Clinical Genetics - 2004

224 articles | Last updated: 2025-12-03 14:12:56
Caucasian
6
White
4
European
3
Other
28
Article Title Cauc. White Euro. Other Phrases Used
T A T A T A T A
Subject index
Erratum
Author index
Genes, screens, and means for advancing the diagnosis and anticipatory care of individuals with congenital intellectual disability
What is OnlineEarly?
Genetics of individual differences in bitter taste perception: lessons from the <i>PTC</i> gene
Glyc‐O‐genetics of Walker–Warburg syndrome
Detection of maternal uniparental disomy at the two imprinted genes on chromosome 7, <i>GRB10</i> and <i>PEG1/MEST</i>, in a Silver–Russell syndrome patient using methylation‐specific PCR assays
Kabuki syndrome: a review
Hedgehog signaling and congenital malformations
Insulin resistance and obesity‐related factors in Prader–Willi syndrome: Comparison with obese subjects
Identification of novel <i>SLC3A1</i> gene mutations in Spanish cystinuria families and association with clinical phenotypes Mediterranean Spanish population; East Mediterranean coast of Spain
Genetic susceptibility screening in schools: attitudes of the school community towards hereditary haemochromatosis
Peroxisomal disorders I: biochemistry and genetics of peroxisome biogenesis disorders
Genetic basis of hearing loss associated with enlarged vestibular aqueducts in Koreans
Compound heterozygosity for two non‐synonymous polymorphisms in <i>NPC1L1</i> in a non‐responder to ezetimibe
The potential impact of genetic counseling for mental illness
Multiplex ligation‐dependent probe amplification is superior for detecting deletions/duplications in Duchenne muscular dystrophy
A novel microdeletion syndrome with loss of the <i>MSH2</i> locus and hereditary non‐polyposis colorectal cancer
A family exhibiting arterial tortuosity syndrome displays homozygosity for markers in the arterial tortuosity locus at chromosome 20q13
Reproductive epigenetics
Errata
From families to genes and back: can a hemochromatosis gene help treat the anemia of chronic disease?
Molecular diagnosis of hereditary hemochromatosis: application of a newly‐developed reverse‐hybridization assay in the South African population African; African-specific; African subjects; South African population
Cognitive, language, and adaptive behavior profiles in individuals with a diagnosis of Cohen syndrome
Studying the genetics of Hirschsprung's disease: unraveling an oligogenic disorder
Psychological impact of genetic testing for hereditary non‐polyposis colorectal cancer
<i>DHCR7</i> mutations and genotype–phenotype correlation in 37 Polish patients with Smith–Lemli–Opitz syndrome Western European populations; ethnic Polish patients
Health motivation and emotional vigilance in genetic testing for prostate cancer risk
Chondrodysplasia punctata in siblings and maternal lupus erythematosus
Response to De Vos et al.
Maternal mutation 677C &gt; T in the methylenetetrahydrofolate reductase gene associated with severe brain injury in offspring
Mutational analysis of the α‐<scp><i>l</i></scp>‐<i>iduronidase</i> gene in 10 unrelated Korean type I mucopolysaccharidosis patients: Identification of four novel mutations
The endless quest for sex determination genes
Erratum
Multiple rare alleles influence a complex trait: the case of HDL cholesterol
The Hunter–McAlpine syndrome results from duplication 5q35–qter
Erratum
Scanning the genetic contributors of schizophrenia and bipolar disorder
Detection of RAG mutations and prenatal diagnosis in families presenting with either T–B– severe combined immunodeficiency or Omenn's syndrome
Low frequency of myocilin mutations in Indian primary open‐angle glaucoma patients
The frequency of GJB2 and GJB6 mutations in the New York State newborn population: feasibility of genetic screening for hearing defects Ashkenazi Jewish; Mediterranean descents; pan-ethnic
Announcement
Evidence for single origins of 35delG and delE120 mutations in the <i>GJB2</i> gene in Anatolia Turkish; Egypt; Turkic populations of the Near East; Anatolia
Mutations in endoglin and in activin receptor‐like kinase 1 among Danish patients with hereditary haemorrhagic telangiectasia
FMR1 alleles in Tasmania: a screening study of the special educational needs population
Autosomal dominant B‐cell immunodeficiency, distal limb anomalies and urogenital malformations (BILU syndrome) – report of a second family
Mutation screening of <i>USH3</i> gene (<i>clarin‐1</i>) in Spanish patients with Usher syndrome: low prevalence and phenotypic variability
Small <i>de novo</i> duplication in the repeat region of the TATA‐box‐binding protein gene manifest with a phenotype similar to variant Creutzfeldt‐Jakob disease
