chotzen syndrome
Substitution of glycine-661 by serine in the α1(I) and α2(I) chains of type I collagen results in different clinical and biochemical phenotypes
Most cases of medium-chain acyl-CoA dehydrogenase deficiency escape detection in France
Polymorphisms of ethanol-oxidizing enzymes in alcoholics with inactive ALDH2
A novel (TA) n polymorphism in the hexokinase II gene: application to noninsulin-dependent diabetes mellitus in the Pima Indians Pima Indians
A solitary human H3 histone gene on chromosome 1
Proportion of the GSTM1 0/0 genotype in some Slavic populations and its correlation with cystic fibrosis and some multifactorial diseases Slavic populations
The enigma of common fragile sites
Small increase in triplet repeat length of cerebellum from patients with myotonic dystrophy
A jumping Robertsonian translocation: a molecular and cytogenetic study
Characterization of familial partial 10p trisomy by chromosomal microdissection, FISH, and microsatellite dosage analysis
Germline mosaicism in 4q35 facioscapulohumeral muscular dystrophy (FSHD1A) occurring predominantly in oogenesis
Germline mutations in the 3′ part of APC exon 15 do not result in truncated proteins and are associated with attenuated adenomatous polyposis coli
A (G-to-A) mutation in the initiation codon of the proteolipid protein gene causing a relatively mild form of Pelizaeus-Merzbacher disease in a Dutch family
The gene for X-linked hypophosphataemic rickets maps to a 200-300 kb region in Xp22.1, and is located on a single YAC containing a putative vitamin D response element (VDRE)
A new base substitution in the 5′ regulatory region of the human A γ globin gene is linked with the β S gene
No mutations found by RET mutation scanning in sporadic and hereditary neuroblastoma
Objective analysis of centromere separation
An analysis of Xq deletions
A novel missense mutation in the thyroid hormone receptor β gene in a kindred with resistance to thyroid hormone
An unusual dicentric Y chromosome with a functional centromere with no detectable alpha-satellite
Identification of three new microsatellite markers in the spinocerebellar ataxia type 2 (SCA2) region and 1.2 Mb physical map
Novel trinucleotide deletion in Fabry's disease
Ablepharon macrostomia syndrome with associated cutis laxa: possible localization to 18q
Dombrock blood group (DO): assignment to chromosome 12p
Amplified product length polymorphism (APLP): a novel strategy for genotyping the ABO blood group
Linkage analysis of Fanconi anaemia in Italy and mapping of the complementation group A gene
The IGHG3 gene shows a structural polymorphism characterized by different hinge lengths: sequence of a new 2-exon hinge gene
A point mutation in exon 7 of the C1-inhibitor gene causing type I hereditary angioedema
YAC and cosmid FISH mapping of an unbalanced chromosomal translocation causing partial trisomy 21 and Down syndrome
Two new mutations in the sterol 27-hydroxylase gene in two families lead to cerebrotendinous xanthomatosis
Transcript analysis of CFTR frameshift mutations in lymphocytes using the reverse transcription-polymerase chain reaction technique and the protein truncation test
A female compound heterozygote (pre- and full mutation) for the CGG FMR1 expansion
Linkage disequilibrium analysis in Machado-Joseph disease patients of different ethnic origins
Phenotypic and genotypic overlap between atelosteogenesis type 2 and diastrophic dysplasia
X-linked adrenal hypoplasia in a large Greenlandic family. Detection of a missense mutation (N440I) in the DAX-1 gene; implication for genetic counselling and carrier diagnosis Greenlandic
Heteroduplex and protein truncation analysis of the BRCA1 185delAG mutation
Systematic screening for mutations in the human serotonin-2A (5-HT 2A ) receptor gene: identification of two naturally occurring receptor variants and association analysis in schizophrenia
Rapid detection of trisomies 21 and 18 and sexing by quantitative fluorescent multiplex PCR
β-Glucuronidase P408S, P415L mutations: evidence that both mutations combine to produce an MPS VII allele in certain Mexican patients
OB gene not linked to human obesity in Mexican American affected sib pairs from Starr County, Texas
Prevalence of ?F508 cystic fibrosis carriers in The Netherlands: logistic regression on sex, age, region of residence and number of offspring
Localization of the human phosphotyrosine phosphatase-related genes (h-PRL-1) to chromosome bands 1p35-p34, 17q12-q21, 11q24-q25 and 12q24
Association between the PI*M3 allele of α1-antitrypsin and Alzheimer's disease? A preliminary report
