Human Genetics - 2002

179 articles | Last updated: 2025-12-03 14:12:56
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T A T A T A T A
Patterns of associations of clinical features in neurofibromatosis 1 (NF1)
FMR1 haplotype analyses among Indians: a weak founder effect and other findings
Testing hypotheses of language replacement in the Caucasus: evidence from the Y-chromosome
Influence of interleukin-12 receptor β1 polymorphisms on tuberculosis
A novel autosomal dominant non-syndromic deafness locus (DFNA48) maps to 12q13-q14 in a large Italian family Italian family
Relevance of sequence and structure elements for deletion events in the dystrophin gene major hot-spot
Susceptibility gene for non-obstructive azoospermia located near HLA-DR and -DQ loci in the HLA class II region
Genetic variation in α1-antichymotrypsin and its association with Alzheimer’s disease
A novel stable polyalanine [poly(A)] expansion in the HOXA13 gene associated with hand-foot-genital syndrome: proper function of poly(A)-harbouring transcription factors depends on a critical repeat l
Psychosocial genetic counseling
The role of mtDNA background in disease expression: a new primary LHON mutation associated with Western Eurasian haplogroup J Western Eurasian
Genetics in medicine (Sixth Edition)
Relaxation of imprinted expression of ZAC and HYMAI in a patient with transient neonatal diabetes mellitus
CIA30 complex I assembly factor: a candidate for human complex I deficiency?
Transmission disequilibrium test with discordant sib pairs when parents are available
Management of genetic syndromes
Molecular characterization of a ring X chromosome in a male with short stature
Structure of the human argininosuccinate synthetase gene and an improved system for molecular diagnostics in patients with classical and mild citrullinemia
Vitiligo: complex segregation and linkage disequilibrium analyses with respect to microsatellite loci spanning the HLA
A case of segmental paternal isodisomy of chromosome 14
Endocrine Development, vol. 4. Hypothalamic-Pituitary Development: Genetic and Clinical Aspects
Antenatal and Neonatal Screening [Second Edition]
Mutations in PATCHED-1, the receptor for SONIC HEDGEHOG, are associated with holoprosencephaly
The left-right determinant inversin has highly conserved ankyrin repeat and IQ domains and interacts with calmodulin
Unstable transmission of the RS447 human megasatellite tandem repetitive sequence that contains the USP17 deubiquitinating enzyme gene
Congenital fibrosis of the vertically acting extraocular muscles maps to the FEOM3 locus
D-loop mutations in mitochondrial DNA: link with mitochondrial DNA depletion?
Genetic diversity and evolution of the human leptin locus tetranucleotide repeat
Mutations in the WFS1 gene that cause low-frequency sensorineural hearing loss are small non-inactivating mutations
A novel and unusual case of chronic granulomatous disease in a child with a homozygous 36-bp deletion in the CYBA gene (A220) leading to the activation of a cryptic splice site in intron 4
Felix Mitelman: Database of chromosome aberrations in cancer
Distribution of intrachromosomal telomeric sequences (ITS) on Macaca fascicularis (Primates) chromosomes and their implication for chromosome evolution
Mutation analysis of the MKKS gene in McKusick-Kaufman syndrome and selected Bardet-Biedl syndrome patients
A new locus for coeliac disease mapped to chromosome 15 in a population isolate
The 5′ end of the KCNQ1OT1 gene is hypomethylated in the Beckwith-Wiedemann syndrome
Not all isolates are equal: linkage disequilibrium analysis on Xq13.3 reveals different patterns in Sardinian sub-populations
Sequence polymorphism at the human apolipoprotein AII gene (APOA2): unexpected deficit of variation in an African-American sample African-American
A point mutation, R59G, within the HMG-SRY box in a female 45,X/46,X, psu dic(Y)(pter→q11::q11→pter)
Megalencephalic leukoencephalopathy with subcortical cysts; a founder effect in Israeli patients and a higher than expected carrier rate among Libyan Jews Libyan Jews; Israeli patients
David L. Rimoin, J. Michael Connor, Reed E. Pyeritz, Bruce R. Korf (eds): Emery and Rimoin’s Principles and Practices of Medical Genetics - Fourth Edition
Chromosome 7p disruptions in Silver Russell syndrome: delineating an imprinted candidate gene region.