Sporadic optic atrophy due to synonymous codon change altering mRNA splicing of <i>OPA1</i>
PMS2 mutations in HNPCC
Fine mapping of the X‐linked split‐hand/split‐foot malformation (SHFM2) locus to a 5.1‐Mb region on Xq26.3 and analysis of candidate genes
Psychiatric and neuropsychological characterization of Pallister‐Hall syndrome
Erratum
The hunt for BRCA2 interactors scores a big hit: EMSY as a prognostic tool for sporadic breast cancer
Pyruvate kinase deficiency: providing protection from <i>Plasmodium</i> parasitism
Simplifying the complex genetics of schizophrenia
The Mainz Severity Score Index: a new instrument for quantifying the Anderson–Fabry disease phenotype, and the response of patients to enzyme replacement therapy
Current clinical selection strategies for identification of hereditary non‐polyposis colorectal cancer families are inadequate: a meta‐analysis
Utilization of prenatal genetic testing by Israeli Moslem women: a national survey
Cascade genetic screening for familial hypercholesterolemia
‘It's (not) only teeth’
Erratum
Allelic heterogeneity of Crigler–Najjar type I syndrome: a study of 24 cases
Low prevalence of Connexin 26 (<i>GJB2</i>) variants in Pakistani families with autosomal recessive non‐syndromic hearing impairment
A dramatic case of early‐onset familial adenomatous polyposis
Melding mind and matter in gene expression studies of the human brain
Genetic counseling of medullary breast cancer patients
Terminal deletion of the long arm of chromosome 10
Novel <i>CFTR</i> mutations in black cystic fibrosis patients black; 'black Africans'; 'black patients'; 'black patients from various ethnies'
Microarray‐based comparative genomic hybridization and its applications in human genetics
Research in human genetics: the tension between doing no harm and personal autonomy
It's only teeth – limits to genetic testing? A response to Aldred, Crawford, Savarirayan, and Savulescu.
Congenital generalized lipodystrophy: profile of the disease and gender differences in two siblings
Erratum
Mutations in TGF‐β receptor type‐2 cause Marfan's syndrome
RNAi applied to a neurodegenerative mouse model
Impact of molecular mechanisms, including deletion size, on Prader–Willi syndrome phenotype: study of 75 patients
A novel <i>STK11</i> germline mutation in two siblings with Peutz–Jeghers syndrome complicated by primary gastric cancer
Genetic counseling for women with breast cancer: the importance of evaluating the spouse
Autism in Angelman syndrome: implications for autism research
Molecular delineation of deletions on 2q37.3 in three cases with an Albright hereditary osteodystrophy‐like phenotype
Mitochondrial A135149 mutation without MELAS but in association with papillary thyroid carcinoma
The dichotomy of causation vs consequence in ID
A new model for prediction of the age of onset and penetrance for Huntington's disease based on CAG length
Mutational spectrum in Usher syndrome type II
Progress in searching for susceptibility loci and genes for smoking‐related behaviour
Attitudes and beliefs concerning prostate cancer genetic screening
Hutchinson–Gilford progeria syndrome
Haplotypic analyses of the aldosterone synthase gene <i>CYP11B2</i> associated with stage‐2 hypertension in northern Han Chinese northern Han Chinese
Letters to the Editor
Anticipated reactions to genetic testing for hereditary non‐polyposis colorectal cancer susceptibility*
High prevalence of V37I genetic variant in the <i>connexin‐26</i> (<i>GJB2</i>) gene among non‐syndromic hearing‐impaired and control Thai individuals East Asian; Thai; populations; ethnicities
Exploration of the impact of messages about genes and race on lay attitudes Blacks
<i>SDHB</i>, <i>SDHC</i>, and <i>SDHD</i> mutation screen in sporadic and familial head and neck paragangliomas
The clinical evaluation and pregnancy outcome of euploid fetuses with increased nuchal translucency
A_16_C haplotype in the <i>Fc</i>ε<i>RI</i>β gene confers a higher risk for atopic asthma in the Indian population
C283Y gamma‐sarcoglycan gene mutation in the Bulgarian Roma (Gypsy) population: prevalence study and carrier screening in a high‐risk community Gypsy; Roma
Epigenetic defects, assisted reproductive technology, and clinical practice: a call for clinicians and genetic counselors
Familial colloid cysts of the third ventricle
‘Secrets and lies’– the difficulties of communicating within families with inherited cancer syndromes
Growing bone knowledge
What happens when the pumps fail? Mutations in <i>ATP1A3</i> are linked to dystonia
Pointing the finger at another mutation in colorectal cancer
Werner syndrome: telomeres to the rescue?
