Clinical Genetics - 2006

203 articles | Last updated: 2025-12-03 14:12:56
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T A T A T A T A
Two novel translocation breakpoints upstream of <i>SOX9</i> define borders of the proximal and distal breakpoint cluster region in campomelic dysplasia
Corrigendum
Hereditary hemochromatosis screening: effect of mutation penetrance and prevalence on cost‐effectiveness of testing algorithms
The correlation of CTG repeat length with material and social deprivation in myotonic dystrophy
Family system characteristics and psychological adjustment to cancer susceptibility genetic testing: a prospective study
Co‐existence of two functional mutations on the same allele of the human ferrochelatase gene in erythropoietic protoporphyria Italian families
Overlapping clinical phenotypes: the road to identifying dysmorphology signalling pathways and their associated risks
The neoplastic risk in children with Noonan syndrome and Costello syndrome
Early lethal autosomal recessive enterocolitis: report of a second family
<i>ENG</i> mutations in <i>MADH4/BMPR1A</i> mutation negative patients with juvenile polyposis
Diagnosis and management of early‐ and late‐onset cerebellar ataxia
Inheritance of a novel RYR1 mutation in a family with myotonic dystrophy type 1
In search of a familial cancer risk assessment tool
Six novel mutations in the <i>GCK</i> gene in MODY patients
Prevalence of C282Y, H63D and S65C mutations of the hemochromatosis (<i>HFE</i>) gene in a population from southeastern Spain (Murcia Region)
A gift or a yoke? Women’s and men’s responses to genetic risk information from BRCA1 and BRCA2 testing
<i>PKD2</i> gene mutation analysis in Korean autosomal dominant polycystic kidney disease patients using two‐dimensional gene scanning Korean
Clinical and molecular analysis of X‐linked Charcot‐Marie‐Tooth disease type 1 in Spanish population Spanish population
Author index
Detection of an unusual 17p13.3 microdeletion by array comparative genomic hybridisation in a patient with lissencephaly
Brachydactyly type B1: report of a family with <i>de novo ROR2</i> mutation
Searching for the Aicardi–Goutières syndrome genes: TREX1 and ribonuclease H2 make the cut
Pejvakin echoes hope for the end of silence
Neighbor trouble: defective exocrine acinar cell function causes diabetes
Mutation analysis of <i>BRCA1</i> and <i>BRCA2</i> from 793 Korean patients with sporadic breast cancer
Subject index
Mutations in the <i>RSK2(RPS6KA3)</i> gene cause Coffin–Lowry syndrome and nonsyndromic X‐linked mental retardation
Recurrence of the p.R156X TNNI2 mutation in distal arthrogryposis type 2B
The modular nature of genetic diseases
A patient with classic Rett syndrome with a novel mutation in MECP2 exon 1
Evidence against a major genetic basis for combined breast and colorectal cancer susceptibility
Utilization of genetic counseling services by surgical oncologists: education a must
Spastin gene mutations in Bulgarian patients with hereditary spastic paraplegia Bulgarian, Turks and Gypsies; Bulgarian SPG4 patients
Wilms’ tumor and novel <i>TRIM37</i> mutations in an Australian patient with mulibrey nanism Finnish population; Australian patient
Predictive and pre‐natal testing for Huntington Disease in Australia: results and challenges encountered during a 10‐year period (1994–2003)
Erratum
The post‐Human Genome Project mindset: race, reliability, and health care race; racial categories; an emphasis on race in medicine and genetics; use of racial categories in m
Erratum
An updated mutation spectrum in an Australian series of PJS patients provides further evidence for only one gene locus
Association of the <i>CT</i> gene (CA) polymorphism with BMD in osteoporotic mexican women
Introduction: genetic diversity and science communication
Bad neighbors cause dementia; a second 17q21‐linked gene responsible for frontotemporal dementia
