European Journal of Human Genetics - 2003

215 articles | Last updated: 2025-12-03 14:12:56
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T A T A T A T A
Introduction
Provision of genetic services in Europe: current practices and issues
Genetic information and testing in insurance and employment: technical, social and ethical issues
Provision of genetic services in Europe: current practices and issues
Population genetic screening programmes: technical, social and ethical issues
Population genetic screening programmes: principles, techniques, practices, and policies
Data storage and DNA banking for biomedical research: informed consent, confidentiality, quality issues, ownership, return of benefits. A professional perspective
Data storage and DNA banking for biomedical research: technical, social and ethical issues
Genetic information and testing in insurance and employment: technical, social and ethical issues
The Genetic Basis of Common Diseases, second edition
Clinical evidence of the nonpathogenic nature of the M34T variant in the connexin 26 gene
Practical genetics: alpha-1-antitrypsin deficiency and the serpinopathies
Difficulties of genetic counseling and prenatal diagnosis in a consanguineous couple segregating for the same translocation (14;15) (q11;q13) and at risk for Prader–Willi and Angelman syndromes
Polymorphism R92Q of the tumour necrosis factor receptor 1 gene is associated with myocardial infarction and carotid intima-media thickness – The ECTIM, AXA, EVA and GENIC Studies
mtDNA polymorphisms in five French groups: importance of regional sampling
Prevalence of lysosomal storage diseases in Portugal
Mutations responsible for 3-phosphoserine phosphatase deficiency
Interleukin-1 gene cluster polymorphisms and susceptibility to clinical malaria in a Gambian case–control study
A comparative expression analysis of four MRX genes regulating intracellular signalling via small GTPases
Six novel mutations in the arginine vasopressin gene in 15 kindreds with autosomal dominant familial neurohypophyseal diabetes insipidus give further insight into the pathogenesis
Familial Mediterranean fever seems to be not uncommon in Greece
Association of the human adiponectin gene and insulin resistance
Biparental expression of ESX1L gene in placentas from normal and intrauterine growth-restricted pregnancies
Susceptibility to pre-eclampsia in Finnish women is associated with R485K polymorphism in the factor V gene, not with Leiden mutation Finnish women
Minimum prevalence, birth incidence and cause of death for Prader–Willi syndrome in Flanders
A high frequency of BRCA2 gene mutations in Polish families with ovarian and stomach cancer Polish
Asymptomatic carriers for homozygous novel mutations in the FKRP gene: the other end of the spectrum
Haplotypes and DNA sequence variation within and surrounding the transthyretin gene: genotype–phenotype correlations in familial amyloid polyneuropathy (V30M) in Portugal and Sweden
Confidentiality and serious harm in genetics – preserving the confidentiality of one patient and preventing harm to relatives
Author Index to Volume 11
Alternatively spliced, truncated human BRCA2 isoforms contain a novel coding exon
Prenatal testing for late-onset neurogenetic diseases
Keyword Index to Volume 11
Genetic information and testing in insurance and employment: technical, social and ethical issues
Population genetic screening programmes: technical, social and ethical issues
Provision of genetic services in Europe: current practices and issues
Data storage and DNA banking for biomedical research: technical, social and ethical issues
Power of QTL detection using association tests with family controls
Meta and pooled analysis of European coeliac disease data European coeliac disease data
Molecular diagnosis of Huntington disease in Portugal: implications for genetic counselling and clinical practice
The deleted in colorectal carcinoma (DCC) gene 201 R → G polymorphism: no evidence for genetic association with autoimmune disease
A missense mutation in PAX9 in a family with distinct phenotype of oligodontia
Molecular genetics of exercise-induced polymorphic ventricular tachycardia: identification of three novel cardiac ryanodine receptor mutations and two common calsequestrin 2 amino-acid polymorphisms
Parietal foramina with cleidocranial dysplasia is caused by mutation in MSX2
Karyotyping of human synaptonemal complexes by cenM-FISH
An unusual arylsulfatase A pseudodeficiency allele carrying a splice site mutation in a metachromatic leukodystrophy patient
Spinocerebellar ataxia type 7 associated with pigmentary retinal dystrophy
Mutations in NSD1 are responsible for Sotos syndrome, but are not a frequent finding in other overgrowth phenotypes
Evidence for linkage of nonsyndromic cleft lip with or without cleft palate to a region on chromosome 2
Evidence for a QTL on chromosome 19 influencing LDL cholesterol levels in the general population
Mitochondrial A12308G polymorphism affects clinical features in patients with single mtDNA macrodeletion
Is the novel SCKL3 at 14q23 the predominant Seckel locus?
