Journal of Medical Genetics - 2001

250 articles | Last updated: 2025-12-03 14:12:57
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T A T A T A T A
Heterogeneous tissue distribution of a mitochondrial DNA polymorphism in heteroplasmic subjects without mitochondrial disorders
Complex and segmental uniparental disomy (UPD): review and lessons from rare chromosomal complements
A community based study on intentions regarding predictive testing for hereditary breast cancer
A clinical study of patients with multiple isolated neurofibromas
Methylation matters
A dominant gene for developmental dyslexia on chromosome 3
Interpreting epidemiological research: blinded comparison of methods used to estimate the prevalence of inherited mutations in BRCA1
DEFECT 11 syndrome associated with agenesis of the corpus callosum
The small patella syndrome: description of five cases from three families and examination of possible allelism with familial patella aplasia-hypoplasia and nail-patella syndrome
Stable non-Robertsonian dicentric chromosomes: four new cases and a review
Myosin light chain mutations in familial hypertrophic cardiomyopathy: phenotypic presentation and frequency in Danish and South African populations
The importance of good images in using hair to screen for breast cancer
Interstitial deletion of chromosome 11 (q22.3-q23.2) in a boy with mild developmental delay
How microsatellite analysis can be exploited for subtelomeric chromosomal rearrangement analysis in mental retardation
Molecular characterisation of a new case of microphthalmia with linear skin defects (MLS)
A case of ring chromosome 2 with growth retardation, mild dysmorphism, and microdeletion of 2p detected using FISH
Twenty CAG repeats are sufficient to cause the SCA6 phenotype
Prevalence of mitochondrial DNA mutations in childhood/congenital onset non-syndromal sensorineural hearing impairment
Evaluation of the ELOVL4 gene in families with retinitis pigmentosa linked to the RP25 locus
Two further cases of WHS with unbalanced de novo translocation t(4;8) characterised by CGH and FISH
A region of homozygosity within 22q11.2 associated with congenital heart disease: recessive DiGeorge/velocardiofacial syndrome?
Alternative centromeric inactivation in a pseudodicentric t(Y;13)(q12;p11.2) translocation chromosome associated with extreme oligozoospermia
A heterozygous endothelin 3 mutation in Waardenburg-Hirschsprung disease: is there a dosage effect of EDN3/EDNRB gene mutations on neurocristopathy phenotypes?
Homoplasmic 3316Gright-arrowA in the ND1 gene of the mitochondrial genome: a pathogenic mutation or a neutral polymorphism?
Science or black magic?
A novel mutation in a family with non-syndromic sensorineural hearing loss that disrupts the newly characterised OTOF long isoforms
Correction for vol. 37, p. 897
Mitochondrial DNA inheritance in patients with deleted mtDNA
A novel acropectoral syndrome maps to chromosome 7q36
A syndrome of overgrowth and acromegaloidism with normal growth hormone secretion is associated with chromosome 11 pericentric inversion
Microdeletion in the FMR-1 gene: an apparent null allele using routine clinical PCR amplification
Rett syndrome and the MECP2 gene
Localisation of a gene for an autosomal recessive syndrome of macrocephaly, multiple epiphyseal dysplasia, and distinctive facies to chromosome 15q26
Laryngeal atresia, encephalocele, and limb deformities (LEL): a possible new syndrome
Molecular characterisation of four cases of intrachromosomal triplication of chromosome 15q11-q14
The expanding phenotype of laminin alpha2 chain (merosin) abnormalities: case series and review
Participation in preconceptional carrier couple screening: characteristics, attitudes, and knowledge of both partners
Population prevalence and estimated birth incidence and mortality rate for people with Prader-Willi syndrome in one UK Health Region
Two hits revisited again
Evidence of a founder effect for four cathepsin C gene mutations in Papillon-Lefevre syndrome patients
Clinical studies on submicroscopic subtelomeric rearrangements: a checklist
Role of TP53 P72R polymorphism in human papillomavirus associated premalignant laryngeal neoplasm
A cysteine substitution in the zona pellucida domain of alpha-tectorin results in autosomal dominant, postlingual, progressive, mid frequency hearing loss in a Spanish family
A unique form of autosomal dominant cataract explained by gene conversion between β-crystallin B2 and its pseudogene
B cell immunodeficiency, distal limb abnormalities, and urogenital malformations in a three generation family: a novel autosomal dominant syndrome?