Localization of the human HuR gene to chromosome 19p13.2
Sex differences in mutational rate and mutational mechanism in the NF1 gene in neurofibromatosis type 1 patients
Two novel missense and frameshift mutations in exons 5 and 6 of the purine nucleoside phosphorylase (PNP) gene in a severe combined immunodeficiency (SCID) patient
Confirmation of a susceptibility locus on chromosome 13 in Australian breast cancer families
A new polymorphism of arylsulfatase A within the coding region
Hb Costa Rica or · 2 ‚ 2 77(EF1)His→Arg: the first example of a somatic cell mutation in a globin gene
High parental age is associated with sporadic hereditary retinoblastoma: the Dutch retinoblastoma register 1862-1994
Absence of somatic mosaicism in 17 families with hemophilia B: an analysis with a sensitivity 10- to 1000-fold greater than that of sequencing gels
Chromosomal localization of the human and mouse histidine decarboxylase genes by in situ hybridization. Exclusion of the HDC gene from the Prader-Willi syndrome region
Extreme variant of the short arm of chromosome 15
Identification of a variable number tandem repeat region in the human T cell receptor alpha-delta (TCRAD) locus
Novel polymorphisms and haplotypes in the human coagulation factor XIII A-subunit gene
Molecular mapping of a Yq deletion in a patient with normal stature
Twelve amplified and expressed genes localized in a single domain in glioma
Microsatellite polymorphism of the α 1 -antichymotrypsin gene locus associated with sporadic Alzheimer's disease
X-linked dominant Charcot-Marie-Tooth neuropathy (CMTX): new mutations in the connexin32 gene
A new mechanism in blue cone monochromatism
The T705I mutation of the low density lipoprotein receptor gene (FH Paris-9) does not cause familial hypercholesterolemia
Preliminary studies on the molecular basis of hyperphenylalaninemia in Egypt
Possible control of dopamine β-hydroxylase via a codominant mechanism associated with the polymorphic (GT) n repeat at its gene locus in healthy individuals
Mutation screening of 17 Japanese patients with neuropathic Gaucher disease
Microsatellite instability and mutations of p53 and TGF-‚ RII genes in gastric cancer
Molecular evidence for human alpha2-HS glycoprotein (AHSG) polymorphism
Refined localization of the pyruvate dehydrogenase E1α gene (PDHA1) by linkage analysis
Chinese achondroplasia is also defined by recurrent G380R mutations of the fibroblast growth factor receptor-3 gene
Prevalence of carriers of the most common medium-chain acyl-CoA dehydrogenase (MCAD) deficiency mutation (G985A) in The Netherlands
Sequence variations in the Fanconi anaemia gene, FAC : pathogenicity of 1806insA and R548X and recognition of D195V as a polymorphic variant
Refined molecular characterization of the breakpoints in small inv dup(15) chromosomes
Characterization of more than 85% of cystic fibrosis alleles in the Greek population, including five novel mutations
An intron 1 splice mutation and a nonsense mutation (W23X) in CYP21 causing severe congenital adrenal hyperplasia
DNA polymorphisms and linkage disequilibrium in the angiotensinogen gene
Dual localization of the human gene encoding hnRNP I/PTB protein to chromosomes 19p13.3 and 14q23
Complex segregation analysis of non-myoclonic idiopathic generalized epilepsy in families ascertained from probands affected with idiopathic epilepsy with tonic-clonic seizures in Antioquia, Colombia
Screening for human mitochondrial DNA polymorphisms with denaturing gradient gel electrophoresis
Association between common alleles of the low-density lipoprotein receptor gene region and interindividual variation in plasma lipid and apolipoprotein levels in a population-based sample from Rochest
Acid alpha-glucosidase deficiency: identification and expression of a missense mutation (S529V) in a Japanese adult phenotype
Congenital alacrima in a patient with G (Opitz Frias) syndrome
Linkage disequilibrium between the four most common cystic fibrosis mutations and microsatellite haplotypes in the Celtic population of Brittany Celtic population of Brittany
Detection of amplified genomic sequences in human small-cell lung carcinoma cells by arbitrarily primed-PCR genomic fingerprinting
Low frequency of deletion alleles in patients with steroid 21-hydroxylase deficiency in a Mexican population
Two novel mutations in the aquaporin-2 and the vasopressin V2 receptor genes in patients with congenital nephrogenic diabetes insipidus
Detection of a new polymorphism in the plasma-membrane Ca 2+ ATPase isoform-3 gene and its exclusion as a candidate for X-linked myotubular myopathy (MTM1)
