European Journal of Human Genetics - 2011

283 articles | Last updated: 2025-12-03 14:12:56
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Codon 72 polymorphism (rs1042522) of TP53 is associated with changes in diastolic blood pressure over time
Splicing analysis of unclassified variants in COL2A1 and COL11A1 identifies deep intronic pathogenic mutations
The novel mitochondrial tRNAAsn gene mutation m.5709T>C produces ophthalmoparesis and respiratory impairment
Genotype and cognitive phenotype of patients with tuberous sclerosis complex
How to deal with the early GWAS data when imputing and combining different arrays is necessary
Reply to Talseth-Palmer et al
Antilipolytic effect of calcimimetics depends on the allelic variant of calcium-sensing receptor gene polymorphism rs1042636 (Arg990Gly)
A genome-wide study reveals rare CNVs exclusive to extreme phenotypes of Alzheimer disease
Routine testing for PALB2 mutations in familial pancreatic cancer families and breast cancer families with pancreatic cancer is not indicated
Clinical utility gene card for: acrodermatitis enteropathica
17q24.2 microdeletions: a new syndromal entity with intellectual disability, truncal obesity, mood swings and hallucinations
8q23.3 and 11q23.1 as modifying loci influencing the risk for CRC in Lynch syndrome
Clinical utility gene card for: Alport syndrome
A flexible likelihood framework for detecting associations with secondary phenotypes in genetic studies using selected samples: application to sequence data
Use of the gamma method for self-contained gene-set analysis of SNP data
Skewed X inactivation and survival: a 13-year follow-up study of elderly twins and singletons
‘Nasal’ speech–hyper or hypo?
Exome sequencing and SNP analysis detect novel compound heterozygosity in fatty acid hydroxylase-associated neurodegeneration
Nasal speech in patients with 12q15 microdeletions
Temporal differentiation across a West-European Y-chromosomal cline: genealogy as a tool in human population genetics West-European
Intellectual disability associated with retinal dystrophy in the Xp11.3 deletion syndrome: ZNF674 on trial. Guilty or innocent?
Researchers’ opinions towards the communication of results of biobank research: a survey study
Clinical utility gene card for: Trimethylaminuria
A statistical method for region-based meta-analysis of genome-wide association studies in genetically diverse populations
How genetically heterogeneous is Kabuki syndrome?: MLL2 testing in 116 patients, review and analyses of mutation and phenotypic spectrum
Severe intellectual disability and autistic features associated with microduplication 2q23.1
Neolithic patrilineal signals indicate that the Armenian plateau was repopulated by agriculturalists
Determination of the real effect of genes identified in GWAS: the example of IL2RA in multiple sclerosis
UGT1A1 is a major locus influencing bilirubin levels in African Americans
Sephardic signature in haplogroup T mitochondrial DNA Sephardic
A unique library of myogenic cells from facioscapulohumeral muscular dystrophy subjects and unaffected relatives: family, disease and cell function
Exploring the somatic NF1 mutational spectrum associated with NF1 cutaneous neurofibromas
Case report: type 1 diabetes in monozygotic quadruplets
ALK germline mutations in patients with neuroblastoma: a rare and weakly penetrant syndrome
A multi-exon deletion within WWOX is associated with a 46,XY disorder of sex development
Preimplantation genetic diagnosis in female and male carriers of reciprocal translocations: clinical outcome until delivery of 312 cycles
Newborn screening for sickle cell disease: whose reproductive benefit?
