Clinical Genetics - 2001

195 articles | Last updated: 2025-12-03 14:12:56
Caucasian
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White
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European
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Other
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Article Title Cauc. White Euro. Other Phrases Used
T A T A T A T A
Treatment of severe hypercholesterolemia in apolipoprotein E‐deficient mice by intramuscular injection of plasmid DNA
Hammerhead ribozyme as a therapeutic agent for hyperlipidemia: Production of truncated apolipoprotein B and hypolipidemic effects in a dyslipidemia murine model
Prolonged correction of hyperlipidemia in mice with familial hypercholesterolemia using an adeno‐associated viral vector expressing very‐low‐density lipoprotein receptor
Long‐term reversal of hypercholesterolemia in low‐density lipoprotein receptor (LDLR)‐deficient mice by adenovirus‐mediated LDLR gene transfer combined with CD154 blockade
Silencing the quiet
A phosphatase mutation implicated in multiple sclerosis
HotSpots
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Real facts from artificial chromosomes
Fetal cells in maternal blood
Prevalence of founder <i>BRCA1</i> and <i>BRCA2</i> mutations in unselected French Canadian women with breast cancer French Canadian; ethnicity/country of origin; French Canadian population
Two unbalanced translocations involving a common 6p25 region in two XY female patients
Identification of two <i>de novo</i> partial trisomies by comparative genomic hybridization
Homozygosity for a 4‐bp deletion in a patient with Wolfram syndrome suggesting possible phenotype and genotype correlation
Multicultural education and genetic counseling
A few more pieces of the DM puzzle
Novel molecular defects in the androgen receptor gene of Mexican patients with androgen insensitivity Mexican patients
Co‐occurrence and contribution of Fabry disease and Klippel–Trénaunay–Weber syndrome to a patient with atypical skin lesions
Comparative genomic hybridisation in mentally retarded patients with dysmorphic features and a normal karyotype
‘The VACTERL association: lessons from the Sonic hedgehog pathway’
Mutations of the <i>NOG</i> gene in individuals with proximal symphalangism and multiple synostosis syndrome
A logistic regression model for measuring gene–longevity associations Italian
Late‐onset ornithine transcarbamylase deficiency in two families with different mutations in the same codon
Human genetic research, DNA banking and consent: a question of ‘form’?
Phenotype variability of two FAP families with an identical <i>APC</i> germline mutation at codon 1465: a potential modifier effect?
Molecular genetic analysis of the cystathionine β<b>‐</b>synthase gene in Portuguese homocystinuria patients: three novel mutations Portuguese
Lipoprotein(a) as a risk factor for maternal cardiovascular disease mortality in kindreds with familial combined hyperlipidemia or familial hypertriglyceridemia
Neurodegeneration: iron weighs in
Evaluation for sleep apnea in patients with Ehlers–Danlos syndrome and Marfan: a questionnaire study
Segmental uniparental disomy of 7q31‐qter is rare in Silver–Russell syndrome
Frequency of the 35delG mutation in the connexin 26 gene in Turkish hearing‐impaired patients
Vietnamese sisters with Grebe syndrome on a TV program in Japan
The expanding world of spinocerebellar ataxia: pentanucleotide repeats expand in SCA10
Four half‐siblings with infantile myrofibromatosis: a case for autosomal‐recessive inheritance
The use of intragenic polymorphisms in determination of the genomic relevance of whole‐exon deletions in <i>MLH1</i> and <i>MSH2</i>
Towards a vaccine for Alzheimer disease
Transgenic monkey raises hope for primate models of human diseases
A nonsense mutation in <i>TRPS1</i> in a Japanese family with tricho‐rhino‐phalangeal syndrome type I
Cryptic duplication of 21q in an individual with a clinical diagnosis of Down syndrome
When p63 goes awry: SAM domain mutations
Risk of obstetric cholestasis in sisters of index patients
Discoveries in Charcot–Marie–Tooth disease, Crohn's disease and Bardet–Biedl syndrome 4
Digesting genetic information in gastric cancers
Pulling roots of the thorny chorea‐acanthocytosis
Genetic landmarks through philately – Charles Robert Darwin
Molecular basis of Mucopolysaccharidosis type II in Portugal: identification of four novel mutations
When body segmentation goes wrong
To test or not to test: an ethical conflict with presymptomatic testing of individuals at 25% risk for Huntington's disorder
Human <i>HOX</i> gene mutations
Split hand/split foot malformation with hearing loss: first report of families linked to the SHFM1 locus in 7q21
Retinal detachment and cataract, facial dysmorphism, generalized osteoporosis, immobile spine and platyspondyly in a consanguinous kindred – a possible new syndrome
Two novel mutations in a purine nucleoside phosphorylase (PNP)‐deficient patient
Alpha‐1‐antitrypsin PI phenotypes S and Z in Europe: an analysis of the published surveys European populations; Europe
Consanguinity and its relevance to clinical genetics
Waardenburg syndrome type 3 (Klein–Waardenburg syndrome) segregating with a heterozygous deletion in the paired box domain of <i>PAX3</i>: a simple variant or a true syndrome?
