Clinical Genetics - 2002

199 articles | Last updated: 2025-12-03 14:12:56
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Article Title Cauc. White Euro. Other Phrases Used
T A T A T A T A
The 27Glu polymorphism of the β<sub>2</sub>‐adrenergic receptor gene interacts with physical activity influencing obesity risk among female subjects
MURCS association with duplicated thumb
Interpreting three new and unique <i>BRCA1</i> mutations in an Ashkenazi Jewish patient Ashkenazi Jewish
Deafness resulting from mutations in the GJB2 (connexin 26) gene in Brazilian patients
A patient homozygous for the <i>SCA6</i> gene with retinitis pigmentosa
Hotspots
Errata
Hotspots
Hotspots
Cryptic chromosome rearrangements detected by subtelomere assay in patients with mental retardation and dysmorphic features
High prevalence of I179S mutation in patients with late‐onset metachromatic leukodystrophy
Man and mouse: A comparison between two genomes
Schizophrenia genetics
Inducible nitric oxide synthase gene and diabetic retinopathy in Asian Indian patients Asian Indian population; Northern Irish population
Identification and characterization of mutations underlying Sanfilippo syndrome type A (mucopolysaccharidosis type IIIA)
Non‐syndromic autosomal‐dominant deafness
A novel frameshift founder mutation in the cytochrome P450 1B1 (CYP1B1) gene is associated with primary congenital glaucoma in Morocco
Keeping an eye on splicing; A FOX speaks; An aneuploidy surprise
A familial cryptic subtelomeric deletion 12p with variable phenotypic effect
Mutation in the <i>FGFR2</i> gene in a Taiwanese patient with Beare–Stevenson cutis gyrata syndrome
Setting sights on inherited retinal disease
Type III hyperlipoproteinema with apolipoprotein E2/2 genotype in Japan
Esophageal and duodenal atresia in a girl with a 12q24.3‐qter deletion
Living with Marfan syndrome III. Quality of life and reproductive planning
Haplotype analysis of a <i>BRCA1</i>: 185delAG mutation in a Chilean family supports its Ashkenazi origins Ashkenazi Jewish; Jewish; non‑Jewish Chilean; Chilean
Apolipoprotein molecular variation in Moroccan Berbers: pentanucleotide (TTTTA)n repeat in the LPA gene and APOE‐C1‐C2 gene cluster Europeans; Sub‐Saharans; Berbers; Mediterraneans; European pattern; Sub‐Saharan influence
A 4‐Mb critical region for intrauterine growth retardation at 15q26
Ring syndrome caused by ring chromosome 7 without loss of subtelomeric sequences
X‐linked recessive Menkes disease: identification of partial gene deletions in affected males
<i>FOXC2</i> truncating mutation in distichiasis, lymphedema, and cleft palate
Fertility and pregnancy outcome in Danish women with Turner syndrome
Distribution of HFE C282Y and H63D mutations in the Balearic Islands (NE Spain) "ethnic groups"; "populations" (populations of the Balearic Islands); "descendants of Majorcan Jews
Molecular cytogenetic analysis of a Xp21.3‐pter deletion in a family with normal and short stature
Gene/environment causes of cleft lip and/or palate
Inherited disorders of cholesterol biosynthesis
Endothelial nitric oxide synthase gene polymorphisms in Fabry's disease
Mutation in the cartilage‐derived morphogenetic protein‐1 (CDMP1) gene in a kindred affected with fibular hypoplasia and complex brachydactyly (DuPan syndrome)
Novel skeletal muscle ryanodine receptor mutation in a large Brazilian family with malignant hyperthermia
The TaqIB and −629C&gt;A polymorphisms at the cholesteryl ester transfer protein locus: associations with lipid levels in a multiethnic population. The 1998 Singapore National Health Survey
<i>De novo</i> supernumerary ring chromosome 7: first report of a non‐mosaic patient and review of the literature
A 4q21‐q22 deletion in a girl with severe growth retardation
IL‐4 receptor alpha chain genetic polymorphism and total IgE levels in the English population: two‐locus haplotypes are more informative than individual SNPs English population (Oxford district)