A common founder for the 35delG GJB2 gene mutation in connexin 26 hearing impairment
Predictive genetic testing in children and adults: a study of emotional impact
Presymptomatic testing in myotonic dystrophy: genetic counselling approaches
Absence of learning difficulties in a hyperactive boy with a terminal Xp deletion encompassing the MRX49 locus
Non-invasive evaluation of arterial involvement in patients affected with Fabry disease
A frameshift mitochondrial complex I gene mutation in a patient with dystonia and cataracts: is the mutation pathogenic?
Molecular cytogenetic characterisation of a complex 46,XY,t(7;8;11;13) chromosome rearrangement in a patient with Moebius syndrome
Male breast cancer in Cowden syndrome patients with germline PTEN mutations
Persisting memories of Cyril Clarke in Baltimore
PTEN mutations are uncommon in Proteus syndrome
Leucodystrophy and oculocutaneous albinism in a child with an 11q14 deletion
Sulphate transporter gene mutations in apparently isolated club foot
Uniparental isodisomy for paternal 2p and maternal 2q in a phenotypically normal female with two isochromosomes, i(2p) and i(2q)
Novel mutations of <i>TMPRSS3</i> in four DFNB8/B10 families segregating congenital autosomal recessive deafness
Agenesis of cruciate ligaments and menisci causing severe knee dysplasia in TAR syndrome
The whole truth and nothing but the truth, but what is the truth?
Atypical clinical picture of the Nijmegen breakage syndrome associated with developmental abnormalities of the brain
MECP2 mutation in non-fatal, non-progressive encephalopathy in a male
Temperature sensitive acyl-CoA oxidase import in group A peroxisome biogenesis disorders
Autosomal dominant polycystic kidney disease unlinked to the PKD1 and PKD2 loci presenting as familial cerebral aneurysm
Coeliac disease in Williams syndrome
Submicroscopic subtelomeric 1qter deletions: a recognisable phenotype?
A missense mutation in the <i>SEDL</i> gene results in delayed onset of X linked spondyloepiphyseal dysplasia in a large pedigree
Distal arthrogryposis: a new type with distinct facial appearance and absent teeth
Screening families with endometrial and colorectal cancers for germline mutations
Mosaicism for 45,X cell line may accentuate the severity of spermatogenic defects in men with AZFc deletion
A new case of exomphalos, short limbs, and macrogonadism syndrome
Interstitial deletion of 3p22.2-p24.2: the first reported case
Mutations in<i>cis</i>can confound genotype-phenotype correlations in hypertrophic cardiomyopathy
Cryptic telomeric rearrangements in subjects with mental retardation associated with dysmorphism and congenital malformations
Sensitivity and predictive value of criteria for p53germline mutation screening
Identification of a novel psoriasis susceptibility locus at 1p and evidence of epistasis between PSORS1 and candidate loci
Cystic fibrosis patients with the 3272-26A&gt;G splicing mutation have milder disease than F508del homozygotes: a large European study
The mannose binding lectin gene influences the severity of chronic liver disease in cystic fibrosis
A novel mutation in the endothelin B receptor gene in a patient with Shah-Waardenburg syndrome and Down syndrome
Novel mutations of SOX10 suggest a dominant negative role in Waardenburg-Shah syndrome
Identification of interstitial maternal uniparental disomy (UPD) (14) and complete maternal UPD(20) in a cohort of growth retarded patients
Split hand/split foot malformation associated with sensorineural deafness, inner and middle ear malformation, hypodontia, congenital vertical talus, and deletion of eight microsatellite markers in 7q2
High frequencies of ICF syndrome-like pericentromeric heterochromatin decondensation and breakage in chromosome 1 in a chorionic villus sample
Eight years' experience of direct molecular testing for myotonic dystrophy in Wales
A partial deletion of the aspartoacylase gene is the cause of Canavan disease in a family from Mexico
Clustering and frequency of mutations in the retinal guanylate cyclase (GUCY2D) gene in patients with dominant cone-rod dystrophies
Identification of a large rearrangement of the<i>BRCA1</i> gene using colour bar code on combed DNA in an American breast/ovarian cancer family previously studied by direct sequencing
High resolution comparative genomic hybridisation in clinical cytogenetics
Ellis-van Creveld syndrome resulting from segmental uniparental disomy of chromosome 4
Risk perception and cancer worry: an exploratory study of the impact of genetic risk counselling in women with a family history of breast cancer
Chromosome 2 interstitial deletion (del(2)(q14.1q21)) associated with connective tissue laxity and an attention deficit disorder
Genetic analysis of the connexin-26 M34T variant: identification of genotype M34T/M34T segregating with mild-moderate non-syndromic sensorineural hearing loss
Cytogenetic and molecular study of a jumping translocation in a baby with Dandy-Walker malformation
Keratosis pilaris/ulerythema ophryogenes and 18p deletion: is it possible that the LAMA1 gene is involved?