Haplotype analysis at the <i>ETM2</i> locus in a Singaporean sample with familial essential tremor
Genetic landmarks through philately – epilepsy and clinical genetic issues
Genetic variation at the perilipin (<i>PLIN</i>) locus is associated with obesity‐related phenotypes in White women
The dynamics of X‐inactivation skewing as women age
Refined mapping of the autosomal recessive non‐syndromic deafness locus <i>DFNB13</i> using eight novel microsatellite markers
Common variants in β<sub>2</sub>‐ and β<sub>3</sub>‐adrenergic receptor genes and uncoupling protein 1 as predictors of the risk for type 2 diabetes and body weight changes. 
The Finnish Diabetes Prev Finnish
Will the new cytogenetics replace the old cytogenetics?
HD aggregates accelerate autophagy
Computational biology to the rescue: the ongoing quest to understand Bardet–Biedl syndrome
Genetic discrimination: the clinician perspective
Genetic analysis of primary microcephaly in Indian families: novel <i>ASPM</i> mutations
Genotype‐phenotype studies in three families with mutations in the polyglutamine‐binding protein 1 gene (<i>PQBP1</i>)
Cost analysis of DNA‐based testing in a large Canadian family with multiple endocrine neoplasia type 2
T‐box genes and congenital heart/limb malformations
Genetic basis of Prader–Willi syndrome in Korea: less uniparental disomy than has been recognized?
Turning up the heat on hereditary neuropathies
Mechanisms regulating the development of the corpus callosum and its agenesis in mouse and human
Screening of <i>SLC26A4</i> (<i>PDS</i>) gene in Pendred's syndrome: a large spectrum of mutations in France and phenotypic heterogeneity French families
Genetic counseling in carriers of reciprocal chromosomal translocations involving long arm of chromosome 16
New insights in congenital bowing of the femora
Corrigendum
Putting mind over matter: rethinking current strategies for unmasking the genetics of mental illness
Clinical and biochemical study of 28 patients with mucopolysaccharidosis type VI
A previously undescribed frameshift deletion mutation of <i>HFE</i> (c.del277; G93fs) associated with hemochromatosis and iron overload in a C282Y heterozygote
TP63 mutation and clefting modifier genes in an EEC syndrome family
Cholesteryl ester transfer protein promoter single‐nucleotide polymorphisms in Sp1‐binding sites affect transcription and are associated with high‐density lipoprotein cholesterol "African Americans"; "ethnic groups"
Genetic analysis of lethal congenital malformations causing perinatal mortality at Nizwa Hospital, Oman
Risk of placental abruption in first‐degree relatives of index patients
A shared chromosome‐21 haplotype among amyotrophic lateral sclerosis families with the A4V SOD1 mutation
Family cancer histories predictive of a high risk of hereditary non‐polyposis colorectal cancer associate significantly with a genomic rearrangement in hMSH2 or hMLH1
Neonatal salt‐wasting and 11 β‐hydroxylase deficiency in a child carrying a homozygous deletion hybrid <i>CYP11B2</i> (aldosterone synthase)–<i>CYP11B1</i> (11 β‐hydroxylase)
Identification of somatic and germline mosaicism for a keratin 5 mutation in epidermolysis bullosa simplex in a family of which the proband was previously regarded as a sporadic case
Genetic counseling for familial conditions during pregnancy: an analysis of patient characteristics
Fishing for new genes in skin biology: impact of cytogenetics on gene discovery
Novel mutations in the <i>EXT1</i> gene in two consanguineous families affected with multiple hereditary exostoses (familial osteochondromatosis)
Enzyme replacement therapy with agalsidase β improves cardiac involvement in Fabry's disease
Genome‐wide screening using automated fluorescent genotyping to detect cryptic cytogenetic abnormalities in children with idiopathic syndromic mental retardation