No evidence for involvement of IL-4R and CD11B from the IBD1 region and STAT6 in the IBD2 region in Crohn's disease
Novel mutations in the PEX12 gene of patients with a peroxisome biogenesis disorder
Novel missense mutations in the TRPS1 transcription factor define the nuclear localization signal
In NF1, CFTR, PER3, CARS and SYT7, alternatively included exons show higher conservation of surrounding intron sequences than constitutive exons
ESHG recommendations on key issues related to the advances in human genetics
MECP2 analysis in mentally retarded patients: implications for routine DNA diagnostics
Genome-wide scanning for linkage in Finnish breast cancer families
Cosegregation of two unlinked mutant alleles in some cases of autosomal dominant familial exudative vitreoretinopathy
Human X-chromosomal lineages in Europe reveal Middle Eastern and Asiatic contacts
Comprehensive evaluation of the association between prostate cancer and genotypes/haplotypes in CYP17A1, CYP3A4, and SRD5A2
Phenotypic and molecular characterisation of the Aarskog–Scott syndrome: a survey of the clinical variability in light of FGD1 mutation analysis in 46 patients
Characterisation of the dysferlin skeletal muscle promoter
Phenylbutyrate increases SMN expression in vitro: relevance for treatment of spinal muscular atrophy
Limb-girdle muscular dystrophy 2I: phenotypic variability within a large consanguineous Bedouin family associated with a novel FKRP mutation
Problems in detecting mosaic DNA methylation in Angelman syndrome
Frequency and parental origin of de novo APC mutations in familial adenomatous polyposis
SLC11A1 (formerly NRAMP1) and susceptibility to visceral leishmaniasis in The Sudan
Risk communication strategies: state of the art and effectiveness in the context of cancer genetic services
Anticipation in familial intracranial aneurysms in consecutive generations
Mutations in the COCH gene are a frequent cause of autosomal dominant progressive cochleo-vestibular dysfunction, but not of Meniere's disease
DFNB40, a recessive form of sensorineural hearing loss, maps to chromosome 22q11.21–12.1
Parametric and nonparametric genome scan analyses for human handedness
Global survey of haplotype frequencies and linkage disequilibrium at the RET locus
Analysis of the French National Registry of unrelated bone marrow donors, using surnames as a tool for improving geographical localisation of HLA haplotypes
From surnames to the history of Y chromosomes: the Sardinian population as a paradigm
DFNB39, a recessive form of sensorineural hearing impairment, maps to chromosome 7q11.22–q21.12
Cell death triggered by a novel mutation in the alphaA-crystallin gene underlies autosomal dominant cataract linked to chromosome 21q
Class II neocentromeres: a putative common neocentromere site in band 4q21.2
Linear increase of structural and numerical chromosome 9 abnormalities in human sperm regarding age
Portuguese families with dentatorubropallidoluysian atrophy (DRPLA) share a common haplotype of Asian origin "Asian origin" and "Portuguese families"
Homozygosity mapping of Marinesco–Sjögren syndrome to 5q31
Maple syrup urine disease in the Austronesian aboriginal tribe Paiwan of Taiwan: a novel DBT (E2) gene 4.7 kb founder deletion caused by a nonhomologous recombination between LINE-1 and Alu and the ca Austronesian aboriginal tribe Paiwan of Taiwan
X-linked spermine synthase gene (SMS) defect: the first polyamine deficiency syndrome
Distribution and frequencies of PDS (SLC26A4) mutations in Pendred syndrome and nonsyndromic hearing loss associated with enlarged vestibular aqueduct: a unique spectrum of mutations in Japanese Japanese
No evidence for DNA copy number change associated with the DUP25 cytogenetic phenotype
Ancient mtDNA analysis and the origin of the Guanches
Recurrent triploidy of maternal origin
Influence of LDL receptor gene mutations and the R3500Q mutation of the apoB gene on lipoprotein phenotype of familial hypercholesterolemic patients from a South European population South European population
A first locus for isolated autosomal recessive optic atrophy (ROA1) maps to chromosome 8q
Gene-gene interaction between the monoamine oxidase A gene and solute carrier family 6 (neurotransmitter transporter, noradrenalin) member 2 gene in anorexia nervosa (restrictive subtype)
The Rapp–Hodgkin syndrome results from mutations of the TP63 gene
Apolipoprotein(a) isoform-specific changes of lipoprotein(a) after kidney transplantation
Lack of a modulative factor in locus 8p23 in a Finnish family with nonsyndromic sensorineural hearing loss associated with the 1555A>G mitochondrial DNA mutation Finnish
Genealogy and genes: tracing the founding fathers of Tristan da Cunha
Polymorphisms of genes of the cardiac calcineurin pathway and cardiac hypertrophy
Genetic polymorphisms in folate and homocysteine metabolism as risk factors for DNA damage
Spondyloepiphyseal dysplasia tarda (SEDL, MIM #313400)
Overgrowth syndromes: is dysfunctional PI3-kinase signalling a unifying mechanism?