Prenatal diagnosis and characterization of an unbalanced whole arm translocation resulting in monosomy for 18p
Recurrent mutations in the <i>COL1A2</i> gene in patients with osteogenesis imperfecta
History of genetics through philately – Carl Linnaeus (Carl von Linné)
Apolipoprotein E polymorphism and lipid levels differ by gender and family history of diabetes: the Rancho Bernardo Study
Living with Marfan syndrome II. Medication adherence and physical activity modification
Pyruvate kinase deficiency: prevalence of the 1456C→T mutation in the Portuguese population Portuguese population
Grebe syndrome in Vietnamese sisters: not Agent Orange
The molecular basis of cystic fibrosis in South Africa South Africans of European origin; coloured and black
Three‐way unbalanced translocation in a mildly dysmorphic mentally retarded child
Shakespeare as a geneticist<sup>1</sup>
Identification of the Arg1086His mutation in the alpha subunit of the voltage‐dependent calcium channel (CACNA1S) in a North American family with malignant hyperthermia
Evidence for locus heterogeneity in the Camurati–Engelmann (DPD1) Syndrome
Fibrillin‐1 (<i>FBN1</i>) gene frameshift mutations in Marfan patients: genotype–phenotype correlation
Both alleles of the M235T polymorphism of the angiotensinogen gene can be a risk factor for myocardial infarction
Further evidence for linkage of low–mid frequency hearing impairment to the candidate region on chromosome 4p16.3
A <i>de novo</i> mutation (<i>R279C</i>) in the <i>P63</i> gene in a patient with EEC syndrome
Shutting off mammalian gene expression the easy way
Lack of association of the common TaqIB polymorphism in the cholesteryl ester transfer protein gene with angiographically assessed coronary atherosclerosis
The frequency of common mutations among patients with mucopolysaccharidosis types I, II and IIIA diagnosed in Austria over the last 17 years
A family with a grand‐maternally derived interstitial duplication of proximal 15q
The beta‐globin locus control region versus gene therapy vectors: a struggle for expression
An HphI polymorphism in the E‐selectin gene is associated with premature coronary artery disease
Advances in the detection of chromosomal aberrations using spectral karyotyping
The ethics of benefit sharing
Severe valvular and aortic arch calcification in a patient with Gaucher's disease homozygous for the D409H mutation<sup>1</sup> Palestinian
Maternal component in the familial aggregation of hypertension
Determination of a positive malignant hyperthermia (MH) disposition without the <i>in vitro</i> contracture test in families carrying the RYR1 Arg614Cys mutation German MH families
Family‐based transmission disequilibrium test (TDT) and case–control association studies reveal surfactant protein A (SP‐A) susceptibility alleles for respiratory distress syndrome (RDS) and possible blacks
Hereditary breast/ovarian cancer – pitfalls in genetic counseling Ashkenazi Jewish population; Jewish Ashkenazi population; Ashkenazi individuals; Ashkenazi women
Right place‐right time; real time protein–protein interactions
Distribution of α1‐antitrypsin PI S and PI Z frequencies in countries outside Europe: a meta‐analysis countries outside Europe; European meta‐analysis
Familial Down syndrome: evidence supporting cytoplasmic inheritance
Mutation in the connexin 50 gene (GJA8) in a Russian family with zonular pulverulent cataract
Brachytelephalangic dwarfism due to the loss of ARSE and SHOX genes resulting from an X;Y translocation
Identification of the intron 14 splicing defect of the cholesteryl ester transfer protein gene in Hong Kong Chinese Hong Kong Chinese
New Alzheimer’s disease susceptibility locus on chromosome 10q
Paranoia in plants
A Down syndrome case with a karyotype of 46,XY,rec(21)dup(21q)inv(21)(p11q22) derived from paternal pericentric inversion of chromosome 21
Sequestering CBP: the problem with expanded polyglutamine repeats
A non‐sex chromosome marker in a patient with an atypical Ullrich–Turner phenotype and mosaicism of 46,X,mar/46,XX
<i>De novo</i> inverted duplication of chromosome 7(q21.3→q35): cytogenetic diagnosis confirmed by FISH analysis
Genetic studies yield conflicting data in schizophrenia
Familial deletion of (8)(q24.13q24.22) associated with a normal phenotype
A Reply
Prevalence of CFTR mutations in hypertrypsinaemia detected through neonatal screening for cystic fibrosis
Dwarfs in art
Relation of cardiac abnormalities and CTG‐repeat size in myotonic dystrophy
The developmental program of the hypothalamus and its disorders