Interstitial deletion of 10p and atrial septal defect in DiGeorge 2 syndrome
So many asthma loci, so little time
Discovery of a new protein isoform of MeCP2 and exon 1 mutations causing Rett syndrome
Fat chance: genetic syndromes with obesity
Putative common origin of two <i>MLH1</i> mutations in Italian‐Quebec hereditary non‐polyposis colorectal cancer families Italian origin; Italian kindred; of Italian origin
Spectrum and frequencies of mutations in the <i>GJB2</i> (<i>Cx26</i>) gene among 156 Czech patients with pre‐lingual deafness "prevalent in Europe"; "Czech population"; "Czech Republic"; "Czech patients"; "gypsy heritage"
Detection of <i>CYP3A4</i>*<i>1B</i> and <i>CYP3A4</i>*<i>2</i> polymorphisms by RFLP. Distribution frequencies in a Mexican population Mexican population
Another four bite the dust: mutations in a ubiquitously expressed filamin protein cause several skeletal dysplasias
New options for prenatal diagnosis in autosomal recessive polycystic kidney disease by mutation analysis of the <i>PKHD1</i> gene
Association of parental consanguinity with congenital malformations among Arab newborns in Jerusalem
High frequency of subtelomeric rearrangements in a cohort of 92 patients with severe mental retardation and dysmorphism
Cholesterol at the crossroads: Alzheimer's disease and lipid metabolism
<i>De novo</i> germline mutation in the serine–threonine kinase <i>STK11/LKB1</i> gene associated with Peutz–Jeghers syndrome
Three new families with X‐linked mental retardation caused by the 428–451dup(24bp) mutation in <i>ARX</i>
Familial iridogoniodysgenesis and skeletal anomalies: a probable new autosomal recessive disorder
Pseudodominant inheritance of spondylocostal dysostosis type 1 caused by two familial <i>delta‐like</i> 3 mutations
Localization of a novel locus for hereditary nail dysplasia to chromosome 17q25.1‐17q25.3 Pakistani
Molecular cytogenetic characterization of multiple intrachromosomal rearrangements of chromosome 2q in a patient with Waardenburg's syndrome and other congenital defects
Molecular evidence that AEC syndrome and Rapp–Hodgkin syndrome are variable expression of a single genetic disorder
Making sense out of nonsense: the role of nonsense‐mediated decay in phenotypic variation
A link between <i>ALOX5AP</i> and myocardial infarction and stroke
Low Akt levels might underlie schizophrenia
The long and short of it: C‐type naturietic peptide as a novel therapy for achondroplasia?
Genetic effect of two polymorphisms in the apolipoprotein A5 gene and apolipoprotein C3 gene on serum lipids and lipoproteins levels in a Chinese population Chinese population
ARX mutation in a boy with transsphenoidal encephalocele and hypopituitarism
Mutation spectrum of the glucose‐6‐phosphatase gene and its implication in molecular diagnosis of Korean patients with glycogen storage disease type Ia
The fronto‐ocular syndrome: second mother‐daughter case
Breaking symmetry: a clinical overview of left‐right patterning
Single nucleotide polymorphism in <i>CTH</i> associated with variation in plasma homocysteine concentration
A rare disorder, ethylmalonic encephalopathy, is caused by mutations in a mitochondrial protein
The frequency of <i>GJB2</i> mutations and the Δ (GJB6‐D13S1830) deletion as a cause of autosomal recessive non‐syndromic deafness in the Kurdish population Kurdish population
Incidental neurodevelopmental episodes in the etiology of schizophrenia: An expanded model involving epigenetics and development
Genetic discoveries identify novel treatments for achondroplasia and Alzheimer's disease and molecular basis of ethylmalonic encephalopathy
Prader–Willi syndrome resulting from an unbalanced translocation: characterization by array comparative genomic hybridization
Bronchiectasis in adult patients: an expression of heterozygosity for <i>CFTR</i> gene mutations?