A polymorphism in the gene for microsomal epoxide hydrolase is associated with pre-eclampsia
Molecular characterisation of a proximal chromosome 18q deletion
Peutz-Jeghers families unlinked to<i>STK11/LKB1</i> gene mutations are highly predisposed to primitive biliary adenocarcinoma
Detection of heterozygous SMN1 deletions in SMA families using a simple fluorescent multiplex PCR method
Wild type huntingtin reduces the cellular toxicity of mutant huntingtin in mammalian cell models of Huntington's disease
Variation of iron loading expression in C282Y homozygous haemochromatosis probands and sib pairs
Genetic association of an LBP-1c/CP2/LSF gene polymorphism with late onset Alzheimer's disease
Ectodermal dysplasias: a new clinical-genetic classification
The Asp1822Val variant of the APC gene is a common polymorphism without clinical implications
Genetic heterogeneity and exclusion of a modifying locus at 17p11.2-p11.1 in Finnish families with van der Woude syndrome
Analysis of the entire coding region of the cystic fibrosis transmembrane regulator gene in neonatal hypertrypsinaemia with normal sweat test
Adducted thumb-club foot syndrome in sibs of a consanguineous Austrian family Austrian
Thrombophilic polymorphisms in pre-eclampsia: altered frequency of the functional 98C&gt;T polymorphism of glycoprotein IIIa
Limited contribution of interchromosomal gene conversion to NF1 gene mutation
A common ancestor for COCH related cochleovestibular (DFNA9) patients in Belgium and The Netherlands bearing the P51S mutation
Mutation detection in long QT syndrome: a comprehensive set of primers and PCR conditions
A novel 3600+11.5 kb C&gt;G homozygous splicing mutation in a black African, consanguineous CF family black African
Functional characterisation of mitochondrial tRNATyr mutation (5877Gright-arrowA) associated with familial chronic progressive external ophthalmoplegia
Dutch patients with glycogen storage disease type II show common ancestry for the 525delT and del exon 18 mutations
Acromicric dysplasia: long term outcome and evidence of autosomal dominant inheritance
A novel germline mutation of the PTEN gene in a patient with macrocephaly, ventricular dilatation, and features of VATER association
Mutations in the glycine receptor α1 subunit (<i>GLRA1</i>) gene in hereditary hyperekplexia pedigrees: evidence for non-penetrance of mutation Y279C
Classification and genetic features of neonatal haemochromatosis: a study of 27 affected pedigrees and molecular analysis of genes implicated in iron metabolism
Trinucleotide repeat contraction: a pitfall in prenatal diagnosis of myotonic dystrophy
Cyril Clarke, Journal of Medical Genetics, and the foundation of clinical genetics
JMG Online: exploiting the potential of electronic publication and manuscript submission
Does Bardet-Biedl syndrome have a characteristic face?