Two more genes involved in recental retardation in an ever x‐panding repertoire of proteins and pathways
Family issues in a psychoeducation group for women with a <i>BRCA</i> mutation
Developing a quality scoring system for epidemiological surveys of genetic disorders
Autosomal spots that mark the ‘X’
Hair as a diagnostic tool in dysmorphology
Genetic heterogeneity in Malattia Leventinese
Sib‐pairs in multifactorial disorders: the sib‐similarity problem
A novel point mutation of the androgen receptor (F804L) in an Egyptian newborn with complete androgen insensitivity associated with congenital glaucoma and hypertrophic pyloric stenosis
Very low incidence of microsatellite instability in rectal cancers from families at risk for HNPCC
A case of <i>de novo</i> distal duplication of chromosome 15
A prospective evaluation of the angiotensin‐converting enzyme <i>D/I</i> polymorphism and left ventricular remodeling in the ‘Healing and Early Afterload Reducing Therapy’ Study
Telomere‐specific fluorescence <i>in situ</i> hybridization analysis of couples with five or more recurrent miscarriages
Effects of apolipoprotein A‐IV genotype on glucose and plasma lipoprotein levels
Homozygosity for the V37I Connexin 26 mutation in three unrelated children with sensorineural hearing loss Ashkenazi Jews; Japanese individuals; Philippine ancestry; Chinese and Cambodian background; Chinese
Tubulopathy, endocrinopathies and encephalomyopathy in a child with a novel large‐scale mitochondrial DNA deletion
Interdependent effect of angiotensin‐converting enzyme and platelet‐activating factor acetylhydrolase gene polymorphisms on the progression of immunoglobulin A nephropathy
Anticipation in hereditary breast cancer
Normal levels of soluble transferrin receptor in Friedreich ataxia
Familial hypocalciuric hypercalcaemia in a large family with neurofibromatosis 1
Association of angiotensin‐converting‐enzyme gene polymorphism with the depressor response to mild exercise therapy in patients with mild to moderate essential hypertension
Pathogenesis of hereditary tumors: beyond the “two‐hit” hypothesis
Tetraploidy in a 26‐month‐old girl (cytogenetic and molecular studies)
Genetic control of caudal development
History of genetics through philately: Sir William Osler (1849–1919) and the Osler–Weber–Rendu syndrome
An Italian severe Salla disease variant associated with a SLC17A5 mutation earlier described in infantile sialic acid storage disease Finnish population; French-Canadian patients; patient's ethnic origin
Towards an understanding of the molecular pathogenesis of autosomal‐recessive polycystic kidney disease (ARPKD): identification of a novel protein, fibrocystin
Influence of the angiotensin‐converting enzyme gene insertion/deletion polymorphism on lipoprotein/lipid response to gemfibrozil
A Japanese patient with cerebrotendinous xanthomatosis has different mutations within two functional domains of <i>CYP27</i>
<i>ABCA1</i> regulatory variants influence coronary artery disease independent of effects on plasma lipid levels
A low prevalence of the C677T mutation in the methylenetetrahydrofolate reductase gene in Asian Indians Asian Indians
Identification of a dup(5)(p15.3) by multicolor banding
HotSpots
Spinal muscular atrophy in black South Africans: concordance with the universal SMN1 genotype
Three novel mutations of the PKD1 gene in Korean patients with autosomal dominant polycystic kidney disease European and American populations; Western population
A second kindred linked to DFNA20 (17q25.3) reduces the genetic interval
Fabry disease: a functional and anatomical study of cardiac manifestations in 20 hemizygous male patients
‘Severe’ Prader–Willi syndrome with a large deletion of chromosome 15 due to an unbalanced t(15,22)(q14;q11.2) translocation
Genetic Services
Autosomal dominant isolated velopharyngeal insufficiency
Reproductive decisions of parents of children with metabolic disorders
HotSpots
Evaluation of the needs of spouses of female carriers of mutations in <i>BRCA1</i> and <i>BRCA2</i>