Novel spastin mutations and their expression analysis in two Italian families
Comprehensive analysis of human subtelomeres with combined binary ratio labelling fluorescence in situ hybridisation
Parental origin of mutations in sporadic cases of Treacher Collins syndrome
Comment on “The Impact of genotyping error on haplotype reconstruction and frequency estimation”
Reply to letter from T Becker and M Knapp
Inverse correlation between SMN1 and SMN2 copy numbers: evidence for gene conversion from SMN2 to SMN1
Association between Gly241Arg ICAM-1 gene polymorphism and serum sICAM-1 concentration in the Stanislas cohort
Mutation analysis of the Smad3 gene in human osteoarthritis
Mutation analysis of the cystic fibrosis transmembrane conductance regulator (CFTR) gene, the cationic trypsinogen (PRSS1) gene, and the serine protease inhibitor, Kazal type 1 (SPINK1) gene in patien
Juvenile hemochromatosis locus maps to chromosome 1q in a French Canadian population French Canadian population
Computer-based recognition of dysmorphic faces
Manic depressive illness in a founder population
Comment on ‘CFTR gene mutations in sarcoidosis’
A locus for hereditary hypotrichosis localized to human chromosome 18q21.1
Medical and lay attitudes towards genetic screening and testing in Finland
Multiple primary malignancies in osteosarcoma patients. Incidence and predictive value of osteosarcoma subtype for cancer syndromes related with osteosarcoma
SCA17 caused by homozygous repeat expansion in TBP due to partial isodisomy 6
Possible consequences of applying guidelines to healthy women with a family history of breast cancer
Genetic information and life insurance: a ‘real’ risk?
Multilocus statistics to uncover epistasis and heterogeneity in complex diseases: revisiting a set of multiple sclerosis data
Genetic variation at the human tissue-type plasminogen activator (tPA) locus: haplotypes and analysis of association to plasma levels of tPA
Indication of linkage and genetic heterogeneity for asthma and atopy on chromosomes 8p and 12q in 107 French EGEA families
Promoter polymorphism influences the effect of dexamethasone on transcriptional activation of the LTC4 synthase gene
Mutations in the SPINK1 gene in idiopathic pancreatitis Italian patients
Mutational spectrum of the CTNS gene in Italy
Erratum: Familial Mediterranean fever is no longer a rare disease in Italy
Novel POLG mutations in progressive external ophthalmoplegia mimicking mitochondrial neurogastrointestinal encephalomyopathy
Myotonic Dystrophy – The Facts
Muscle glycogenosis with low phosphorylase kinase activity: mutations in PHKA1, PHKG1 or six other candidate genes explain only a minority of cases
In vitro follicular growth affects oocyte imprinting establishment in mice
CATSPER2, a human autosomal nonsyndromic male infertility gene
Identification of 11 novel and common single nucleotide polymorphisms in the interleukin-7 receptor-α gene and their associations with multiple sclerosis
Y chromosomal heritage of Croatian population and its island isolates Croatian population
Erratum: Four novel thymidine phosphorylase gene mutations in mitochondrial neurogastrointestinal encephalomyopathy syndrome (MNGIE) patients
Erratum: Spectrum of mutations in PTPN11 and genotype–phenotype correlation in 96 patients with Noonan syndrome and five patients with cardio-facio-cutaneous syndrome