De novo mutations in the 5' regulatory region of the Norrie disease gene in retinopathy of prematurity
Clinical and molecular characterisation of 80 patients with 5p deletion: genotype-phenotype correlation
Nijmegen breakage syndrome in a Dutch patient not resulting from a defect in <i>NBS1</i>
Supernumerary marker chromosome (1) of paternal origin and maternal uniparental disomy 1 in a developmentally delayed child
Association of a novel constitutional translocation t(1q;3q) with familial renal cell carcinoma
The −48 C/T polymorphism in the presenilin 1 promoter is associated with an increased risk of developing Alzheimer's disease and an increased Aβ load in brain
An atlas of preimplantation genetic diagnosis
VHL c.505 T&gt;C mutation confers a high age related penetrance but no increased overall mortality
Recessively inherited lower incisor hypodontia
Sponastrime dysplasia: presentation in infancy
Use of a set of highly polymorphic minisatellite probes for the identification of cryptic 1p36.3 deletions in a large collection of patients with idiopathic mental retardation
Germline mutation analysis of the transforming growth factor beta receptor type II (TGFBR2) and E-cadherin (CDH1) genes in early onset and familial colorectal cancer
SHOX point mutations in dyschondrosteosis
A novel missense mutation in the GTPase activating protein homology region of TSC2 in two large families with tuberous sclerosis complex
Cultural aspects of cancer genetics: setting a research agenda
A supernumerary marker chromosome with a neocentromere derived from 5p14right-arrowpter
Ocular malformations, postaxial polydactyly, and delayed intramembranous ossification: a new autosomal dominant condition
Congenital diaphragmatic hernia and interstitial deletion of chromosome 3
Hereditary and somatic DNA mismatch repair gene mutations in sporadic endometrial carcinoma
No evidence of allelic heterogeneity in the DYT1 gene of European patients with early onset torsion dystonia European patients
De novo recurrent germline mutation of the BRCA2 gene in a patient with early onset breast cancer
No evidence for mosaicism in Silver-Russell syndrome
Segregation of a totally skewed pattern of X chromosome inactivation in four familial cases of Rett syndrome without MECP2 mutation: implications for the disease
Disruption of one intra-chain disulphide bond in the carboxyl-terminal propeptide of the proalpha1(I) chain of type I procollagen permits slow assembly and secretion of overmodified, but stable procol
A case of Roberts syndrome described in 1737
An epidemiological study of Wolf-Hirschhorn syndrome: life expectancy and cause of mortality
Mutation and haplotype analysis of the CFTR gene in atypically mild cystic fibrosis patients from Northern Ireland
New problems in testing for Huntington's disease: the issue of intermediate and reduced penetrance alleles
<i>BRCA1</i> and<i>BRCA2</i> mutation status and cancer family history of Danish women affected with multifocal or bilateral breast cancer at a young age Danish; other populations
Distinct phenotypes distinguish the molecular classes of Angelman syndrome
Pure partial 7p trisomy including the TWIST, HOXA, and GLI3 genes
Hair roots as the ideal source of mRNA for genetic testing
beta1-adrenergic antagonists improve sleep and behavioural disturbances in a circadian disorder, Smith-Magenis syndrome
Familial non-medullary thyroid cancer in Iceland
Triplication of several PAR1 genes and part of the Homo sapiens specific Yp11.2/Xq21.3 region of homology in a 46,X,t(X;Y)(p22.33;p11.2) male with schizophrenia
Association between the defective Pro369Ser mutation and in vivo intrahepatic alpha1-antitrypsin accumulation
Oncology nurse training in cancer genetics
Clinical heterogeneity in lymphoedema-distichiasis with FOXC2 truncating mutations
GATA3 abnormalities and the phenotypic spectrum of HDR syndrome Japanese families
SHOX haploinsufficiency and overdosage: impact of gonadal function status
Prenatal testing for Huntington's disease: experience within the UK 1994-1998
Defining the genetic contribution of type 2 diabetes mellitus
Inherited Disorders and their Genes in Different European Populations European Populations
Comparative Genomics.
