Human Genetics - 2001

210 articles | Last updated: 2025-12-03 14:12:56
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T A T A T A T A
Human Gene Mutations
Back mutation can produce phenotype reversion in Bloom syndrome somatic cells
Connexin 26 ( GJB2 ) mutations in the Turkish population: implications for the origin and high frequency of the 35delG mutation in Caucasians
Recombination in the pseudoautosomal region in a 47,XYY male
The mutation spectrum of the facilitative glucose transporter gene SLC2A2 (GLUT2) in patients with Fanconi-Bickel syndrome
Linkage disequilibrium between GABRB3 gene and nonsyndromic familial cleft lip with or without cleft palate
Critique of “Chromosome 17 and the inducible nitric oxide synthase gene in human essential hypertension” by Rutherford et al., Human Genetics, published online September 2001
Cytogenetic mapping of a novel locus for type II Waardenburg syndrome
Authors response to: Critique of "Chromosome 17 and inducible nitric oxide synthase gene in human essential hypertension" by Rutherford et al. in Human Genetics published on-line September 2001
Acknowledgement to Referees 2001
A silent mutation induces exon skipping in the phenylalanine hydroxylase gene in phenylketonuria
Transmission ratio distortion in offspring of heterozygous female carriers of Robertsonian translocations
Characterization of new mutations in the coding sequence and 5'-untranslated region of the human prostacyclin synthase gene ( CYP8A1)
Numerical chromosome abnormalities in the spermatozoa of the fathers of children with trisomy 21 of paternal origin: generalised tendency to meiotic non-disjunction
Polymorphism in promoter region of Fcα receptor gene in patients with IgA nephropathy
A new multicolor-FISH approach for the characterization of marker chromosomes: centromere-specific multicolor-FISH (cenM-FISH)
A summary of 20 CACNA1F mutations identified in 36 families with incomplete X-linked congenital stationary night blindness, and characterization of splice variants
A method for sex assignment in mixed samples
Haplotype analysis of genomic polymorphisms in and around the myotonic dystrophy locus in diverse populations of India
Late-onset Stargardt disease is associated with missense mutations that map outside known functional regions of ABCR ( ABCA4 )
Characterisation of novel point mutations in the survival motor neuron gene SMN , in three patients with SMA
Mutation analysis of the origin recognition complex subunit 5 ( ORC5L ) gene in adult patients with myeloid leukemias exhibiting deletions of chromosome band 7q22
Delineation of the critical interval for the familial exudative vitreoretinopathy gene by linkage and haplotype analysis
TheTNFRSF6 gene is not implicated in familial early-onset Alzheimer's disease
Evaluation of the fragile X (FRAXA) syndrome with methylation-sensitive PCR
Partial maternal heterodisomy of chromosome 17q25 in a case of severe mental retardation
Interaction between the melanocortin-1 receptor andP genes contributes to inter-individual variation in skin pigmentation phenotypes in a Tibetan population
"Bar-coding" primate chromosomes: molecular cytogenetic screening for the ancestral hominoid karyotype
Frequency of replication/transcription errors in (A)/(T) runs of human genes
Glutamine repeats and neurodegenerative diseases: molecular aspects
A second case of somatic triple mosaicism in the CYBB gene causing chronic granulomatous disease
The position of t(11;22)(q23;q11) constitutional translocation breakpoint is conserved among its carriers
Ulrich R. Hengge, Beatrix Volc-Platzer (eds): The skin and gene therapy
Familial idiopathic basal ganglia calcification (Fahr's disease) without neurological, cognitive and psychiatric symptoms is not linked to the IBGC1 locus on chromosome 14q
Paraoxonase gene Gln192Arg (Q192R) polymorphism is associated with coronary artery spasm
Identification, genomic organization, chromosomal mapping and mutation analysis of the human INV gene, the ortholog of a murine gene implicated in left-right axis development and biliary atresia
Novel missense mutations outside the allosteric domain of glutamate dehydrogenase are prevalent in European patients with the congenital hyperinsulinism-hyperammonemia syndrome European patients
Lack of association between α2-macroglobulin polymorphisms and Alzheimer's disease
Isolation and characterization of the UBASH3A gene on 21q22.3 encoding a potential nuclear protein with a novel combination of domains
CFTR gene mutations - including three novel nucleotide substitutions - and haplotype background in patients with asthma, disseminated bronchiectasis and chronic obstructive pulmonary disease