Inherited association of breast and colorectal cancer: limited role of <i>CHEK2</i> compared with high‐penetrance genes
Pathogenesis in SCA8 is a two‐way street
Age‐related dysfunction of the cellular powerplants
Chimera and other fertilization errors
Large deletions of the <i>MECP2</i> gene in Chinese patients with classical Rett syndrome
Science communication in transition: genomics hype, public engagement, education and commercialization pressures
Hereditary lymphedema type I associated with <i>VEGFR3</i> mutation: the first <i>de novo</i> case and atypical presentations
Heterozygosity for a Mendelian disorder as a risk factor for complex disease
Identification of novel and recurrent glucokinase mutations in Belgian and Luxembourg maturity onset diabetes of the young patients
Molecular background of polyendocrinopathy–candidiasis–ectodermal dystrophy syndrome in a Polish population: novel <i>AIRE</i> mutations and an estimate of disease prevalence Polish population; in Poland; background population
Germline mosaicism for a <i>MECP2</i> mutation in a man with two Rett daughters
A novel mutation in <i>BAP/SIL1</i> gene causes Marinesco–Sjögren syndrome in an extended pedigree
The genetics of hydatidiform moles: new lights on an ancient disease
Maturity of judgement in decision making for predictive testing for nontreatable adult‐onset neurogenetic conditions: a case against predictive testing of minors
Presymptomatic and predictive genetic testing in minors: a systematic review of guidelines and position papers
Predictive testing for Huntington disease: interpretation and significance of intermediate alleles
Application of <i>BRCA1</i> and <i>BRCA2</i> mutation carrier prediction models in breast and/or ovarian cancer families of French Canadian descent French Canadian descent
A frameshift mutation in peroxisome‐proliferator‐activated receptor‐γ in familial partial lipodystrophy subtype 3 (FPLD3; MIM 604367)
Ironing out neurodegeneration: mutations in a phospholipase A<sub>2</sub> cause neurodegenerative diseases with iron accumulation
Variability and inequity in testing of somatic tissue for hereditary cancer: a survey of UK clinical practice
Characterizing genetic wrinkles in sperm of advanced paternal age
Genes for Joubert syndrome: <i>CEP290</i> is in the middle of it
Stem cell research: cloning, therapy and scientific fraud
Dystrophic epidermolysis bullosa pruriginosa in Italy: clinical and molecular characterization Italian
Commentary – Genetic diversity and science communication – some issues of ‘translation’ and language
The laminopathies: a clinical review
Origin of the <i>SCA7</i> gene mutation in South Africa: implications for molecular diagnostics
Assessing risk assessment: genetic testing and screening for complex disease
The challenges of translating genomic knowledge
Developmental programming of hypothalamic feeding circuits
Risk‐reducing surgery, screening and chemoprevention practices of <i>BRCA1</i> and <i>BRCA2</i> mutation carriers: a prospective cohort study
Note
Freeman–Sheldon syndrome and Sheldon–Hall syndrome: contracting new genotypes
When bone becomes your enemy: fibrodysplasia ossificans progressiva
E‐cadherin promoter polymorphism (C‐160A) and risk of recurrence in patients with superficial bladder cancer
<i>CYLD</i> mutations underlie Brooke–Spiegler, familial cylindromatosis, and multiple familial trichoepithelioma syndromes
Mutation analysis of the <i>PMP22</i>, <i>MPZ</i>, <i>EGR2</i>, <i>LITAF</i>, and <i>GJB1</i> genes in Korean patients with Charcot‐Marie‐Tooth neuropathy type 1
Genetic and other diseases in the pottery of Tumaco‐La Tolita culture in Colombia–Ecuador
Spectrum of pathogenic mutations and associated polymorphisms in a cohort of 44 unrelated patients with long QT syndrome
Hemochromatosis gene mutations in patients with alcoholic cirrhosis
Caps off: Dkc1 makes the IRESes bloom!