Preimplantation genetic diagnosis (PGD) for Huntington's disease: the experience of three European centres
Clinical utility gene card for: Adrenoleukodystrophy
Copy number detection in discordant monozygotic twins of Congenital Diaphragmatic Hernia (CDH) and Esophageal Atresia (EA) cohorts
Health-care providers’ views on pursuing reproductive benefit through newborn screening: the case of sickle cell disorders
Identification of the first PAR1 deletion encompassing upstream SHOX enhancers in a family with idiopathic short stature
Underexpression and abnormal localization of ATM products in ataxia telangiectasia patients bearing ATM missense mutations
Genome-wide pooling approach identifies SPATA5 as a new susceptibility locus for alopecia areata
Effects of single-nucleotide polymorphisms in the human holocarboxylase synthetase gene on enzyme catalysis
Unique and atypical deletions in Prader–Willi syndrome reveal distinct phenotypes
Novel mutation and three other sequence variants segregating with phenotype at keratoconus 13q32 susceptibility locus
Evolutionary conserved longevity genes and human cognitive abilities in elderly cohorts
Clinical utility gene card for: Multi-minicore disease
Novel NCC mutants and functional analysis in a new cohort of patients with Gitelman syndrome
Association study of the single nucleotide polymorphisms of PARK2 and PACRG with leprosy susceptibility in Chinese population
Clinical utility gene card for: Central core disease
No evidence of association between complement factor I genetic variant rs10033900 and age-related macular degeneration
Reply to Cipriani et al
First description of ABCB4 gene deletions in familial low phospholipid-associated cholelithiasis and oral contraceptives-induced cholestasis
Genome-wide association study of coronary artery disease in the Japanese
De novo deletions and duplications detected by array CGH: a study of parental origin in relation to mechanisms of formation and size of imbalance
Association of TMEM18 variants with BMI and waist circumference in children and correlation of mRNA expression in the PFC with body weight in rats
Permanence of the information given during oncogenetic counseling to persons at familial risk of breast/ovarian and/or colon cancer
Alagille syndrome: pathogenesis, diagnosis and management
Unexpanded and intermediate CAG polymorphisms at the SCA2 locus (ATXN2) in the Cuban population: evidence about the origin of expanded SCA2 alleles
Delineation of a deletion region critical for corpus callosal abnormalities in chromosome 1q43–q44
On the statistical properties of family-based association tests in datasets containing both pedigrees and unrelated case–control samples
Practical guidelines for interpreting copy number gains detected by high-resolution array in routine diagnostics
Isolated NIBPL missense mutations that cause Cornelia de Lange syndrome alter MAU2 interaction
Involvement of surfactant protein D in emphysema revealed by genetic association study
Clinical utility gene card for: Fabry disease
Erratum: MECP2 duplications in six patients with complex sex chromosome rearrangements
Erratum: A dominant mutation in RPE65 identified by whole-exome sequencing causes retinitis pigmentosa with choroidal involvement
Erratum: Association between ORMDL3, IL1RL1 and a deletion on chromosome 17q21 with asthma risk in Australia
Erratum: A genome-wide scan of 10 000 gene-centric variants and colorectal cancer risk
Parental insertional balanced translocations are an important cause of apparently de novo CNVs in patients with developmental anomalies
Clinical utility gene card for: Phenylketonuria
Advances in Alport syndrome diagnosis using next-generation sequencing
Detection of classical 17p11.2 deletions, an atypical deletion and RAI1 alterations in patients with features suggestive of Smith–Magenis syndrome
Genome-wide homozygosity and multiple sclerosis in Orkney and Shetland Islanders
Clinical utility gene card for: Mayer–Rokitansky–Küster–Hauser syndrome
Imaging genetics of FOXP2 in dyslexia
Coronary artery disease in Alström syndrome
No evidence for pathogenic variants or maternal effect of ZFP57 as the cause of Beckwith–Wiedemann Syndrome
Clinical utility gene card for: Cystinuria
Clinical utility gene card for: multiple endocrine neoplasia type 2
Clinical utility gene card for: Mucopolysaccharidosis type II
Beckwith–Wiedemann syndrome caused by maternally inherited mutation of an OCT-binding motif in the IGF2/H19-imprinting control region, ICR1
C1q enhances cone photoreceptor survival in a mouse model of autosomal recessive retinitis pigmentosa
Brazilian urban population genetic structure reveals a high degree of admixture
Erratum: Extended extraocular phenotype of PROM1 mutation in kindreds with known autosomal dominant macular dystrophy
Erratum: An updated tree of Y-chromosome Haplogroup O and revised phylogenetic positions of mutations P164 and PK4
A systematic eQTL study of cis–trans epistasis in 210 HapMap individuals
Functional analysis of a promoter variant identified in the CFTR gene in cis of a frameshift mutation
Identification and functional analysis of novel THAP1 mutations
Surname and Y chromosome in Southern Europe: a case study with Colom/Colombo
Cerebral cavernous malformations: from molecular pathogenesis to genetic counselling and clinical management