A distinct splice form of APC is highly expressed in neurones but not commonly mutated in neuroepithelial tumours
Anderson-Fabry disease: clinical manifestations and impact of disease in a cohort of 60 obligate carrier females
Psychological studies in Huntington's disease: making up the balance
Haptoglobin genotype as a risk factor for postmenopausal osteoporosis
Suggestive linkage of situs inversus and other left-right axis anomalies to chromosome 6p
Breakpoint analysis of Turner patients with partial Xp deletions: implications for the lymphoedema gene location
Wolfram syndrome: a clinical and molecular genetic analysis
High frequency of the ApoB-100 R3500Q mutation in Bulgarian hypercholesterolaemic subjects
Atypical HNPCC owing to MSH6 germline mutations: analysis of a large Dutch pedigree
Phenotypic heterogeneity of CYP1B1: mutations in a patient with Peters' anomaly
A novel 3' mutation in the APC gene in a family presenting with a desmoid tumour
Molecular characterisation of a supernumerary ring chromosome in a patient with VATER association
Multiple metachromatic leucodystrophy alleles in an unaffected subject: a case of dispermic chimaerism
E-cadherinis not frequently mutated in hereditary gastric cancer
Frequency of mitochondrial transfer RNA mutations and deletions in 225 patients presenting with respiratory chain deficiencies
Silver-Russell syndrome: a dissection of the genetic aetiology and candidate chromosomal regions
A Chinese adult onset type II citrullinaemia patient with 851del4/1638ins23 mutations in the SLC25A13 gene
Maternal gene effect in neurofibromatosis 2: fact or artefact?
Mutation analysis of TBX1 in non-deleted patients with features of DGS/VCFS or isolated cardiovascular defects
MET mutation and familial gastric cancer
The spectrum and evolution of phenotypic findings in PTEN mutation positive cases of Bannayan-Riley-Ruvalcaba syndrome
Familial medullary thyroid carcinoma and prominent corneal nerves associated with the germline V804M and V778I mutations on the same allele of RET
Low prevalence of SPINK1 gene mutations in adult patients with chronic idiopathic pancreatitis
Pseudoxanthoma elasticum: evidence for the existence of a pseudogene highly homologous to the ABCC6 gene
A broad spectrum of clinical presentations in congenital disorders of glycosylation I: a series of 26 cases
The spastic paraplegia SPG10 locus: narrowing of critical region and exclusion of sodium channel gene SCN8A as a candidate
Surveillance or surgery? A description of the factors that influence high risk premenopausal women's decisions about prophylactic oophorectomy
Evidence of somatic mosaicism for a MECP2 mutation in females with Rett syndrome: diagnostic implications
Molecular genetic heterogeneity in autosomal dominant drusen
Anderson-Fabry disease: clinical manifestations and impact of disease in a cohort of 98 hemizygous males
Increased risk of sensorineural hearing loss and migraine in patients with a rare mitochondrial DNA variant 4336A&gt;G in tRNA<sup>Gln</sup>
Genotype-phenotype correlation in hereditary multiple exostoses
A transmitted deletion of 2q13 to 2q14.1 causes no phenotypic abnormalities
Satellites on the terminal short arm of chromosome 12 (12ps), inherited through several generations in three families: a new variant without phenotypic effect
Comprehension of cancer risk one and 12 months after predictive genetic testing for hereditary non-polyposis colorectal cancer
A silent mutation in exon 14 of the APC gene is associated with exon skipping in a FAP family
Novel mutations of FOXP3 in two Japanese patients with immune dysregulation, polyendocrinopathy, enteropathy, X linked syndrome (IPEX)
Segmental uniparental isodisomy (UPD) for 2p16 without clinical symptoms: implications for UPD and other genetic studies of chromosome 2
Recurrent mutations in the deafness gene GJB2 (connexin 26) in British Asian families
Diaphragmatic hernia, hydrocephalus, and cardiac malformations in four pregnancies of a non-consanguineous couple
Predisposing chromosome for spinocerebellar ataxia type 6 (SCA6) in Japanese