Mosaic maternal uniparental isodisomy for chromosome 7q21‐qter
Applications and limitations of empiric data in provision of recurrence risks for schizophrenia: a practical review for healthcare professionals providing clinical psychiatric genetics consultations
Large genomic alterations in <i>hMSH2</i> and <i>hMLH1</i> in early‐onset colorectal cancer: identification of a large complex <i>de novo hMLH1</i> alteration
Cryptic mosaicism for monosomy 20 identified in renal tract cells
A novel locus for alopecia with mental retardation syndrome (APMR2) maps to chromosome 3q26.2‐q26.31
Neuropsychiatric findings of Möbius sequence – a review
Microsatelite GT polymorphism in the toll‐like receptor 2 is associated with colorectal cancer Croatians
The genotype–phenotype correlation of hereditary multiple exostoses
Phenotypic variability in isodicentric Y patients: study of nine cases
Spinocerebellar ataxias in 114 Brazilian families: clinical and molecular findings
The C‐480T hepatic lipase polymorphism is associated with HDL‐C but not with risk of coronary heart disease
Identification of novel mutations in the RSK2 gene (<i>RPS6KA3</i>) in patients with Coffin–Lowry syndrome
A role for genetics in the outcome of antidepressant treatment for major depressive disorder
Human embryo and early fetus research
Osteogenesis imperfecta: clinical, biochemical and molecular findings
Screening for<i> MECP2 </i>mutations in Spanish patients with an unexplained mental retardation
Cleidocranial dysplasia in a Polish population: high frequency of the R193X mutation
Molecular and phenotypic characteristics of patients with phenylketonuria in Serbia and Montenegro Serbia and Montenegro; Balkan Peninsula
Frequency of the hemochromatosis gene mutations in the population of Serbia and Montenegro
Farnesyl transferase inhibitors: the ‘fountain of youth’ for progeria syndromes
A GLUT of sugar leads to a tortuous future
A cryptic full mutation in a male with a classical Fragile X phenotype
Genetic association between endothelial nitric oxide synthase and Alzheimer disease
Neurofibromin: a general outlook
Mutations in PHD‐like domain of the <i>ATRX</i> gene correlate with severe psychomotor impairment and severe urogenital abnormalities in patients with ATRX syndrome
A novel nonsense mutation in the <i>EYA1</i> gene associated with branchio‐oto‐renal/branchiootic syndrome in an Afrikaner kindred Afrikaner descent
Note
Announcement
Gonadal mosaicism in hereditary angioedema
Ferroportin (SLC40A1) gene in thalassemic patients of Indian descent Indian descent
Maternal origin of a novel C‐terminal truncation mutation in <i>CDKL5</i> causing a severe atypical form of Rett syndrome
Frequency and nature of <i>hMSH6</i> germline mutations in Polish patients with colorectal, endometrial and ovarian cancers
Detection of a novel Alu‐mediated <i>BRCA1</i> exon 13 duplication in Chinese breast cancer patients and implications for genetic testing Chinese
Is loss of function of <i>PCSK9</i> a key in the protection against atherosclerotic cardiovascular disease?