People of the British Isles: preliminary analysis of genotypes and surnames in a UK-control population
EMQN best practice guidelines for the laboratory diagnosis of osteogenesis imperfecta
Clinical utility gene card for: Alveolar rhabdomyosarcoma
Peutz–Jeghers syndrome and family planning: the attitude towards prenatal diagnosis and pre-implantation genetic diagnosis
Transgenic complementation of MeCP2 deficiency: phenotypic rescue of Mecp2-null mice by isoform-specific transgenes
Evidence of linkage to chromosomes 10p15.3–p15.1, 14q24.3–q31.1 and 9q33.3–q34.3 in non-syndromic colorectal cancer families
A British approach to sampling
Pan-ethnic carrier screening and prenatal diagnosis for spinal muscular atrophy: clinical laboratory analysis of >72 400 specimens
Attitudes to reproductive genetic testing in women who had a positive BRCA test before having children: a qualitative analysis
Epigenetic regulation of the X-chromosomal macrosatellite repeat encoding for the cancer/testis gene CT47
Impact of gene patents on diagnostic testing: a new patent landscaping method applied to spinocerebellar ataxia
Discrepancies in reporting the CAG repeat lengths for Huntington's disease
Sequencing of high-complexity DNA pools for identification of nucleotide and structural variants in regions associated with complex traits
A genome-wide study of panic disorder suggests the amiloride-sensitive cation channel 1 as a candidate gene
Fine-grained facial phenotype–genotype analysis in Wolf–Hirschhorn syndrome
Natural positive selection and north–south genetic diversity in East Asia
Combining gene mapping and phenotype assessment for fast mutation finding in non-consanguineous autosomal recessive retinitis pigmentosa families
Data-driven approach to detect common copy-number variations and frequency profiles in a population-based Korean cohort
The potential influence of KIR cluster profiles on disease patterns of Canadian Aboriginals and other indigenous peoples of the Americas Canadian Aboriginals; indigenous peoples of the Americas
CGene: an R package for implementation of causal genetic analyses
Motivators for participation in a whole-genome sequencing study: implications for translational genomics research
A novel locus for autosomal dominant congenital cerulean cataract maps to chromosome 12q
Initial interrogation, confirmation and fine mapping of modifying genes: STAT3, IL1B and IFNGR1 determine cystic fibrosis disease manifestation
Normal glycosylation screening does not rule out SRD5A3-CDG
iPS cells to model CDKL5-related disorders
2p15–p16.1 microdeletion syndrome: molecular characterization and association of the OTX1 and XPO1 genes with autism spectrum disorders
A mild form of Mucopolysaccharidosis IIIB diagnosed with targeted next-generation sequencing of linked genomic regions
Serpentine fibula polycystic kidney syndrome is part of the phenotypic spectrum of Hajdu–Cheney syndrome
Balanced into array: genome-wide array analysis in 54 patients with an apparently balanced de novo chromosome rearrangement and a meta-analysis
Maternally inherited mitochondrial DNA disease in consanguineous families
Identification of a Gypsy SHOX mutation (p.A170P) in Léri-Weill dyschondrosteosis and Langer mesomelic dysplasia Gypsy
The tRNAMet 4435A>G mutation in the mitochondrial haplogroup G2a1 is responsible for maternally inherited hypertension in a Chinese pedigree
Mortality in neurofibromatosis 1: in North West England: an assessment of actuarial survival in a region of the UK since 1989
Application of SNP array for rapid prenatal diagnosis: implementation, genetic counselling and diagnostic flow
Genetic determination of human facial morphology: links between cleft-lips and normal variation
Copy number variants and infantile spasms: evidence for abnormalities in ventral forebrain development and pathways of synaptic function
Opinions and intentions of parents of an autistic child toward genetic research results: two typical profiles
Characterization of autosomal copy-number variation in African Americans: the HyperGEN Study
Contribution of bioinformatics predictions and functional splicing assays to the interpretation of unclassified variants of the BRCA genes
Clinical utility gene card for: Haemophilia A
Gene and genetic diagnostic method patent claims: a comparison under current European and US patent law
A dominant mutation in RPE65 identified by whole-exome sequencing causes retinitis pigmentosa with choroidal involvement
An atlas of tissue-specific conserved coexpression for functional annotation and disease gene prediction
Haploinsufficiency of ANKRD11 causes mild cognitive impairment, short stature and minor dysmorphisms
Clinical utility gene card for: Aarskog–Scott syndrome (faciogenital dysplasia)
Mosaics and moles
Desbuquois dysplasia type I and fetal hydrops due to novel mutations in the CANT1 gene
Renal coloboma syndrome
Duplication at chromosome 2q31.1-q31.2 in a family presenting syndactyly and nystagmus
Analysis of H19 methylation in control and abnormal human embryos, sperm and oocytes
Fatty acid desaturase 2 promoter mutation is not responsible for Δ6-desaturase deficiency
Are the kids really all right? Direct-to-consumer genetic testing in children: are company policies clashing with professional norms?