Candidate genes for recessive non‐syndromic mental retardation on chromosome 3p (<i>MRT2A</i>)*
Study of the WFS1 gene and mitochondrial DNA in Spanish Wolfram syndrome families Spanish
Genetic validation of β<b>‐</b>secretase as a drug target for Alzheimer's Disease
Texas physicians' perceptions of genomic medicine as an innovation
Getting at the heart of coronary disease
Variations in high‐density lipoprotein cholesterol in relation to physical activity and <i>Taq</i> 1B polymorphism of the cholesteryl ester transfer protein gene
Genomics goes to the dogs
A new genomic duplication syndrome complementary to the velocardiofacial (22q11 deletion) syndrome
Mutations in the <i>PCSK9</i> gene in Norwegian subjects with autosomal dominant hypercholesterolemia Norwegian
Mutations in the <i>hMLH1</i> gene in Slovenian patients with gastric carcinoma Slovenian
Genetic landmarks through philately – autism spectrum disorders: a genetic update
Association between −250G/A polymorphism of the hepatic lipase gene promoter and coronary artery disease and HDL‐C levels in a Southern Brazilian population
Prenatal diagnosis of <i>de novo</i> X;autosome translocations
Molecular heterogeneity in two families with auditory pigmentary syndromes: the role of neuroimaging and genetic analysis in deafness
Implementation of HaemScreen, a workplace‐based genetic screening program for hemochromatosis
Deletion of the <i>TWIST</i> gene in a large five‐generation family
A novel microdeletion, del(2)(q22.3q23.3) in a mentally retarded patient, detected by array‐based comparative genomic hybridization
A homozygous <i>HAMP</i> mutation in a multiply consanguineous family with pseudo‐dominant juvenile hemochromatosis
Germline‐sequence variants S836S and L769L in the RE arranged during Transfection (RET) proto‐oncogene are not associated with predisposition to sporadic medullary carcinoma in the French population French population
Skeletal phenotype in patients with Shwachman–Diamond syndrome and mutations in <i>SBDS</i>
The locus responsible for horizontal gaze palsy/progressive scoliosis and brainstem hypoplasia is refined to a 9‐cM region on chromosome 11q23
Alström syndrome: intrafamilial phenotypic variability in sibs with a novel nonsense mutation of the <i>ALMS1</i> gene
A model for disclosing the first trimester part of an integrated Down's syndrome screening test
Sanfilippo B syndrome: molecular defects in Greek patients
Hardly a stroke of luck! (Uncovering a gene for stroke is hard work)
A putative E3 ubiquitin ligase is deficient in progressive myoclonus epilepsy
Novel mutations in the <i>CHST6</i> gene causing macular corneal dystrophy Italian families
Genetic variation at the hormone sensitive lipase: gender‐specific association with plasma lipid and glucose concentrations
Concordance of phenotypic expression and gender identity in a large kindred with a mutation in the androgen receptor German origin
The influence of <i>APOAV</i> polymorphisms (T‐1131&gt;C and S19&gt;W) on plasma triglyceride levels and risk of myocardial infarction Czech population
Real‐time PCR‐based gene dosage assay for detecting <i>BRCA1</i> rearrangements in breast–ovarian cancer families French families
Bilateral complete radioulnar synostosis associated with ectrodactyly and sensorineural hearing loss: a variant of SHFM1
Identification of the first Lebanese mutation in the <i>LPL</i> gene and description of a rapid detection method
Gaucher's disease: a paradigm for interventional genetics Ashkenazi Jewish community
Effective long‐term control of cardiac events with β‐blockers in a family with a common LQT1 mutation
Heterozygous manifestations in female carriers of Mal de Meleda
RNA‐mediated neurodegeneration from a different point of view: rCGG in the <i>Drosophila</i> eye
Hereditary non‐polyposis colorectal cancer and the role of <i>hPMS2</i> and <i>hEXO1</i> mutations
Supernumerary marker 15 chromosome in a patient with Prader–Willi syndrome
Polymorphisms in cytokines and growth factor genes and their association with acute rejection and recurrence of hepatitis C virus disease in liver transplantation
Genetic testing in spinocerebellar ataxia in Taiwan: expansions of trinucleotide repeats in SCA8 and SCA17 are associated with typical Parkinson's disease ethnic Chinese; Taiwanese; Japanese
The clinical picture of the Börjeson–Forssman–Lehmann syndrome in males and heterozygous females with <i>PHF6</i> mutations
The Δ&gt;15 Kb deletion French Canadian founder mutation in familial hypercholesterolemia: rapid polymerase chain reaction‐based diagnostic assay and prevalence in Quebec
Taking it to the max: The genetic and developmental mechanisms coordinating midfacial morphogenesis and dysmorphology
Mutations in a G protein‐coupled receptor cause hypogonadotropic hypogonadism in humans and mice
MEF2A at the heart of coronary artery disease?
RUNX1: transcription factor scores a hat‐trick of autoimmune diseases