Association of a single nucleotide polymorphism in <i>CPB2</i> encoding the thrombin‐activable fibrinolysis inhibitor (TAFI) with blood pressure aboriginal Canadians
A PCR‐based method for detecting known mutations in the human UDP galactose‐4′‐epimerase gene associated with epimerase‐deficiency galactosemia
Founder mutations in the <i>BRCA1</i> gene in west Belarusian breast‐ovarian cancer families
Recombinant Down syndrome: a case report and literature review
Silencing the quiet
Familial aggregation of QT‐interval variability in a general population: results from the NHLBI Family Heart Study
Identification of novel C253Y missense and Y864X nonsense mutations in the insulin receptor gene in type A insulin‐resistant patients
Cenani–Lenz syndrome with renal hypoplasia is not linked to <i>FORMIN</i> or <i>GREMLIN</i>
Genetic analysis of early onset cerebellar ataxia with retained tendon reflexes in four Tunisian families
Cryptic familial t(11;18)(q25;q23) incidentally detected by interphase FISH
APOC3, CETP, fibrinogen, and MTHFR are genetic determinants of carotid intima‐media thickness in healthy men (the Stanislas Cohort)
The DSL domain in mutant JAG1 ligand is essential for the severity of the liver defect in Alagille syndrome Japanese
IRS1 and GRB2 as members of the IGF signal transduction pathway are not associated with intrauterine growth retardation and Silver–Russell syndrome
Genetic landmarks through philately: the Human Genome Project and the new millennium
LDL‐receptor gene mutations and the hypocholesterolemic response to statin therapy populations with recent genetic admixture (<400 years)
Flow cytometry: an ‘old’ tool for novel applications in medical genetics
Familial resemblance for glucose and insulin metabolism indices derived from an intravenous glucose tolerance test in Blacks and Whites of the HERITAGE Family Study Blacks, Whites, Black families, White families
Haplotype analysis of the USH1D locus and genotype–phenotype correlations
Association of a single nucleotide polymorphism in the TIGR/MYOCILIN gene promoter with the severity of primary open‐angle glaucoma
Features within the holoprosencephaly spectrum in sibs with a Robertsonian (14q;22q) translocation chromosome
Effects of pravastatin therapy on serum lipids and coronary reactivity are not associated with SREBP cleavage‐activating protein polymorphism in healthy young men
A molecular link between two disorders
A case for CREB‐dependent transcription activation in neurodegenerative disorders
Sex determination: lessons from families and embryos
Treating a mouse model for Sly disease with a novel form of gene therapy
A super sensor for DNA integrity: bigger is better
Distal limb malformations: underlying mechanisms and clinical associations
Trisomy rescue by postzygotic unbalanced (X;14) translocation in a girl with dysmorphic features
Real‐time quantitative PCR analysis for α‐thalassemia‐1 of Southeast Asian type deletion in Taiwan
Tandem duplication mosaicism: characterization of a mosaic dup(5q) and review
The role of genomic imprinting in human developmental disorders: lessons from Prader–Willi syndrome
Mutational screening of the cationic trypsinogen gene in a large cohort of subjects with idiopathic chronic pancreatitis
Monsters, myths and syndromes depicted on stamps
Genetic evidence to exclude the androgen receptor co‐factor, ARA70 (NCOA4) as a candidate gene for the causation of undermasculinised genitalia
<i>CYP21</i> mutations and congenital adrenal hyperplasia
The Smith–Lemli–Opitz syndrome: a novel metabolic way of understanding developmental biology, embryogenesis, and dysmorphology
Tetrasomy 21 due to a <i>de novo</i> Robertsonian translocation t(14;21) and an additional free trisomy 21
Quantification of all fetal nucleated cells in maternal blood between the 18th and 22nd weeks of pregnancy using molecular cytogenetic techniques
Discoveries in Charcot–Marie–Tooth disease, Crohn's disease and Bardet–Biedl syndrome 4
Trisomy 17p10‐p12 resulting from a supernumerary marker chromosome derived from chromosome 17: molecular analysis and delineation of the phenotype
PCR–PRINS–FISH analysis of structurally abnormal sex chromosomes in eight patients with Turner phenotype
A systematic search for single nucleotide polymorphisms (SNPs) in the insulin receptor gene: association of an SNP with hyperlipidemia in Japanese type 2 diabetic subjects Japanese
Identification of a common variant in the lipoprotein lipase gene in a large Utah kindred ascertained for coronary heart disease: the −93G/D9N variant predisposes to low HDL‐C/high triglycerides