Evidence for a gene influencing heart rate on chromosome 4 among hypertensives
Functional characterization of a natural variant of luteinizing hormone
Identification of a novel SCA locus ( SCA19 ) in a Dutch autosomal dominant cerebellar ataxia family on chromosome region 1p21-q21 Dutch
Frontiers of hormone research (series editor: A.B. Grossman), volume 28: genetic disorders of endocrine neoplasia
Mutation analysis of five new patients affected by prolidase deficiency: the lack of enzyme activity causes necrosis-like cell death in cultured fibroblasts
Associations between gene expressions in breast cancer and patient survival
A novel mutation in the IHH gene causes brachydactyly type A1: a 95-year-old mystery resolved
Y-chromosomal evidence for a strong reduction in male population size of Yakuts
Proposed guidelines for papers describing DNA polymorphism-disease associations
Variability of X chromosome inactivation: effect on levels of TIMP1 RNA and role of DNA methylation
Genetic study of SMA patients without homozygous SMN1 deletions: identification of compound heterozygotes and characterisation of novel intragenic SMN1 mutations
Identification of novel mutations in MLC1 responsible for megalencephalic leukoencephalopathy with subcortical cysts
A novel locus for Usher syndrome type I, USH1G, maps to chromosome 17q24–25
Homocysteine in Health and Disease
A locus for hereditary capillary malformations mapped on chromosome 5q
Human Gene Mutations
Identity-by-descent approach to gene localisation in eight individuals affected by keratoconus from north-west Tasmania, Australia
A loss-of-function mutation in the CFC domain of TDGF1 is associated with human forebrain defects
RT-PCR splicing analysis of the NF1 open reading frame
Timing of the absence of FMR1 expression in full mutation chorionic villi
Homogeneity and distinctiveness of Polish paternal lineages revealed by Y chromosome microsatellite haplotype analysis
Nonsyndromic recessive deafness DFNB18 and Usher syndrome type IC are allelic mutations of USHIC
PKLR-GBA region shows almost complete linkage disequilibrium over 70 kb in a set of worldwide populations
Allele-specific PCR amplification due to sequence identity between a PCR primer and an amplicon : is direct sequencing so reliable?
Mutations in the alternatively spliced exons of USH1C cause non-syndromic recessive deafness
Analysis of phylogenetically reconstructed mutational spectra in human mitochondrial DNA control region
Human connexin26 (GJB2) deafness mutations affect the function of gap junction channels at different levels of protein expression.
Simopoulos AP, Pavlou KN (eds): Nutrition and fitness: Diet, genes, physical activity and health. World review of nutrition and dietetics, vol 89
Erratum to: Identification of novel mutations in MLC1 responsible for megalencephalic leukoencephalopathy with subcortical cysts
Progressive hearing loss, and recurrent sudden sensorineural hearing loss associated with GJB2 mutations – phenotypic spectrum and frequencies of GJB2 mutations in Austria
Folate and human development
DNA hypermethylation: when tumour suppressor genes go silent
Differential rates of frameshift alterations in four repeat sequences of hereditary nonpolyposis colorectal cancer tumors
Molecular characterisation of t(17;22)(q11.2;q11.2) is not consistent with NF1 gene duplication
Complex segregation analysis of hypospadias
A genome screen for linkage disequilibrium in HLA-DRB1*15-positive Germans with multiple sclerosis based on 4666 microsatellite markers Germans
Population variation in linkage disequilibrium across the COMT gene considering promoter region and coding region variation
Duplication of the CYP21A2 gene complicates mutation analysis of steroid 21-hydroxylase deficiency: characteristics of three unusual haplotypes
The prevalence of connexin 26 ( GJB2 ) mutations in the Chinese population
Positive results in association studies are associated with departure from Hardy-Weinberg equilibrium: hint for genotyping error?