Phenotypic heterogeneity in British patients with a founder mutation in the FHL1 gene
Rapid aneuploidy detection or karyotyping? Ethical reflection
17p13.3 microduplications are associated with split-hand/foot malformation and long-bone deficiency (SHFLD)
Homozygosity mapping in 64 Syrian consanguineous families with non-specific intellectual disability reveals 11 novel loci and high heterogeneity
Finding disease genes: a fast and flexible approach for analyzing high-throughput data
A common spinal muscular atrophy deletion mutation is present on a single founder haplotype in the US Hutterites
Alterations in KLRB1 gene expression and a Scandinavian multiple sclerosis association study of the KLRB1 SNP rs4763655
Treatment of spinal muscular atrophy cells with drugs that upregulate SMN expression reveals inter- and intra-patient variability
Clinical utility gene card for: Dyskeratosis congenita
Assessing the pathological relevance of SPINK1 promoter variants
GWAS Integrator: a bioinformatics tool to explore human genetic associations reported in published genome-wide association studies
Clinical presentation and mutations in Danish patients with Wilson disease Danish patients
Establishment of the first WHO international genetic reference panel for Prader Willi and Angelman syndromes
The causality of de novo copy number variants is overestimated
Fabry or not Fabry – a question of ascertainment
The first missense mutation of NHS gene in a Tunisian family with clinical features of NHS syndrome including cardiac anomaly
Genetic variation in the Sorbs of eastern Germany in the context of broader European genetic diversity
The desmosterolosis phenotype: spasticity, microcephaly and micrognathia with agenesis of corpus callosum and loss of white matter
Worldwide spatial genetic structure of angiotensin-converting enzyme gene: a new evolutionary ecological evidence for the thrifty genotype hypothesis
Clinical utility gene card for: fragile X mental retardation syndrome, fragile X-associated tremor/ataxia syndrome and fragile X-associated primary ovarian insufficiency
Clinical utility gene card for: Loeys-Dietz syndrome (TGFBR1/2) and related phenotypes
Genomic and clinical characteristics of six patients with partially overlapping interstitial deletions at 10p12p11
Clinical utility gene card for: Alström syndrome
Muscular dystrophy with marked Dysferlin deficiency is consistently caused by primary dysferlin gene mutations
Gene-ontology enrichment analysis in two independent family-based samples highlights biologically plausible processes for autism spectrum disorders
The DFNA5 gene, responsible for hearing loss and involved in cancer, encodes a novel apoptosis-inducing protein
An updated tree of Y-chromosome Haplogroup O and revised phylogenetic positions of mutations P164 and PK4
Nasal speech and hypothyroidism are common hallmarks of 12q15 microdeletions
Genotype–phenotype correlations in patients with retinoblastoma and interstitial 13q deletions
Clinical utility gene card for: Menkes disease
Cancer GAMAdb: database of cancer genetic associations from meta-analyses and genome-wide association studies
Multiplex ligation-depending probe amplification is not suitable for detection of low-grade mosaicism
Everything a health professional needs to know about genetics
Gene set analysis of SNP data: benefits, challenges, and future directions
Founder effect and estimation of the age of the French Gypsy mutation associated with Glanzmann thrombasthenia in Manouche families
High-throughput sequencing of complete human mtDNA genomes from the Caucasus and West Asia: high diversity and demographic inferences Caucasus; West Asia
Clinical utility gene card for: Joubert syndrome