Still more from FGFR: LADD syndrome caused by different mutations in FGFR and their ligands
Is there an influence of X‐chromosomal imprinting on the phenotype in Klinefelter syndrome? A clinical and molecular genetic study of 61 cases
Cerebrospinal fluid levels of orexin‐A are not a clinically useful biomarker for Huntington disease
Pharmacogenetics of antidepressants: serotonin 2A receptor genotype strongly associated with treatment outcome
Subject index
First evidence for a molecular link between Usher 1 and Usher 2 syndromes
Author index
Factors in decision making following genetic counseling for pre‐natal diagnosis of <i>de novo</i> chromosomal rearrangements
Splicing generates a polycystin‐2 variant with inverted topology
Digitisation of <i>Clinical Genetics</i>
Association of lipoprotein lipase S447X, apolipoprotein E exon 4, and apoC3 −455T&gt;C polymorphisms on the susceptibility to diabetic nephropathy
Clinical and genetic variability of oculodentodigital dysplasia
Genetic knowledge among participants of a German pilot study on hemochromatosis screening
Insights into a new gene involved in cobalamin metabolism
A novel mutation in the <i>EIF2AK3</i> gene with variable expressivity in two patients with Wolcott–Rallison syndrome
Craniofacial malformations: intrinsic <i>vs</i> extrinsic neural crest cell defects in Treacher Collins and 22q11 deletion syndromes
Developmental malformations of the eye: the role of <i>PAX6</i>, <i>SOX2</i> and <i>OTX2</i>
Genetic heterogeneity of synpolydactyly: a novel locus SPD3 maps to chromosome 14q11.2‐q12 Pakistani
Multiple primary malignancies in a patient with situs ambiguus
High‐efficiency multiplex capillary electrophoresis single strand conformation polymorphism (multi‐CE‐SSCP) mutation screening of <i>SCN5A</i>: a rapid genetic approach to cardiac arrhythmia
The personal experience of juvenile Huntington's disease: an interpretative phenomenological analysis of parents' accounts of the primary features of a rare genetic condition
Novel germline mutations in the <i>APC</i> gene of Cypriot patients with familial and sporadic adenomatous polyposis Cypriot population; ethnic molecular study; unique genetic pool in the Cypriot population; Cypriot
Letter to the Editor in response to Duncan RE and Delatycki MB. Predictive genetic testing in young people for adult‐onset conditions: Where is the empirical evidence?′
Response to Semaka
Non‐syndromic, autosomal‐recessive deafness
Response to Fiona Richards' ‘Letter to the Editor’
Linkage with the Ser9Gly DRD3 polymorphism in essential tremor families
Crigler–Najjar type II syndrome may result from several types and combinations of mutations in the <i>UGT1A1</i> gene
Maternal <i>MTR</i> genotype contributes to the risk of non‐syndromic cleft lip and palate in the Polish population Polish population
Genetic architecture of the <i>F7</i> gene in a Spanish population: implication for mapping complex diseases and for functional assays
Views of female breast cancer patients who donated biologic samples regarding storage and use of samples for genetic research
The mapping of <i>DFNB62,</i> a new locus for autosomal recessive non‐syndromic hearing impairment, to chromosome 12p13.2‐p11.23 Pakistani
Cholesteryl ester transfer protein gene mutations in Brazilian hyperalphalipoproteinemia
Genotype‐phenotype correlation of R870H mutation in hypertrophic cardiomyopathy
Hemizygosity for chromosome 2q14.2‐q22.1 spanning the <i>GLI2</i> and <i>PROC</i> genes associated with growth hormone deficiency, polydactyly, deep vein thrombosis and urogenital abnormalities
High prevalence of the IVS 1 + 1 G to A/<i>GJB2</i> mutation among Czech hearing impaired patients with monoallelic mutation in the coding region of <i>GJB2</i> Central European or Slavic populations; Czech; Spain and France; Slavic
Expanding the phenotypic spectrum of L1CAM‐associated disease
Screening of the <i>CAPN3</i> gene in patients with possible LGMD2A
Inflammatory bowel disease and innate immune response genes: the challenge of complex polygenic disorders for the clinical geneticist
Successful umbilical cord blood stem cell transplantation in a patient with Rothmund–Thomson syndrome and combined immunodeficiency
Late‐onset treatment in Menkes disease: is there a correlation between genotype and response to therapy?