Complex segregation analysis of Parkinson's disease in the Finnish population
Identification of a new point mutation in the human xanthine dehydrogenase gene responsible for a case of classical type I xanthinuria
Genomic organization, chromosomal localization, alternative splicing, and isoforms of the human synaptosome-associated protein-23 gene implicated in vesicle-membrane fusion processes
Increased reproductive success of MHC class II heterozygous males among free-ranging rhesus macaques
The genes for the human VPS10 domain-containing receptors are large and contain many small exons
Nomenclature for the description of human sequence variations
The structure of duplications on human acrocentric chromosome short arms derived by the analysis of 15p
Distribution of glucose-6-phosphate dehydrogenase mutations in Southeast Asia
Analysis of lymphoedema-distichiasis families forFOXC2 mutations reveals small insertions and deletions throughout the gene
Population-based risk estimates of Wilms tumor in sporadic aniridia
Niemann-Pick type C disease: NPC1 mutations associated with severe and mild cellular cholesterol trafficking alterations
Prevalence of BRCA1 and BRCA2 mutations among clinic-based African American families with breast cancer
Expression pattern, genomic structure and evaluation of the human SLC30A4 gene as a candidate for acrodermatitis enteropathica
A molecular approach to dominance in hypophosphatasia
Analysis of splicing parameters in the dystrophin gene: relevance for physiological and pathogenetic splicing mechanisms
Archival, demographic and genetic studies define a Sardinian sub-isolate as a suitable model for mapping complex traits Sardinian
Opposite association of two PPARG variants with cancer: overrepresentation of H449H in endometrial carcinoma cases and underrepresentation of P12A in renal cell carcinoma cases
Joyce C. Harper, Joy D.A. Delhanty, Alan H. Handyside (eds): Preimplantation genetic diagnosis
Genetic variations in the cholesteryl ester transfer protein gene and high density lipoprotein cholesterol levels in Taiwanese Chinese
Epstein syndrome: another renal disorder with mutations in the nonmuscle myosin heavy chain 9 gene
A detailed analysis of the MECP2 gene: prevalence of recurrent mutations and gross DNA rearrangements in Rett syndrome patients
The relationship between Y chromosome DNA haplotypes and Y chromosome deletions leading to male infertility
Human type I hair keratin pseudogene ? hHaA has functional orthologs in the chimpanzee and gorilla: evidence for recent inactivation of the human gene after the Pan-Homo divergence
Neonatal presentation of adult-onset type II citrullinemia
The missense Glu298Asp variant of the endothelial nitric oxide synthase gene is strongly associated with placental abruption
TSC1 and TSC2 deletions differ in size, preference for recombinatorial sequences, and location within the gene
Cross-sectional study on cytokine polymorphisms, cytokine production after T-cell stimulation and clinical parameters in a random sample of a German population
Elite swimmers and the D allele of the ACE I/D polymorphism
SMN gene duplication and the emergence of the SMN2 gene occurred in distinct hominids: SMN2 is unique to Homo sapiens
De novo mutation of the connexin 26 gene associated with dominant non-syndromic sensorineural hearing loss
Scandinavian CDG-Ia patients: genotype/phenotype correlation and geographic origin of founder mutations
Topoisomerase-I- and Alu-mediated genomic deletions of the APC gene in familial adenomatous polyposis
Oto-facio-cervical (OFC) syndrome is a contiguous gene deletion syndrome involving EYA1 : molecular analysis confirms allelism with BOR syndrome and further narrows the Duane syndrome critical region
Segregation of a mutation in CNGB1 encoding the β-subunit of the rod cGMP-gated channel in a family with autosomal recessive retinitis pigmentosa
DHPLC-based germline mutation screening in the analysis of the VHL tumor suppressor gene: usefulness and limitations
Molecular and functional characterisation of mild MCAD deficiency
Cloning and characterization of the human GFRA2 locus and investigation of the gene in Hirschsprung disease
Mutation analysis of 4 candidate genes for hereditary neuralgic amyotrophy (HNA)
Statistical estimation and pedigree analysis ofCCR2-CCR5 haplotypes
Evidence for a prostate cancer linkage to chromosome 20 in 159 hereditary prostate cancer families
Proteomics - from protein sequence to function
Identification of duplicated genes in 17q11.2 using FISH on stretched chromosomes and DNA fibers
