European Journal of Human Genetics - 2010

237 articles | Last updated: 2025-12-03 14:12:56
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Mutation in mitochondrial ribosomal protein MRPS22 leads to Cornelia de Lange-like phenotype, brain abnormalities and hypertrophic cardiomyopathy
Splicing mutations in glycogen-storage disease type II: evaluation of the full spectrum of mutations and their relation to patients’ phenotypes
A candidate gene study of the type I interferon pathway implicates IKBKE and IL8 as risk loci for SLE
Concurrent genetic alterations in DNA polymerase proofreading and mismatch repair in human colorectal cancer
SGCE isoform characterization and expression in human brain: implications for myoclonus–dystonia pathogenesis?
Arrayed primer extension technology simplifies mutation detection in Bardet–Biedl and Alström syndrome
Relation of a common variant of the adiponectin gene to serum adiponectin concentration and metabolic traits in an aged Japanese population
Clinical utility gene card for: Silver–Russell syndrome
Replication of genetic variants from genome-wide association studies with metabolic traits in an island population of the Adriatic coast of Croatia
Phenotypic manifestations of copy number variation in chromosome 16p13.11
Tumour-specific methylation of PTPRG intron 1 locus in sporadic and Lynch syndrome colorectal cancer
Association between ORMDL3, IL1RL1 and a deletion on chromosome 17q21 with asthma risk in Australia
EPIBLASTER-fast exhaustive two-locus epistasis detection strategy using graphical processing units
A novel, non-stop mutation in FOXE3 causes an autosomal dominant form of variable anterior segment dysgenesis including Peters anomaly
A genome-wide analysis of population structure in the Finnish Saami with implications for genetic association studies
Clinical utility gene card for: Wolf–Hirschhorn (4p-) syndrome
Clinical utility gene card for: Laing distal myopathy
Clinical utility gene card for: Deletion 22q13 syndrome
Clinical utility gene card for: Bardet–Biedl syndrome
Clinical utility gene card for: Retinoblastoma
NDUFA10 mutations cause complex I deficiency in a patient with Leigh disease
Distinctive microRNA expression profiles in CD34+ bone marrow cells from patients with myelodysplastic syndrome
Prevalence of CDKN2A mutations in pancreatic cancer patients: implications for genetic counseling
Visual arts and genetics: lessons from the past
Integrated analysis of clinical signs and literature data for the diagnosis and therapy of a previously undescribed 6p21.3 deletion syndrome
Personal genetics: regulatory framework in Europe from a service provider's perspective
Influences of history, geography, and religion on genetic structure: the Maronites in Lebanon
Reply to He et al
MECP2 duplications in six patients with complex sex chromosome rearrangements
A population-based study of polymorphisms in genes related to sex hormones and abdominal aortic aneurysm
On the origin and diffusion of BRCA1 c.5266dupC (5382insC) in European populations European populations
Mutation in subdomain G' of mitochondrial elongation factor G1 is associated with combined OXPHOS deficiency in fibroblasts but not in muscle
Family-based association study of ITGB3 in autism spectrum disorder and its endophenotypes
Caution in generalizing known genetic risk markers for breast cancer across all ethnic/racial populations
A large homozygous deletion in the SAMHD1 gene causes atypical Aicardi–Goutiéres syndrome associated with mtDNA deletions
Experiences with array-based sequence capture; toward clinical applications
Discriminative accuracy of genomic profiling comparing multiplicative and additive risk models
Does the HSD17B10 gene escape from X-inactivation?
LTBP2 and CYP1B1 mutations and associated ocular phenotypes in the Roma/Gypsy founder population Roma/Gypsy founder population
Comprehensive fine mapping of chr12q12-14 and follow-up replication identify activin receptor 1B (ACVR1B) as a muscle strength gene
The clinical spectrum of complete FBN1 allele deletions
Mitochondrial dysfunction and organic aciduria in five patients carrying mutations in the Ras-MAPK pathway
Mitochondrial cardiomyopathies: how to identify candidate pathogenic mutations by mitochondrial DNA sequencing, MITOMASTER and phylogeny
Clinical utility gene card for: hypophosphatasia
Advanced age increases chromosome structural abnormalities in human spermatozoa
Erratum: Corrigendum to: EMQN Best Practice Guidelines for molecular genetic testing of SCAs
Genome-wide association of breast cancer: composite likelihood with imputed genotypes
Expanding the clinical spectrum of the 16p11.2 chromosomal rearrangements: three patients with syringomyelia
Clinical utility gene card for: Axenfeld–Rieger syndrome
Empowerment: qualitative underpinning of a new clinical genetics-specific patient-reported outcome
Gene-based interaction analysis by incorporating external linkage disequilibrium information
Estimating penetrance from multiple case families with predisposing mutations: extension of the ‘genotype-restricted likelihood’ (GRL) method
Does heritability hide in epistasis between linked SNPs?