LADD syndrome is caused by<i>FGF10</i>mutations
Identification of a submicroscopic deletion of <i>SHH</i> associated with the holoprosencephaly spectrum by array‐based CGH
Psychological aspects of pre‐symptomatic testing for Machado–Joseph disease and familial amyloid polyneuropathy type I
Erratum
A successful approach for the detection of Fabry patients in Argentina
Methylenetetrahydrofolate reductase gene and susceptibility to breast cancer: a meta‐analysis East Asians
The incidence patterns of Down syndrome in Qatar
Clinical profiles of four patients with Rett syndrome carrying a novel exon 1 mutation or genomic rearrangement in the <i>MECP2</i> gene
Skin abnormalities as an early predictor of neurologic outcome in Gaucher disease
Paraoxonase‐1 Q192R polymorphism and risk of sporadic amyotrophic lateral sclerosis
Gene discovery in methylmalonic aciduria and homocystinuria
New developmental insights from high‐throughput biological analysis in <i>Caenorhabditis elegans</i>
Loss of function mutations in <i>SIL1</i> cause Marinesco–Sjögren syndrome
The beat goes on: ciliary proteins are defective in Meckel syndrome.
Genetic knowledge and moral responsibility: ambiguity at the interface of genetic research and clinical practice
Pregnancy‐associated plasma protein A, free human chorionic gonadotrophin and nuchal translucency as predictors of miscarriage
Molecular genetic analysis of 1053 Danish individuals with clinical signs of familial hypercholesterolemia
HMLH1 and HMSH2 germline mutations in Greek families with hereditary non‐polyposis colorectal cancer
Comparative transcriptome maps: a new approach to the diagnosis of colorectal carcinoma patients using cDNA microarrays
Transglutaminase 5 mutations result in Acral Peeling Skin Syndrome.
The first characterization of homozygous HOX gene mutation in humans
A break near SLITRK1: A breakthrough in Tourette syndrome
A novel homozygous <i>MMP2</i> mutation in a family with Winchester syndrome
Succinic semialdehyde dehydrogenase deficiency: clinical, biochemical and molecular characterization of a new patient with severe phenotype and a novel mutation
MLPA <i>vs</i> multiprobe FISH: comparison of two methods for the screening of subtelomeric rearrangements in 50 patients with idiopathic mental retardation
Clinical and molecular aspects of an informative family with neurofibromatosis type 1 and Noonan phenotype
A genetic model for determining <i>MSH2</i> and <i>MLH1</i> carrier probabilities based on family history and tumor microsatellite instability
Clinical and molecular characterization of a new syndrome: the case of 3q29 microdeletion syndrome
Evaluation of two different models to predict BRCA1 and BRCA2 mutations in a cohort of Danish hereditary breast and/or ovarian cancer families Danish
Corrigendum
Announcement
Jewels in the rough – the study of non‐coding genomic sequences as a conduit to understanding human disease
Molecular investigation and long‐term clinical progress in Greek Cypriot families with recessive distal renal tubular acidosis and sensorineural deafness due to mutations in the <i>ATP6V1B1</i> gene Greek Cypriot; Turkish origin
<i>Trans</i>‐species aneuploidy model: the little mouse that could
Cognitive correlates of a functional COMT polymorphism in children with 22q11.2 deletion syndrome
A questionnaire study for 128 patients with Gaucher disease
Mutation analysis in hereditary haemorrhagic telangiectasia in Germany reveals 11 novel <i>ENG</i> and 12 novel <i>ACVRL1/ALK1</i> mutations German
Nine novel mutations in the hydroxymethylbilane synthase gene of Polish patients with acute intermittent porphyria
Common variants of multiple genes that control reverse cholesterol transport together explain only a minor part of the variation of HDL cholesterol levels
Vesicoureteric reflux and renal malformations: a developmental problem
Prevalence of HFE C282Y and H63D in Jewish populations and clinical implications of H63D homozygosity
Quantification of all fetal nucleated cells in maternal blood in different cases of aneuploidies
Multiple gynecologic tumors as rare associated phenotypes of FAP/Gardner syndrome in a family with the novel germline mutation in the <i>APC</i> gene
Educational outcomes of a workplace screening program for genetic susceptibility to hemochromatosis
15q Duplication associated with autism in a multiplex family with a familial cryptic translocation t(14;15)(q11.2;q13.3) detected using array‐CGH