Meta-analysis of genome-wide association for migraine in six population-based European cohorts European cohorts
Variants of gene for microsomal prostaglandin E2 synthase show association with disease and severe inflammation in rheumatoid arthritis
Genetic architecture of circulating lipid levels
Reduced transcript expression of genes affected by inherited and de novo CNVs in autism
Clinical and functional data implicate the Arg(151)Ser variant of MSX1 in familial hypodontia
Bivariate association analysis in selected samples: application to a GWAS of two bone mineral density phenotypes in males with high or low BMD
A novel approach for small sample size family-based association studies: sequential tests
Intermediate FMR1 alleles and cognitive and/or behavioural phenotypes
Variable set enrichment analysis in genome-wide association studies
Genomic inflation factors under polygenic inheritance
Clinical utility gene card for: CHARGE syndrome
Strengthening the reporting of genetic risk prediction studies: the GRIPS statement
New disease gene location and high genetic heterogeneity in idiopathic scoliosis
Mild CFTR mutations and genetic predisposition to lactase persistence in cystic fibrosis
Cancer risk in patients with Noonan syndrome carrying a PTPN11 mutation
Not so simple: a quasi-experimental study of how researchers adjudicate genetic research results
Determining the pathogenicity of patient-derived TSC2 mutations by functional characterization and clinical evidence
The genetic component of human longevity: analysis of the survival advantage of parents and siblings of Italian nonagenarians
Association of TH01 with human longevity revisited
Model-Based Multifactor Dimensionality Reduction to detect epistasis for quantitative traits in the presence of error-free and noisy data
Strengthening the reporting of genetic risk prediction studies (GRIPS): explanation and elaboration
Novel mutations affecting LRP5 splicing in patients with osteoporosis-pseudoglioma syndrome (OPPG)
Evaluation of Lynch syndrome modifier genes in 748 MMR mutation carriers
Genetic testing and common disorders in a public health framework: how to assess relevance and possibilities
Translocation breakpoint at 7q31 associated with tics: further evidence for IMMP2L as a candidate gene for Tourette syndrome
Clinical utility gene card for: Usher syndrome
Children, biobanks and the scope of parental consent
The expanded human disease network combining protein–protein interaction information
von Hippel–Lindau disease: A clinical and scientific review
A novel autosomal dominant condition consisting of congenital heart defects and low atrial rhythm maps to chromosome 9q
Simple strategies for haplotype analysis of the X chromosome with application to age-related macular degeneration
Bohring–Opitz (Oberklaid–Danks) syndrome: clinical study, review of the literature, and discussion of possible pathogenesis
Epistasis between neurochemical gene polymorphisms and risk for ADHD
Clinical utility gene card for: Meckel syndrome
Polymorphisms in MC3R promoter and CTSZ 3′UTR are associated with tuberculosis susceptibility
Pathway-based identification of SNPs predictive of survival
Heterogeneity of genetic associations of CDKAL1 and HHEX with susceptibility of type 2 diabetes mellitus by gender
TSEN54 mutations cause pontocerebellar hypoplasia type 5
Clinical utility gene card for: α-Mannosidosis
Human balanced translocation and mouse gene inactivation implicate Basonuclin 2 in distal urethral development
Clinical utility gene card for: Familial adenomatous polyposis (FAP) and attenuated FAP (AFAP)
Clinical utility gene card for: 3M syndrome
Intellectual disability without epilepsy associated with STXBP1 disruption
Consanguinity around the world: what do the genomic data of the HGDP-CEPH diversity panel tell us?