Galactosemia: deletion in the 5′ upstream region of the GALT gene reduces promoter efficiency
Linkage and association to candidate regions in Swedish atopic dermatitis families Swedish
Combined segregation-linkage analysis of plasma thrombin activatable fibrinolysis inhibitor (TAFI) antigen levels with TAFI gene polymorphisms
Identification and characterization of a tissue-specific silencer element in the first intron of the human acid maltase gene
Germ line insertion of mtDNA at the breakpoint junction of a reciprocal constitutional translocation
Laminin 5 mutations in junctional epidermolysis bullosa: molecular basis of Herlitz vs non-Herlitz phenotypes
Rate of homologous chromosome bivalents in spermatocytes may predict completion of spermatogenesis in azoospermic men
Dissecting the epidemiology of a trinucleotide repeat disease – example of FRDA in Finland
A major marker for normal tension glaucoma: association with polymorphisms in the OPA1 gene
A genomic map of a 6-Mb region at 13q21-q22 implicated in cancer development: identification and characterization of candidate genes
Evidence of a founder effect and refinement of the hereditary neuralgic amyotrophy (HNA) locus on 17q25 in American families
Mutations in human glycine N-methyltransferase give insights into its role in methionine metabolism
Fetal gender determination in early pregnancy through qualitative and quantitative analysis of fetal DNA in maternal serum
Three major lineages of Asian Y chromosomes: implications for the peopling of east and southeast Asia
Investigation of the functional effect of monoamine oxidase polymorphisms in human brain
The USH1C 216G→A mutation and the 9-repeat VNTR(t,t) allele are in complete linkage disequilibrium in the Acadian population
Effectiveness of computational methods in haplotype prediction
Patterns of haplotype diversity within the serpin gene cluster at 14q32.1: insights into the natural history of the α1-antitrypsin polymorphism
The human olfactory subgenome: from sequence to structure and evolution
AFLP fingerprinting of the human genome
Diverse prevalence of large deletions within the OA1 gene in ocular albinism type 1 patients from Europe and North America
Hybrids monosomal for human chromosome 5 reveal the presence of a spinal muscular atrophy (SMA) carrier with two SMN1 copies on one chromosome
Marked differences in unilateral isolated retinoblastomas from young and older children studied by comparative genomic hybridization
Characterisation of SMN hybrid genes in Spanish SMA patients: de novo, homozygous and compound heterozygous cases
Clonal maintenance of imprinted expression of SNRPN and IPW in normal lymphocytes: correlation with allele-specific methylation of SNRPN intron 1 but not intron 7
Quantitation of fetal DNA in maternal serum in normal and aneuploid pregnancies
Mitochondrial DNA haplogroups and APOE4 allele are non-independent variables in sporadic Alzheimer's disease
Phenotypic effects of balanced X-autosome translocations in females: a retrospective survey of 104 cases reported from UK laboratories
A single nucleotide polymorphism (SNP) in the leptin receptor is associated with BMI, fat mass and leptin levels in postmenopausal Caucasian women
Molecular analysis of the fibrinogen gene cluster in 16 patients with congenital afibrinogenemia: novel truncating mutations in the FGA and FGG genes
Carrier rate of APC I1307K is not increased in inflammatory bowel disease patients of Ashkenazi Jewish origin Ashkenazi Jewish origin
Hearing impairment in patients with 3243A→G mtDNA mutation: phenotype and rate of progression
Linkage of prostate cancer susceptibility loci to chromosome 1
Complete and rapid scanning of the cystic fibrosis transmembrane conductance regulator (CFTR) gene by denaturing high-performance liquid chromatography (D-HPLC): major implications for genetic counsel
Identification of novel DKC1 mutations in patients with dyskeratosis congenita: implications for pathophysiology and diagnosis
Improved definition of chromosomal breakpoints using high-resolution multicolour banding
Refinement of the chromosome 5p locus for craniometaphyseal dysplasia
Nigel M. Hooper (editor): Alzheimer’s disease. Methods and protocols
Physical and transcriptional mapping of the X-linked cleft palate and ankyloglossia (CPX) critical region
Acrocentric chromosome disomy is increased in spermatozoa from fathers of Turner syndrome patients
Profiles of accepted mutation: from neutrality in a pseudogene to disease-causing mutation on its homologous gene