Detection of a germline mutation and somatic homozygous loss of the von Hippel-Lindau tumor-suppressor gene in a family with a de novo mutation
Analysis of chromosomal equipment in spermatozoa of a 46,XY/47,XY/+8 male by means of multicolour fluorescent in situ hybridization: confirmation of a mosaicism and evaluation of risk for offspring
The world-wide distribution of allele frequencies at the human dopamine D4 receptor locus
Identification of a Ser857-Asn857 substitution in DRK1 (KCNB1), population frequencies and lack of association to the low voltage alpha EEG trait
Analysis of sex and ΔF508 in single amniocytes using primer extension preamplification
Yeast artificial chromosome mapping of the cystinosis locus on chromosome 17p by fluorescence in situ hybridization
Autosomal dominant spastic paraplegia with anticipation maps to a 4-cM interval on chromosome 2p21-p24 in a large German family
Localization of the gene (or genes) for a syndrome with X-linked mental retardation, ataxia, weakness, hearing impairment, loss of vision and a fatal course in early childhood
Characterization of a cytogenetic 17q11.2 deletion in an NF1 patient with a contiguous gene syndrome
Sequence-specific and length-dependent interaction of C 2 H 2 zinc fingers and (TA) n microsatellites
Cloning of the cDNA for a human homolog of the rat PEP-19 gene and mapping to chromosome 21q22.2-q22.3
Molecular basis of variegate porphyria: a de novo insertion mutation in the protoporphyrinogen oxidase gene
Superoxide dismutase 1: Identification of a novel mutation in a case of familial amyotrophic lateral sclerosis
A detailed investigation of two cases exhibiting characteristics of the 6p deletion syndrome
Simultaneous detection of structural and numerical chromosome abnormalities in sperm of healthy men by multicolor fluorescence in situ hybridization
X-chromosome methylation ratios as indicators of chromosomal activity: evidence of intraindividual divergencies among tissues of different embryonal origin
Diagnosis of DiGeorge syndrome in nuclei released from archival autoptic heart specimens using fluorescence in situ hybridization
Recurrent and unexpected segregation of the FMR1 CGG repeat in a family with fragile X syndrome
Alternative splicing of dystrophin exon 4 in normal human muscle
A novel postzygotic nonsense mutation in SRY in familial XY gonadal dysgenesis
A polymorphism but no mutations in the GADD45 gene in breast cancers
Identification of a novel mutation in CFTR gene exon 8 (L375F) in a CUAVD phenotype
A pericentric inversion of chromosome six in a patient with Peutz-Jeghers' syndrome and the use of FISH to localise the breakpoints on a genetic map
Stability of triplet repeats of myotonic dystrophy and fragile X loci in human mutator mismatch repair cell lines
Mutation analysis reveals an insertional hotspot in exon 4 of the LDL receptor gene
Investigation of deletions at 7q11.23 in 44 patients referred for Williams-Beuren syndrome, using FISH and four DNA polymorphisms
A novel point mutation in congenital erythropoietic porphyria in two members of Japanese family
Differential underacetylation of histones H2A, H3 and H4 on the inactive X chromosome in human female cells
Malignant familial hypertrophic cardiomyopathy in a family with a 453Arg→Cys mutation in the ‚-myosin heavy chain gene: coexistence of sudden death and end-stage heart failure
Fanconi anaemia in Italy: high prevalence of complementation group A in two geographic clusters
A novel polymorphism in the coding region of CYBB, the human gp91- phox gene
H-Y antigens
Allelic association between a Ser-9-Gly polymorphism in the dopamine D3 receptor gene and schizophrenia
Geographic distribution and origin of CFTR mutations in Germany
Allelic loss of the short arm of chromosome 4 in neuroblastoma suggests a novel tumour suppressor gene locus
Mosaicism of a microdeletion of 486 bp involving the CGG repeat of the FMR1 gene due to misalignment of GTT tandem repeats at chi-like elements flanking both breakpoints and a full mutation
Linkage of type I diabetes to 15q26 (IDDM3) in the Danish population Danish population
Fetal cells in maternal blood: recovery by charge flow separation
Antenatal screening and fetal diagnosis of β-thalassemia in a Chinese population: prevalence of the β-thalassemia trait in the Guangzhou area of China Chinese population; Guangzhou area of China
Genetic mapping of RP1 on 8q11-q21 in an Australian family with autosomal dominant retinitis pigmentosa reduces the critical region to 4 cM between D8S601 and D8S285
Is the mismatch repair deficient type of Muir-Torre syndrome confined to mutations in the hMSH2 gene?