Nonsense-mediated RNA decay in the TSC1 gene suggests a useful tool pre- and post-positional cloning
Jan Klein, Naoyuki Takahata: Where do we come from? The molecular evidence for human descent
Mitochondrial DNA 5178A polymorphism and longevity
Derek Chadwick, Jamie Goode (eds): The Genetics and Biology of Sex Determination (Novartis Symposium 244)
Pyruvate dehydrogenase E3 binding protein deficiency
Handbook of statistical genetics:
Identification and characterization of a novel liver-specific enhancer of the human phenylalanine hydroxylase gene
Contribution of arylsulfatase A mutations located on the same allele to enzyme activity reduction and metachromatic leukodystrophy severity
Universal, robust, highly quantitative SNP allele frequency measurement in DNA pools
Estimation of single nucleotide polymorphism allele frequency in DNA pools by using Pyrosequencing
Study of 30 patients with unexplained developmental delay and dysmorphic features or congenital abnormalities using conventional cytogenetics and multiplex FISH telomere (M-TEL) integrity assay
A life of Sir Francis Galton. From African exploration to the birth of eugenics
SLEB3 in systemic lupus erythematosus (SLE) is strongly related to SLE families ascertained through neuropsychiatric manifestations
Prevalence of the CCR5Δ32 mutation in Brazilian populations and cell susceptibility to HIV-1 infection
Juvenile polyposis: massive gastric polyposis is more common in MADH4 mutation carriers than in BMPR1A mutation carriers
Announcement
Organization of the human liver carnitine palmitoyltransferase 1 gene (CPT1A) and identification of novel mutations in hypoketotic hypoglycaemia
Major gene evidence after MTHFR-segregation analysis of serum homocysteine in families of patients undergoing coronary arteriography
London Dysmorphology Database, London Neurogenetics Database and Dysmorphology Photo Library on CD-ROM [Version 3] 2001
Human Chromosomes, fourth edition
Functional characterization of the new human GABAA receptor mutation β3(R192H)
FOXP2: novel exons, splice variants, and CAG repeat length stability
A genome-wide screen reveals evidence for a locus on chromosome 11 influencing variation in LDL cholesterol in the NHLBI Family Heart Study
Alan R. Clarke (ed): Transgenesis techniques — principles and protocols, 2nd edn.
Thymidylate synthase: a novel genetic determinant of plasma homocysteine and folate levels
Duplications of chromosome 11p15 of maternal origin result in a phenotype that includes growth retardation
Presence of fetal DNA in maternal plasma decades after pregnancy: further comments
Mutations in PATCHED-1, the receptor for SONIC HEDGEHOG, are associated with holoprosencephaly
Linkage of one gene for familial glucocorticoid deficiency type 2 (FGD2) to chromosome 8q and further evidence of heterogeneity
Markers that discriminate between European and African ancestry show limited variation within Africa European and African ancestry
Autosomal recessive renal glucosuria attributable to a mutation in the sodium glucose cotransporter (SGLT2)
Association found between the promoter region polymorphism in the apolipoprotein A-V gene and the serum triglyceride level in Japanese schoolchildren
Bioinformatics - from genomes to drugs (2 volumes)
ZNT4 gene is not responsible for acrodermatitis enteropathica in Japanese families
Polymorphic GGC repeats in the androgen receptor gene are associated with hereditary and sporadic prostate cancer risk
Evidence of multiple causal sites affecting weight in the IGF2-INS-TH region of human chromosome 11
Origin and affinities of indigenous Siberian populations as revealed by HLA class II gene frequencies
C.R. Scriver, A.L. Beaudet, W.S. Sly, D. Valle (eds.), B. Childs, K.W. Kinzler, B. Vogelstein (associate eds.): Molecular and metabolic bases of inherited disease, 8th edition
Genetics of congenital deafness in the Palestinian population: multiple connexin 26 alleles with shared origins in the Middle East
Characterisation of interstitial duplications and triplications of chromosome 15q11–q13
A candidate gene for congenital bilateral isolated ptosis identified by molecular analysis of a de novo balanced translocation
Linkage analysis identifies the thyroglobulin gene region as a major locus for familial congenital hypothyroidism
Wiley encyclopedia of molecular medicine
Homozygosity mapping of a Weill-Marchesani syndrome locus to chromosome 19p13.3-p13.2
Genetics and the electroencephalogram
Haploinsufficiency of PAX9 is associated with autosomal dominant hypodontia
Tandem duplication of the NF1 gene detected by high-resolution FISH in the 17q11.2 region
Identification of additional transcripts in the Williams-Beuren syndrome critical region
Investigating the association between OPA1 polymorphisms and glaucoma: comparison between normal tension and high tension primary open angle glaucoma
FISH-mapping of a 100-kb terminal 22q13 deletion
An Alu-mediated deletion at Xp11.23 leading to Wiskott-Aldrich syndrome
Nuclear mitochondrial interplay in the modulation of the homopolymeric tract length heteroplasmy in the control (D-loop) region of the mitochondrial DNA
Genome-wide linkage analysis assessing parent-of-origin effects in the inheritance of birth weight
Analysis of 22 deletion breakpoints in dystrophin intron 49
Robert J. Gorlin, M. Michael Cohen Jr, Raoul C.M. Hennekam (eds): Syndromes of the Head and Neck, 4th edition
CYP3A4-V and prostate cancer in African Americans: causal or confounding association because of population stratification?