Imputation of low-frequency variants using the HapMap3 benefits from large, diverse reference sets
Respiratory chain complex I deficiency caused by mitochondrial DNA mutations
The GENCODE exome: sequencing the complete human exome
Population frequency of myotonic dystrophy: higher than expected frequency of myotonic dystrophy type 2 (DM2) mutation in Finland
Clinical utility gene card for: Gitelman syndrome
Identification of ACOX2 as a shared genetic risk factor for preeclampsia and cardiovascular disease
Clinical utility gene card for: Mowat–Wilson syndrome
Analysis of prevalence and degree of macrocephaly in patients with germline PTEN mutations and of brain weight in Pten knock-in murine model
De novo MECP2 duplication in two females with random X-inactivation and moderate mental retardation
Parents’ and children's communication about genetic risk: a qualitative study, learning from families’ experiences
Clinical utility gene card for: renal coloboma (Papillorenal) syndrome
Differential secretion of the mutated protein is a major component affecting phenotypic severity in CRLF1-associated disorders
Isolated hypogonadotropic hypogonadism with SOX2 mutation and anophthalmia/microphthalmia in offspring
Multiple independent variants in 6q21-22 associated with susceptibility to celiac disease in the Dutch, Finnish and Hungarian populations
Pericentric inversion of chromosome 18 in parents leading to a phenotypically normal child with segmental uniparental disomy 18
Metopic and sagittal synostosis in Greig cephalopolysyndactyly syndrome: five cases with intragenic mutations or complete deletions of GLI3
Identification of regions of positive selection using Shared Genomic Segment analysis
Clinical utility gene card for: Gorlin syndrome
Common variants near TERC are associated with leukocyte telomere length in the Chinese Han population
The 12q14 microdeletion syndrome: six new cases confirming the role of HMGA2 in growth
Clinical utility gene card for: achromatopsia
Clinical utility gene card for: blue cone monochromatism
Cancer risk management strategies and perceptions of unaffected women 5 years after predictive genetic testing for BRCA1/2 mutations
Clinical utility gene card for: hypertrophic cardiomyopathy (type 1–14)
Hybridisation-based resequencing of 17 X-linked intellectual disability genes in 135 patients reveals novel mutations in ATRX, SLC6A8 and PQBP1
Genetic testing and common disorders in a public health framework
A nonsense mutation in FMR1 causing fragile X syndrome
Does the new HapMap throw the baby out with the bath water?
The effect of genome-wide association scan quality control on imputation outcome for common variants
Type B mandibuloacral dysplasia with congenital myopathy due to homozygous ZMPSTE24 missense mutation
Dexamethasone normalizes aberrant elastic fiber production and collagen 1 secretion by Loeys–Dietz syndrome fibroblasts: a possible treatment?
Breast and ovarian cancer screening of non-carriers from BRCA1/2 mutation-positive families: 2-year follow-up of cohorts from France and Quebec
Clinical utility gene card for: α-1-antitrypsin deficiency
The phenotype of recurrent 10q22q23 deletions and duplications
HTT haplotypes contribute to differences in Huntington disease prevalence between Europe and East Asia
Deletions flanked by breakpoints 3 and 4 on 15q13 may contribute to abnormal phenotypes
Three novel mutations in the ACTA2 gene in German patients with thoracic aortic aneurysms and dissections
Relationship between the polymorphism of tumor necrosis factor-α-308 G>A and susceptibility to inflammatory bowel diseases and colorectal cancer: a meta-analysis
ALK2 mutation in a patient with Down's syndrome and a congenital heart defect
Craniosynostosis
Beckwith–Wiedemann syndrome and uniparental disomy 11p: fine mapping of the recombination breakpoints and evaluation of several techniques
Clinical utility gene card for: Diamond Blackfan anemia
Genome-wide analysis of genetic susceptibility to language impairment in an isolated Chilean population
Genome-wide association study confirms extant PD risk loci among the Dutch
Homozygosity mapping in outbred families with mental retardation
Congenital muscular dystrophy type 1D (MDC1D) due to a large intragenic insertion/deletion, involving intron 10 of the LARGE gene
Association of a variant in the CHRNA5-A3-B4 gene cluster region to heavy smoking in the Italian population Italian population
Clinical utility gene card for: malignant hyperthermia
SUMF1 mutations affecting stability and activity of formylglycine generating enzyme predict clinical outcome in multiple sulfatase deficiency
Clinical utility gene card for: WAGR syndrome
Mitochondrial analysis of a Byzantine population reveals the differential impact of multiple historical events in South Anatolia
Dutch myotonic dystrophy type 2 patients and a North-African DM2 family carry the common European founder haplotype European founder haplotype; Dutch
Evidence for RPGRIP1 gene as risk factor for primary open angle glaucoma
Dissection of the MYCN locus in Feingold syndrome and isolated oesophageal atresia
Analysis of genetic deletions and duplications in the University College London bipolar disorder case control sample
Clinical utility gene card for: von Willebrand disease
An atypical case of hypomethylation at multiple imprinted loci