Pregnancy outcome and long term prognosis in 868 children born after second trimester amniocentesis for maternal serum positive triple test screening and normal prenatal karyotype
A simple non-isotopic method to show pitfalls during mutation analysis of the glucocerebrosidase gene
Maternally inherited duplication of the possible imprinted 14q31 region
Kufor-Rakeb syndrome, pallido-pyramidal degeneration with supranuclear upgaze paresis and dementia, maps to 1p36
Mutations in SURF1 are not specifically associated with Leigh syndrome
Founder effect in multiple endocrine neoplasia type 1 (MEN 1) in Finland
Angelman syndrome phenotype associated with mutations in MECP2, a gene encoding a methyl CpG binding protein
A G339R mutation in the CTNS gene is a common cause of nephropathic cystinosis in the south western Ontario Amish Mennonite population Amish Mennonite population
Correction for vol. 37, p. 241
Submicroscopic deletions of the APC gene: a frequent cause of familial adenomatous polyposis that may be overlooked by conventional mutation scanning
Hereditary spastic paraplegia linked to chromosome 14q11-q21: reduction of the SPG3 locus interval from 5.3 to 2.7 cM
Fibrinogen genotypes (alpha and beta) are associated with plasma fibrinogen levels in Chinese Chinese
A comparison of methods for gene dosage analysis in HMSN type 1
Potential mapping of corneal dermoids to Xq24-qter
Identification of two novel mutations in the CACNA1A gene responsible for episodic ataxia type 2
The androgen receptor and DXS15-134 markers show a high rate of discordance for germline X chromosome inactivation
GDNF as a candidate modifier in a type 1 neurofibromatosis (NF1) enteric phenotype
Molecular and clinical study of two myotonic dystrophy homozygotes
Clinical and cytogenetic characterisation of a patient with Down syndrome resulting from a 21q22.1-&gt;qter duplication
Detection of 11 germline inactivating TP53 mutations and absence of TP63 and HCHK2 mutations in 17 French families with Li-Fraumeni or Li-Fraumeni-like syndrome
Identification of a new TWIST mutation (7p21) with variable eyelid manifestations supports locus homogeneity of BPES at 3q22
Deletion 22q11 syndrome: acknowledging a lost eponym as we say farewell to an acronym
A majority of fragile X males with methylated, full mutation alleles have significant levels of FMR1 messenger RNA
Mosaic terminal del(19)(q13.33:) in a girl with seizures and mental retardation
Autosomal recessive non-syndromic hearing loss in the Lebanese population: prevalence of the 30delG mutation and report of two novel mutations in the connexin 26 (GJB2) gene Lebanese population
Maternal uniparental isodisomy 11q13right-arrowqter in a dysmorphic and mentally retarded female with partial trisomy mosaicism 11q13right-arrowqter
Homozygosity for a splice site mutation of the COL1A2 gene yields a non-functional proalpha2(I) chain and an EDS/OI clinical phenotype
Development and application of linkage analysis in genetic diagnosis of familial hypertrophic cardiomyopathy
Anauxetic dysplasia, a spondylometaepiphyseal dysplasia with extreme dwarfism
Annual Review of Genomics and Human Genetics
De novo terminal deletion of chromosome 15q26.1 characterised by comparative genomic hybridisation and FISH with locus specific probes
Identification of a transcriptionally compromised allele ofc-MYC in a North American family
Decoding Darkness - The Search for the Genetic Causes of Alzheimer's Disease.
Cyril Astley Clarke
Interaction of coding region mutations and the Gilbert-type promoter abnormality of the UGT1A1 gene causes moderate degrees of unconjugated hyperbilirubinaemia and may lead to neonatal kernicterus
Detection of a large TBX5 deletion in a family with Holt-Oram syndrome
A novel mutation and novel features in Nijmegen breakage syndrome
Do patients with maternal uniparental disomy for chromosome 7 have a distinct mild Silver-Russell phenotype?
Genetic analysis of the connexin-26 M34T variant
Hirschsprung disease, associated syndromes, and genetics: a review
Analysis of the SRY gene in Turner syndrome patients with Y chromosomal material
Six novel mutations in the PRF1 gene in children with haemophagocytic lymphohistiocytosis
An atlas of preimplantation genetic diagnosis