Analysis of candidate genes for intrinsic neuropathy in a family with chronic idiopathic intestinal pseudo‐obstruction
A phosphatase mutation implicated in multiple sclerosis
A novel mutation in exon 2 of the low‐density lipoprotein‐receptor gene in a patient with homozygous familial hypercholesterolemia
Epidemiology of myotonic dystrophy in Italy: re‐apprisal after genetic diagnosis
Risk factors for cataract in Chinese patients with type 2 diabetes: evidence for the influence of the aldose reductase gene
Novel mutation of the Spastin gene in familial spastic paraplegia
Rett syndrome from quintuple and triple deletions within the <i>MECP2</i> deletion hotspot region
Genetic heterogeneity in Korean families with autosomal‐dominant polycystic kidney disease (ADPKD): the first Asian report Western countries; Western population
High incidence rate and absent family histories in one quarter of patients newly diagnosed with Huntington disease in British Columbia
Comparative analysis of isodisomic and heterodisomic segments in cases with maternal uniparental disomy 14 suggests more than one imprinted region
The LPL S447X cSNP is associated with decreased blood pressure and plasma triglycerides, and reduced risk of coronary artery disease
Apolipoproteins AI, B, and E polymorphisms in severe aortic valve stenosis
The fragile X gene and its function
History of genetics through philately – deafness
The ‘flap’ endonuclease gene FEN1 is excluded as a candidate gene implicated in the CAG repeat expansion underlying Huntington disease
Clinical and molecular studies of a large family with desmin‐associated restrictive cardiomyopathy
Identification of fifteen novel mutations and genotype–phenotype relationship in Fabry disease
Hemochromatosis mutations C282Y and H63D in ‘cis’ phase Irish/Belgian; Irish/Belgian female
<i>PAX6</i> mutation in a family with aniridia, congenital ptosis, and mental retardation
Discoveries in Charcot–Marie–Tooth disease, Crohn's disease and Bardet–Biedl syndrome 4
Medical genetics and patient use of the Internet
Genetic determinants of type 2 diabetes mellitus
Familial case of Potocki–Shaffer syndrome associated with microdeletion of <i>EXT2</i> and <i>ALX4</i>
Resolution of a mispaired secondary structure intermediate could account for a novel micro‐insertion/deletion (387 insA/del 8 bp) in the <i>PYGM</i> gene causing McArdle's disease
Mice and men waltz together towards an understanding of hereditary deafness
Analysis of myocilin gene mutations in Japanese patients with normal tension glaucoma and primary open‐angle glaucoma Japanese, Chinese
W44C mutation in the connexin 26 gene associated with dominant non‐syndromic deafness
The VACTERL association: lessons from the Sonic hedgehog pathway
The risk for congenital heart defects in offspring of individuals with congenital heart defects
Fundus albipunctatus and retinitis punctata albescens in a pedigree with an R150Q mutation in <i>RLBP1</i>
The additive effect of neurotransmitter genes in pathological gambling race
Goals of genetic counseling
Lack of mutations in the RANK gene in Spanish patients with Paget disease of bone
Hypermobility type of Ehlers–Danlos syndrome: influence of pregnancies
Living with Marfan syndrome I. Perceptions of the condition
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Human artificial chromosomes: emerging from concept to reality in biomedicine
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7th International Meeting on Psychosocial Aspects of Genetic Testing for Hereditary Breast and/or Ovarian Cancer (HBOC) and Hereditary Non‐polyposis Colorectal Cancer (HNPCC)
Hammerhead ribozyme as a therapeutic agent for hyperlipidemia: Production of truncated apolipoprotein B and hypolipidemic effects in a dyslipidemia murine model
Prolonged correction of hyperlipidemia in mice with familial hypercholesterolemia using an adeno-associated viral vector expressing very-low-density lipoprotein receptor
BAC to basics in understanding human Down syndrome
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British Human Genetics Conference
Antley–Bixler syndrome and maternal virilization: a proposal of genetic heterogeneity
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The budding utility of yeast artificial chromosomes in modeling human disease
Long-term reversal of hypercholesterolemia in low-density lipoprotein receptor (LDLR)-deficient mice by adenovirus-mediated LDLR gene transfer combined with CD154 blockade