DNA polymorphisms in potential regulatory elements of the CFTR gene alter transcription factor binding
Centromeric association of chromosome 16- and 18-derived microchromosomes
Identification of a large deletion and three novel mutations in exon 13 of the factor V gene in a Spanish family with normal factor V coagulant and anticoagulant properties
Haplotype study of West European and North African Unverricht-Lundborg chromosomes: evidence for a few founder mutations West European; North African
Different familial adenomatous polyposis phenotypes resulting from deletions of the entire APC exon 15
Unexpectedly high prevalence of the mild form of propionic acidemia in Japan: presence of a common mutation and possible clinical implications
Microsatellite polymorphism in promoter of heme oxygenase-1 gene is associated with susceptibility to coronary artery disease in type 2 diabetic patients
A detailed physical map of the 6p reading disability locus, including new markers and confirmation of recombination suppression
Molecular analysis in true hermaphroditism: demonstration of low-level hidden mosaicism for Y-derived sequences in 46,XX cases
Androgen receptor CAG polymorphism and prostate cancer risk
Evolution of a VNTR located within the promoter region of the thiopurine methyltransferase gene: inferences from population and sequence data
Assignment of a locus for autosomal dominant idiopathic scoliosis (IS) to human chromosome 17p11
Mutation analysis of the coding sequence of the MECP2 gene in infantile autism
Spatial and temporal distribution of cystic fibrosis and of its mutations in Brittany, France: a retrospective study from 1960
Gene dosage of the spermidine/spermine N1-acetyltransferase (SSAT) gene with putrescine accumulation in a patient with a Xp21.1p22.12 duplication and keratosis follicularis spinulosa decalvans (KFSD)
Genomic evidence versus characterisation of a single (17;22) translocation on NF1 gene duplication: lessons from deletions in "balanced" chromosomal rearrangements. Reply
Evidence against the long-term persistence of fetal DNA in maternal plasma after pregnancy
Genetic basis of inosine triphosphate pyrophosphohydrolase deficiency
Investigation of oxidized LDL-receptor 1 (OLR1) as the candidate gene for Alzheimer's disease on chromosome 12
Absence of PTPN11 mutations in 28 cases of cardiofaciocutaneous (CFC) syndrome
Periodic catatonia: confirmation of linkage to chromosome 15 and further evidence for genetic heterogeneity
CTLA-4 gene polymorphisms in systemic lupus erythematosus: a highly significant association with a determinant in the promoter region
Evidence for a founder effect for pseudoxanthoma elasticum in the Afrikaner population of South Africa
Evidence for differential S100 gene over-expression in psoriatic patients from genetically heterogeneous pedigrees
Intronic mutations outside of Alu -repeat-rich domains of the LDL receptor gene are a cause of familial hypercholesterolemia
Identification and characterisation of novel mammalian homologues of Drosophila polyhomeotic permits new insights into relationships between members of the polyhomeotic family
ELAC2 and prostate cancer risk in Afro-Caribbeans of Tobago Afro-Caribbeans
A novel locus for Meckel-Gruber syndrome, MKS3 , maps to chromosome 8q24
A novel approach for identifying candidate imprinted genes through sequence analysis of imprinted and control genes
Hereditary haemochromatosis: only 1% of adult HFE C282Y homozygotes in South Wales have a clinical diagnosis of iron overload
Genetic testing and risk assessment for spinal muscular atrophy (SMA)
Unusual deep intronic mutations in the COL4A5 gene cause X linked Alport syndrome
Sequence polymorphism at the human apolipoprotein AII gene ( APOA2 ): unexpected deficit of variation in an African-American sample African-American
Advances in Genetics, volume 14. Tay-Sachs Disease
MECP2 mutations in Rett syndrome adversely affect lymphocyte growth, but do not affect imprinted gene expression in blood or brain
Presence of fetal DNA in maternal plasma decades after pregnancy
Allelic and locus heterogeneity in autosomal recessive gelatinous drop-like corneal dystrophy
SNP identification, haplotype analysis, and parental origin of mutations in TSC2
Favourable mutation test outcomes for individuals at risk for Huntington disease change the perspectives of first-degree relatives
SOX10 mutations in chronic intestinal pseudo-obstruction suggest a complex physiopathological mechanism
Argininosuccinate lyase (ASL) deficiency: mutation analysis in 27 patients and a completed structure of the human ASL gene
October 2002