Deletion analysis of the SMN and NAIP genes in Kuwaiti patients with spinal muscular atrophy
Exclusion of the MSX1 homeobox gene as the gene for the Ellis van Creveld syndrome in the Amish
Estimation of the prevalence of familial hypercholesterolaemia in a rural Afrikaner community by direct screening for three Afrikaner founder low density lipoprotein receptor gene mutations Afrikaner
Allelic variants of human calcitonin receptor in the Japanese population
Molecular reconstruction and homology modelling of the catalytic domain of the common ancestor of the haemostatic vitamin-K-dependent serine proteinases
Genetic fine mapping of the gene for recessive Stargardt disease
Activation status of the X chromosome in human micronucleated lymphocytes
Distribution of mosaicism in human placentae
Haplotype identity between individuals who share a CFTR mutation allele "identical by descent": demonstration of the usefulness of the haplotype-sharing concept for gene mapping in real populations
Identification of the nucleotide substitution that generates the fourth polymorphic site in human deoxyribonuclease I (DNase I)
Genetic mapping of the human pituitary-specific transcriptional factor gene and its analysis in familial panhypopituitary dwarfism
Exclusion of the phosphatidylinositol-specific phospholipase C β3 (PLC β3) gene as candidate for the multiple endocrine neoplasia type 1 (MEN 1) gene
Mosaicism for the Charcot-Marie-Tooth disease type 1A duplication suggests somatic reversion
The Ca 2+ -sensing receptor gene (PCAR1) mutation T151M in isolated autosomal dominant hypoparathyroidism
A novel mutation of presenilin 1 in familial Alzheimer's disease in Israel detected by denaturing gradient gel electrophoresis
Quantification by flow cytometry of chromosome-17 deletions in Smith-Magenis syndrome patients
Genetic polymorphisms of the renin-angiotensin system and angiographic extent and severity of coronary artery disease: the CORGENE study
Tetrasomy 18p de novo: identification by FISH with conventional and microdissection probes and analysis of parental origin and formation by short sequence repeat typing
Duplication of the PMP22 gene in 17p partial trisomy patients with Charcot-Marie-Tooth type-1A neuropathy
Mapping of the variegate porphyria (VP) gene: contradictory evidence for linkage between VP and microsatellite markers at chromosome 14q32
Isolation of the human chromosome 22q telomere and its application to detection of cryptic chromosomal abnormalities
Evidence of genetic heterogeneity of Leber's congenital amaurosis (LCA) and mapping of LCA1 to chromosome 17p13
A PCR-based test suitable for screening for fragile X syndrome among mentally retarded males
Pycnodysostosis: refined linkage and radiation hybrid analyses reduce the critical region to 2 cM at 1q21 and map two candidate genes
New mutations in the ataxia telangiectasia gene
A LINE element is present at the site of a 300-kb deletion starting in intron 10 of the PAX6 gene in a case of familial aniridia
Precise localization of the human gene encoding cell adhesion kinase β (CAKβ/PYK2) to chromosome 8 at p21.1 by fluorescence in situ hybridization
Mutations of the Btk gene in 12 unrelated families with X-linked agammaglobulinemia in Japan
Novel rhodopsin mutation in an autosomal dominant retinitis pigmentosa family: phenotypic variation in both heterozygote and homozygote Val137Met mutant patients
Characterization of double minute chromosomes' DNA content in a human high grade astrocytoma cell line by using comparative genomic hybridization and fluorescence in situ hybridization
Secretory phospholipase A 2 does not appear to be associated with phenotypic variation in familial adenomatous polyposis