Polymorphisms at the <i>SRBI</i> locus are associated with lipoprotein levels in subjects with heterozygous familial hypercholesterolemia
Exclusion of candidate gene loci for adult onset primary open‐angle glaucoma in a genetically isolated population
Homozygosity in Huntington's disease: new ethical dilemma caused by molecular diagnosis
Apolipoprotein E gene polymorphism in cerebrovascular disease: a case–controlstudy Asian Indians
657del5 mutation in the NBS1 gene is associated with Nijmegen breakage syndrome in a Turkish family Slav populations; Turkish population
Supernumerary marker chromosomes derived from chromosome 15: analysis of 32 new cases
Homologous telomere association of 19q in a female with premature ovarian failure
Genetic landmarks through philately: Luís Morquio 1867–1935
Characterization of partial trisomy 9p due to insertional translocation by chromosomal (micro)FISH
Genetic technologies, health care policy and the patent bargain
A previously undescribed nonsense mutation of the <i>HFE</i> gene
Effects of apolipoprotein A‐I genetic variations on plasma apolipoprotein, serum lipoprotein and glucose levels
The relationship between plasma t‐PA and PAI‐1 levels is dependent on epistatic effects of the<i>ACE I/D</i>and<i>PAI‐1 4G/5G</i>polymorphisms African Americans
Increased sensitivity to 4‐chloro‐m‐cresol and caffeine in primary myotubes from malignant hyperthermia susceptible individuals carrying the ryanodine receptor 1 Thr2206Met (C6617T) mutation
Craniofacial features in Cohen syndrome: an anthropometric and cephalometric analysis of 14 patients
A study of the influence of different genotypes on the physical and behavioral phenotypes of children and adults ascertained clinically as having PWS
Acute intermittent porphyria in Sweden. Molecular, functional and clinical consequences of some new mutations found in the porphobilinogen deaminase gene
The novel R75Q mutation in the <i>GJB2</i> gene causes autosomal dominant hearing loss and palmoplantar keratoderma in a Turkish family
A new case of dup(3q) syndrome due to a pure duplication of 3qter
Involvement of a palindromic chromosome 22‐specific low‐copy repeat in a constitutional t(X; 22)(q27;q11)
X‐linked mental retardation: vanishing boundaries between non‐specific (MRX) and syndromic (MRXS) forms
Standardization of PCR amplification for fragile X trinucleotide repeat measurements*
Association of a polymorphism of the ecNOS gene with myocardial infarction in a subgroup of Turkish MI patients
Communicating a new gene vital for speech and language
Complete screening of the <i>CFTR</i> gene in Argentine cystic fibrosis patients heterogeneous ethnic origin of the Argentine population
Familial adenomatous polyposis in two Black South African families Black; indigenous Black; African population; Xhosa; Zulu
Cardiomyopathy in congenital complete lipodystrophy
Quantification of CFTR splice variants in adults with disseminated bronchiectasis, using the TaqMan fluorogenic detection system
X‐linked recessive Menkes disease: carrier detection in the case of a partial gene deletion
The association of cholesteryl ester transfer protein polymorphism with high‐density lipoprotein cholesterol and coronary artery disease in Koreans Koreans; other ethnic groups
Familial Mediterranean fever: the potential for misdiagnosis of E148V using the E148Q usual RFLP detection method
HotSpots
Acanthocytosis in a patient with homozygous familial hypobetalipoproteinemia due to a novel <i>APOB</i> splice site mutation
A CGH study of 27 patients with CHARGE association
<i>RMRP</i> gene sequence analysis confirms a cartilage‐hair hypoplasia variant with only skeletal manifestations and reveals a high density of single‐nucleotide polymorphisms Finnish
A patient with treatment‐resistant schizophrenia and cytochrome P4502D6 gene duplication
Re‐engineering dystrophin for gene therapy of Duchenne muscular dystrophy