Analyses of genetic abnormalities in type I CD36 deficiency in Japan: identification and cell biological characterization of two novel mutations that cause CD36 deficiency in man
A 1.1-kb duplication in the p67-phox gene causes chronic granulomatous disease
Preimplantation genetic diagnosis for spinal and bulbar muscular atrophy (SBMA)
Identification of novel mutations in SHH and ZIC2 in a South American (ECLAMC) population with holoprosencephaly
Novel mtDNA mutations and oxidative phosphorylation dysfunction in Russian LHON families
Duplication and transposition of the NF1 pseudogene regions on chromosomes 2, 14, and 22
The Basques according to polymorphic Alu insertions
Glycerol kinase deficiency: Evidence for complexity in a single gene disorder
Chimpanzee apolipoprotein H (β2-glycoprotein I): report on the gene structure, a common polymorphism, and a high prevalence of antiphospholipid antibodies
The polyglutamine motif is highly conserved at the Clock locus in various organisms and is not polymorphic in humans
Large-scale molecular screening for galactosemia alleles in a pan-ethnic population
A polymorphism in the promoter region of catalase is associated with blood pressure levels
Genetic basis of mitochondrial HMG-CoA synthase deficiency
Mutation analyses in 17 patients with deficiency in acid β-galactosidase: three novel point mutations and high correlation of mutation W273L with Morquio disease type B
AcroM fluorescent in situ hybridization analyses of marker chromosomes
Mutational spectrum in the cardioauditory syndrome of Jervell and Lange-Nielsen
Analysis of the CC chemokine receptor 5 (CCR5) delta-32 polymorphism in inflammatory bowel disease
Genome-wide distribution of linkage disequilibrium in the population of Palau and its implications for gene flow in Remote Oceania
Somatic NF1 mutational spectrum in benign neurofibromas: mRNA splice defects are common among point mutations
Erratum to Back mutation can produce phenotype reversion in Bloom syndrome somatic cells
Marfan syndrome caused by a mutation in FBN1 that gives rise to cryptic splicing and a 33 nucleotide insertion in the coding sequence
Genetic risk factors of venous thrombosis
Correcting for multiple testing in genetic association studies: the legend lives on
Extent of linkage disequilibrium between the androgen receptor gene CAG and GGC repeats in human populations: implications for prostate cancer risk
Eberhard Passarge: Color Atlas of Genetics. (2nd edition, enlarged and revised)
G. Scherer and M. Schmid (eds): Genes and mechanisms in vertebrate sex determination
Ettore Olmo and Carlo Alberto Redi (eds): Chromosomes today, vol 13
Helmut Schenkel-Brunner: Human blood groups: chemical and biological basis of antigen, 2nd edn., Springer-Verlag, Wien New York. ISBN 3-211-83471-0. DM 198.–, US$ 99.0. Hardcover, 637 pages
Hemifacial microsomia: progress in understanding the genetic basis of a complex malformation syndrome
Qualitative and quantitative analysis of mRNA associated with four putative splicing mutations (621+3A→G, 2751+2T→A, 296+1G→C, 1717–9T→C-D565G) and one nonsense mutation (E822X) in the CFTR gene
Physical activity modulates the combined effect of a common variant of the lipoprotein lipase gene and smoking on serum triglyceride levels and high-density lipoprotein cholesterol in men
Subtelomeric rearrangements: results from a study of selected and unselected probands with idiopathic mental retardation and control individuals by using high-resolution G-banding and FISH
Mutation spectrum and splicing variants in the OPA1 gene
Scanning for telomeric deletions and duplications and uniparental disomy using genetic markers in 120 children with malformations
Submicroscopic terminal deletions and duplications in retarded patients with unclassified malformation syndromes
Characterisation of two mutations in the ABCD1 gene leading to low levels of normal ALDP
Absence of correlation between late-replication and spreading of X inactivation in an X;autosome translocation
Genomic structure of karyopherin ?2 ( KPNA2 ) within a low-copy repeat on chromosome 17q23-q24 and mutation analysis in patients with Russell-Silver syndrome
Structure of human holocarboxylase synthetase gene and mutation spectrum of holocarboxylase synthetase deficiency
Identification of 96 single nucleotide polymorphisms in eight genes involved in iron metabolism: efficiency of bioinformatic extraction compared with a systematic sequencing approach
Announcement
A novel intronic mutation of the TAZ ( G4.5 ) gene in a patient with Barth syndrome: creation of a 5' splice donor site with variant GC consensus and elongation of the upstream exon
The molecular basis of familial hypercholesterolemia in The Netherlands