Reciprocal fusion transcripts of two novel Zn-finger genes in a female with absence of the corpus callosum, ocular colobomas and a balanced translocation between chromosomes 2p24 and 9q32
Genetic linkage and imprinting effects on body mass index in children and young adults
Apolipoprotein B 3′-VNTR polymorphism in Eastern European populations Eastern European populations
Over-representation of two specific haplotypes among chromosomes harbouring BRCA1 mutations
Clinical and molecular analysis of nine families with Adams–Oliver syndrome
Independent effects of the −219 G>T and ε2/ε3/ε4 polymorphisms in the apolipoprotein E gene on coronary artery disease: The Southampton Atherosclerosis Study
A gene for familial isolated chronic nail candidiasis maps to chromosome 11p12–q12.1
Similar contributions of BRCA1 and BRCA2 germline mutations to early-onset breast cancer in Germany
An empirical survey on biobanking of human genetic material and data in six EU countries
CGH and direct diagnosis of mosaic structural chromosomal abnormalities: description of a mosaic ring chromosome 17 and review of the literature
Mutations in PAX1 may be associated with Klippel–Feil syndrome
PCR-generated padlock probes distinguish homologous chromosomes through quantitative fluorescence analysis
Measured haplotype analysis of the aldosterone synthase gene and heart size
Influence of missense mutation and silent mutation of LHβ-subunit gene in Japanese patients with ovulatory disorders
Spatial patterns of cystic fibrosis mutation spectra in European populations
Multiplex single-tube screening for mutations in the Nijmegen Breakage Syndrome (NBS1) gene in Hodgkin's and non-Hodgkin's lymphoma patients of Slavic origin Slavic origin
Congenital cataract as the first symptom of a neuromuscular disease caused by a novel single large-scale mitochondrial DNA deletion
MUC1 polymorphism confers increased risk for intestinal metaplasia in a Colombian population with chronic gastritis
Identification of a locus (LCA9) for Leber's congenital amaurosis on chromosome 1p36
A new locus for postaxial polydactyly type A/B on chromosome 7q21–q34
Cys611Ser mutation in RET proto-oncogene in a kindred with medullary thyroid carcinoma and Hirschsprung's disease
Transient expression of wild-type and mutant glucocerebrosidases in hybrid vaccinia expression system
DiGeorge subtypes of nonsyndromic conotruncal defects: evidence against a major role of TBX1 Gene
A single Mediterranean, possibly Jewish, origin for the Val59Gly CDKN2A mutation in four melanoma-prone families Mediterranean, possibly Jewish
Rearrangement in the PITX2 and MIPOL1 genes in a patient with a t(4;14) chromosome
Analysis of nine chromosome probes in first polar bodies and metaphase II oocytes for the detection of aneuploidies
Disease-associated mutations in conserved residues of ALK-1 kinase domain
PNA on human sperm: a new approach for in situ aneuploidy estimation
Skewed X chromosome inactivation in carriers is not a constant finding in FG syndrome
Apparent intrachromosomal exchange on the human Y chromosome explained by population history
Chromosome instability and nibrin protein variants in NBS heterozygotes
Recent advances in the diagnosis of malignant hyperthermia susceptibility: How confident can we be of genetic testing?