Genetic landmarks through philately: Woodrow Wilson ‘Woody’ Guthrie and Huntington disease
LDL receptor mutation genotype and vascular disease phenotype in heterozygous familial hypercholesterolaemia
Service provision of complex mutation analysis: a technical and economic appraisal using dystrophin point mutation analysis as an example
Gene conversion (655G splicing mutation) and the founder effect (Gln318Stop) contribute to the most frequent severe point mutations in congenital adrenal hyperplasia (21‐hydroxylase deficiency) in the Spanish population
Genotyping of Israeli infertile men with idiopathic oligozoospermia
Genetic polymorphisms of the apolipoprotein A‐IV in a Greek population and their relation to plasma lipid and lipoprotein levels Greek population
Losing your inhibitions: a mutation in the α1 subunit of the GABA<sub>A</sub> receptor causes autosomal dominant juvenille myoclonic epilepsy
The A1555G mtDNA mutation in Danish hearing‐impaired patients: frequency and clinical signs Spanish; Asian; Danish
Diabetic flies? Using <i>Drosophila melanogaster</i> to understand the causes of monogenic and genetically complex diseases
An unstable dicentric Robertsonian translocation in a markedly discordant twin
SCA8 in the Spanish population including one homozygous patient Spanish population
Genetic origins in a South American clefting population Amerindian; Native American; African; Spanish and Portuguese
Partial Xp duplication in a girl with dysmorphic features: the change in replication pattern of late‐replicating dupX chromosome
The complexities of ocular genetics
Genetics of population isolates
Novel neuropeptide Y1 and Y5 receptor gene variants: associations with serum triglyceride and high‐density lipoprotein cholesterol levels
ATP binding cassette G5 C1950G polymorphism may affect blood cholesterol concentrations in humans
A novel mutation (N32K) of GLUT2 gene in a Japanese patient with Fanconi–Bickel syndrome
Pharmacogenomics: the future of drug therapy
Germline mutations in the <i>PTEN</i> gene in Israeli patients with Bannayan–Riley–Ruvalcaba syndrome and women with familial breast cancer
Enteroviruses prefer the dystrophin‐deficient heart
Frequency of the thermolabile variant C677T in the MTHFR gene and lack of association with neural tube defects in the State of Yucatan, Mexico
Klinefelter syndrome is a common cause for mental retardation of unknown etiology among prepubertal males
Identification of two novel mutations, L105R and C342R, in Type I Gaucher disease
Mutations in the X‐linked spondyloepiphyseal dysplasia tarda (SEDL) coding sequence are not a common cause of early primary osteoarthritis in men
Epigenetic contributors to the discordance of monozygotic twins
α1‐Antitrypsin null alleles: evidence for the recurrence of the L353fsX376 mutation and a novel G→A transition in position +1 of intron IC affecting normal mRNA splicing
Lack of association between methylenetetrahydrofolate reductase (MTHFR) C677T and ischaemic heart disease (IHD): family‐based association study in a Spanish population Spanish population
Genetic and clinical analysis of spinocerebellar ataxia type 8 repeat expansion in Yugoslavia
Sleep disturbance in mucopolysaccharidosis type III (Sanfilippo syndrome): a survey of managing clinicians
Down with mutant <i>GATA1</i>
The effect of six polymorphisms in the Apolipoprotein B gene on parameters of lipid metabolism in a Danish population
An exonic mutation of the GH‐1 gene causing familial isolated growth hormone deficiency type II
<i>DGAT1</i> promoter polymorphism associated with alterations in body mass index, high density lipoprotein levels and blood pressure in Turkish women Turkish
Mutation analysis of the STK11/LKB1 gene and clinical characteristics of an Australian series of Peutz–Jeghers syndrome patients
PROMM and deafness: exclusion of ZNF9 as the disease gene in DFNA18 suggests a polygenic origin of the PROMM/DM2 phenotype
Hotspots
The E326K mutation and Gaucher disease: mutation or polymorphism?