Mutational scanning of the ABCR gene with double-gradient denaturing-gradient gel electrophoresis (DG-DGGE) in Italian Stargardt disease patients
Evaluation of denaturing high performance liquid chromatography (DHPLC) for the mutational analysis of the neurofibromatosis type 1 ( NF1 ) gene
Identification of ABCC6 pseudogenes on human chromosome 16p: implications for mutation detection in pseudoxanthoma elasticum
Mutational analysis of 85 mucopolysaccharidosis type I families: frequency of known mutations, identification of 17 novel mutations and in vitro expression of missense mutations
Christopher Gillberg and Mary Coleman: The biology of the autistic syndromes, 3rd edn
Satellite 2 methylation patterns in normal and ICF syndrome cells and association of hypomethylation with advanced replication
Muscular Dystrophy: methods and protocols
Armenian Y chromosome haplotypes reveal strong regional structure within a single ethno-national group
Prenatal diagnosis and fetal pathology in a Turkish family harboring a novel nonsense mutation in the lysosomal α-N-acetyl-neuraminidase (sialidase) gene
Respiratory distress syndrome: evaluation of genetic susceptibility and protection by transmission disequilibrium test
Phenotypic variability at the TGF-β1 locus in Camurati-Engelmann disease
DNA sequence comparison of human and mouse retinitis pigmentosa GTPase regulator (RPGR) identifies tissue-specific exons and putative regulatory elements
Announcement
Are we hardwired? The role of genes in human behaviour
Supravalvular aortic stenosis: genetic and molecular dissection of a complex mutation in the elastin gene
Identification and characterization of two novel human mitochondrial elongation factor genes, hEFG2 and hEFG1, phylogenetically conserved through evolution
A full-length and potentially active LINE element is integrated polymorphically within the IGL locus in a genomically unstable region of chromosome 22
Association between nasal allergy and a coding variant of the Fc ε RI β gene Glu237Gly in a Japanese population
Polymorphism in the alpha1-antichymotrypsin (ACT) gene promoter: effect on expression in transfected glial and liver cell lines and plasma ACT concentrations
Polymorphisms within the prion-like protein gene (Prnd) and their implications in human prion diseases, Alzheimer's disease and other neurological disorders
Genetic case-control association studies - correcting for multiple testing
Chromosome 17 and the inducible nitric oxide synthase gene in human essential hypertension
TGFβ1 allele association with asthma severity
Krystyne E. Wisniewski and Nanbert Zhong (eds): Batten disease: diagnosis, treatment and research. (Advances in Genetics, vol 45)
A genome-wide survey of human thioredoxin and glutaredoxin family pseudogenes
Analysis of short stature homeobox-containing gene ( SHOX ) and auxological phenotype in dyschondrosteosis and isolated Madelung deformity
Molecular pathology and evolutionary and physiological implications of pancreatitis-associated cationic trypsinogen mutations
Linkage of body mass index to chromosome 20 in Utah pedigrees
A frameshift mutation in exon 28 of the OPA1 gene explains the high prevalence of dominant optic atrophy in the Danish population: evidence for a founder effect
Novel mutations of MYO15A associated with profound deafness in consanguineous families and moderately severe hearing loss in a patient with Smith-Magenis syndrome
A new strategy for the detection of subtelomeric rearrangements
Suomi-Neidon Geenit (The Genes of Maiden Finland)
Long mutant dystrophins and variable phenotypes: evasion of nonsense-mediated decay?
Association of the mitochondrial DNA 5178 A/C polymorphism with serum lipid levels in the Japanese population Japanese population
The HUGO Gene Nomenclature Committee (HGNC)
ASP – a simulation-based power calculator for genetic linkage studies of qualitative traits, using sib-pairs
Genetics and public health in the 21st century
Insulator: from chromatin domain boundary to gene regulation
Substantial linkage disequilibrium across the insulin-degrading enzyme locus but no association with late-onset Alzheimer's disease
The murine orthologue of the Golgi-localized TPTE protein provides clues to the evolutionary history of the human TPTE gene family
Karen Steinberg: The Genetic Basis of Cancer [CD Rom]
Compound heterozygosity and nonsense mutations in the α1-subunit of the inhibitory glycine receptor in hyperekplexia
Genomic structures and population histories of linguistically distinct tribal groups of India
Genetic predisposition to ventricular septal defect in Down syndrome