Reduced folate carrier polymorphism (80A→G) and neural tube defects
Finnish and Swedish genotypes and risk of cancer in Sweden
Italian family with two independent mutations: 3358T/A in BRCA1 and 8756delA in BRCA2 genes Italian
Mitochondrial DNA analysis reveals diverse histories of tribal populations from India
Collagen XI sequence variations in nonsyndromic cleft palate, Robin sequence and micrognathia
Microsatellite marker association at chromosome region 2p13 in Finnish patients with preeclampsia and obstetric cholestasis suggests a common risk locus Finnish
Combined analysis of genomewide scans for adult height: results from the NHLBI Family Blood Pressure Program
ABCC6/MRP6 mutations: further insight into the molecular pathology of pseudoxanthoma elasticum
Association of NOD2 with Crohn's Disease in a homogenous Irish population homogenous Irish population
A population-based study of the effect of the HFE C282Y and H63D mutations on iron metabolism
Inverse correlation between SMN1 and SMN2 copy numbers: evidence for gene conversion from SMN2 to SMN1
Spectrum of mutations in PTPN11 and genotype–phenotype correlation in 96 patients with Noonan syndrome and five patients with cardio-facio-cutaneous syndrome
Screening for PAX6 gene mutations is consistent with haploinsufficiency as the main mechanism leading to various ocular defects
Combined high resolution linkage and association mapping of quantitative trait loci
On the role of CFTR, PSSR1 and PST1/SPINK1 in idiopathic chronic pancreatitis
Reply: ‘Gain of function’ PRSS1 mutations are rare in ICP
A new quantitative PCR multiplex assay for rapid analysis of chromosome 17p11.2-12 duplications and deletions leading to HMSN/HNPP
A new locus (DFNA47) for autosomal dominant non-syndromic inherited hearing loss maps to 9p21-22 in a large Italian family Italian
No evidence for a parental inversion polymorphism predisposing to rearrangements at 22q11.2 in the DiGeorge/Velocardiofacial syndrome
Clustering of Crohn's disease within affected sibships
Mapping of a new autosomal recessive nonsyndromic hearing loss locus (DFNB32) to chromosome 1p13.3-22.1
Genome-wide search in Finnish families with inflammatory bowel disease provides evidence for novel susceptibility loci
The epsilon-sarcoglycan gene (SGCE), mutated in myoclonus-dystonia syndrome, is maternally imprinted
A candidate region for Asperger syndrome defined by two 17p breakpoints
Identification and characterisation of the retinitis pigmentosa 1-like1 gene (RP1L1): a novel candidate for retinal degenerations
Carrier frequency of the V377I (1129G>A) MVK mutation, associated with Hyper-IgD and periodic fever syndrome, in the Netherlands
Familial juvenile hyperuricaemic nephropathy (FJHN): linkage analysis in 15 families, physical and transcriptional characterisation of the FJHN critical region on chromosome 16p11.2 and the analysis o
Haplotype structure and association to Crohn's disease of CARD15 mutations in two ethnically divergent populations
Linkage of aggressive prostate cancer to chromosome 7q31-33 in German prostate cancer families
Cystathionine β-synthase polymorphisms and hyperhomocysteinaemia: an association study
Global genetic variation at nine short tandem repeat loci and implications on forensic genetics
The CTLA4 region as a general autoimmunity factor: An extended pedigree provides evidence for synergy with the HLA locus in the etiology of type 1 diabetes mellitus, Hashimoto's thyroiditis and Graves
A novel autosomal recessive non-syndromic deafness locus (DFNB35) maps to 14q24.1–14q24.3 in large consanguineous kindred from Pakistan
Cryptic subtelomeric 6p deletion in a girl with congenital malformations and severe language impairment
Four novel thymidine phosphorylase gene mutations in mitochondrial neurogastrointestinal encephalomyopathy syndrome (MNGIE) patients
Genetic discrimination: Too few data
PTPN11 mutations are not responsible for the Cardiofaciocutaneous (CFC) syndrome
A Dictionary of Genetics
Psychological distress in the 5-year period after predictive testing for Huntington's disease
Arrhythmogenic right ventricular cardiomyopathy type 1 (ARVD1): confirmation of locus assignment and mutation screening of four candidate genes
PTPN11 mutation in a large family with Noonan syndrome and dizygous twinning
Familial Mediterranean fever is no longer a rare disease in Italy
Discovery of old diseases: the molecular approach
A new web-based data mining tool for the identification of candidate genes for human genetic disorders
A novel mutation in the G4.5 (TAZ) gene in a kindred with Barth syndrome
Cationic trypsinogen and pancreatic secretory trypsin inhibitor gene mutations in neonatal hypertrypsinaemia
Absence of association of fetal MTHFR C677T polymorphism with prenatal Down syndrome pregnancies
Erratum: MTHFR C677T and A1298C polymorphisms and mutated sequences occurring in cis