Participation rates of Ashkenazi Jews in a colon cancer community‐based screening/prevention study Ashkenazi Jews
Cognitive function in Coffin–Lowry syndrome African‐American
Development and diseases of the pancreas
A comparison of combinatorial partitioning and linear regression for the detection of epistatic effects of the<i>ACE I/D</i>and<i>PAI‐1 4G/5G</i>polymorphisms on plasma PAI‐1 levels African Americans
Sibship stability of genotype and phenotype in myotonic dystrophy
Comparison of two different protocols of neonatal screening for cystic fibrosis
Hereditary motor neuron disease caused by mutations in the ALS2 gene: ‘The long and the short of it’
Analysis of the two common alpha‐1‐antitrypsin deficiency alleles PiMS and PiMZ as modifiers of <i>Pseudomonas aeruginosa</i> susceptibility in cystic fibrosis
Modeling classic female Rett Syndrome in male mice
HIP1 as a marker of aggressive prostate cancer
The changing survival profile of people with Down's syndrome: implications for genetic counselling
Association between G<sup>−308</sup>A polymorphism of the tumor necrosis factor‐α gene and 24‐hour ambulatory blood pressure values in type 1 diabetic adolescents Hungarian reference population
Prevalence and ethnic differences of autosomal‐dominant cerebellar ataxia in Singapore ethnic Malay; ethnic Indian; ethnic Chinese; Malay race; ancestry of Singaporeans; ethnic difference
Non‐syndromic X‐linked mental retardation associated with a missense mutation (P312L) in the FGD1 gene
Two novel mutations in the sterol 27‐hydroxylase gene causing cerebrotendinous xanthomatosis
At last – atlastin provides new insight into spastic paraplegia
Presence of the d8/17 B‐cell marker in children with rheumatic fever in Israel
Facial clefting in an Arab town in Israel
How many phenotypes in the DTDST family chondrodysplasias?
Bronchomalacia associated with pulmonary atresia, ventricular septal defect and major aortopulmonary collateral arteries, and chromosome 22q11.2 deletion
Diploid/triploid mosaicism in dysmorphic patients
A predominant increase in the APC gene isoform with exon 9a in a case of attenuated familial adenomatous polyposis
Errata
CALL FOR ABSTRACTS
Erratum
An SEDL gene mutation in a Japanese kindred of X‐linked spondyloepiphyseal dysplasia tarda Japanese
New mutations in the <i>CBFA1</i> gene in two Mexican patients with cleidocranial dysplasia Mexican
Recurrent 22q11.2 deletion in a sibship suggestive of parental germline mosaicism in velocardiofacial syndrome
Early development of megarectum in myotonic dystrophy
Inherited hypertrichoses
<i>De novo MECP2</i> frameshift mutation in a boy with moderate mental retardation, obesity and gynaecomastia
Angiotensinogen genotype, plasma protein and mRNA concentration in isolated systolic hypertension
Familial pericentric inversion of chromosome 4: inv(4)(p16.1q12)
Developmental Genome Anatomy Project (DGAP)
Erratum
Should the chromosome region 15q11q13 be tested systematically by FISH in the case of an autistic‐like syndrome?
Apolipoprotein AI promoter variant in blood pressure determination
Attention‐deficit/hyperactivity disorder (ADHD): feasibility of linkage analysis in a genetic isolate using extended and multigenerational pedigrees
Familial lethal skeletal dysplasia with cloverleaf skull and multiple anomalies of brain, eye, face and heart: a new autosomal recessive multiple congenital anomalies syndrome Arabic‐Muslim
<i>MECP2</i> mutations in Swedish Rett syndrome clusters