Characterisation of TSC1 promoter deletions in tuberous sclerosis complex patients
The Y-chromosome landscape of the Philippines: extensive heterogeneity and varying genetic affinities of Negrito and non-Negrito groups
Erratum: The 2q23.1 microdeletion syndrome: clinical and behavioural phenotype
An account of our past to inform our future
Extended extraocular phenotype of PROM1 mutation in kindreds with known autosomal dominant macular dystrophy
Rapid testing versus karyotyping in Down's syndrome screening: cost-effectiveness and detection of clinically significant chromosome abnormalities
In the heartland of Eurasia: the multilocus genetic landscape of Central Asian populations
Mutation rate estimates for 110 Y-chromosome STRs combining population and father–son pair data
Genomic analysis of partial 21q monosomies with variable phenotypes
Associations of Y-chromosome subdeletion gr/gr with the prevalence of Y-chromosome haplogroups in infertile patients
Duplication of MAOA, MAOB, and NDP in a patient with mental retardation and epilepsy
Genome-wide association study with DNA pooling identifies variants at CNTNAP2 associated with pseudoexfoliation syndrome
Statement of the ESHG on direct-to-consumer genetic testing for health-related purposes
Preparation and validation of the first WHO international genetic reference panel for Fragile X syndrome
Duplications of FOXG1 in 14q12 are associated with developmental epilepsy, mental retardation, and severe speech impairment
A major Y-chromosome haplogroup R1b Holocene era founder effect in Central and Western Europe
A genomic analysis identifies a novel component in the genetic structure of sub-Saharan African populations
Sex chromosome trisomies in Europe: prevalence, prenatal detection and outcome of pregnancy
Distinguishing the 4qA and 4qB variants is essential for the diagnosis of facioscapulohumeral muscular dystrophy in the Chinese population Chinese population
Erratum: Corrigendum to: Sporadic cases are the norm for complex disease
Erratum: Therapeutic exon skipping for dysferlinopathies?
The EuroGentest Clinical Utility Gene Cards
Approaches to quality management and accreditation in a genetic testing laboratory
Erratum: Separating the post-Glacial coancestry of European and Asian Y chromosomes within haplogroup R1a European and Asian
Erratum: Corrigendum to: Linkage and candidate gene studies of autism spectrum disorders in European populations European populations
Exon deletions of the EP300 and CREBBP genes in two children with Rubinstein–Taybi syndrome detected by aCGH
FGF21 signalling pathway and metabolic traits – genetic association analysis
FTO genotype and adiposity in children: physical activity levels influence the effect of the risk genotype in adolescent males
p.Ser1235Arg should no longer be considered as a cystic fibrosis mutation: results from a large collaborative study
Alternative splice variants of the USH3A gene Clarin 1 (CLRN1)
Characterization of novel SLC6A8 variants with the use of splice-site analysis tools and implementation of a newly developed LOVD database
Further delineation of the phenotype of severe congenital neutropenia type 4 due to mutations in G6PC3
Cervix smear abnormalities: linking pathology data in female twins, their mothers and sisters
Type II familial synpolydactyly: report on two families with an emphasis on variations of expression
Genetic marker polymorphisms on chromosome 8q24 and prostate cancer in the Dutch population: DG8S737 may not be the causative variant Dutch population
Genetic diversity on the Comoros Islands shows early seafaring as major determinant of human biocultural evolution in the Western Indian Ocean
Next generation sequencing in a family with autosomal recessive Kahrizi syndrome (OMIM 612713) reveals a homozygous frameshift mutation in SRD5A3
Nicotinic α5 receptor subunit mRNA expression is associated with distant 5′ upstream polymorphisms
Disruption of the ATP8A2 gene in a patient with a t(10;13) de novo balanced translocation and a severe neurological phenotype
Genetic variability at the PARK16 locus
Erratum to: Stickler syndrome caused by COL2A1 mutations: genotype–phenotype correlation in a series of 100 patients
Hemizygous deletion of COL3A1, COL5A2, and MSTN causes a complex phenotype with aortic dissection: a lesson for and from true haploinsufficiency
Comparing family members’ motivations and attitudes towards genetic testing for hereditary breast and ovarian cancer: a qualitative analysis
Genotype–phenotype relationship in three cases with overlapping 19p13.12 microdeletions
Potential role of TCF7L2 gene variants on cardiac sympathetic/parasympathetic activity
Hypothetical and factual willingness to participate in biobank research
Clinical utility gene card for: Lesch–Nyhan syndrome
Clinical utility gene card for: Holoprosencephaly
Gene expression associated with the onset of hearing detected by differential display in rat organ of Corti
Confirmation of association between multiple sclerosis and CYP27B1
Mesomelic dysplasia Kantaputra type is associated with duplications of the HOXD locus on chromosome 2q
Genetic male infertility and mutation of CATSPER ion channels
X-inactivation of HSD17B10 revealed by cDNA analysis in two female patients with 17β-hydroxysteroid dehydrogenase 10 deficiency
A standardized framework for the validation and verification of clinical molecular genetic tests
Rubinstein–Taybi syndrome (CREBBP, EP300)
The role of disease perceptions and results sharing in psychological adaptation after genetic susceptibility testing: the REVEAL Study
Lack of association between a new tag SNP in the FTO gene and BMI in Czech–Slavonic population Czech–Slavonic population
NordicDB: a Nordic pool and portal for genome-wide control data
Reply to Dlouha et al
The PSEN1 I143T mutation in a Swedish family with Alzheimer's disease: clinical report and quantification of Aβ in different brain regions
Population structure and genome-wide patterns of variation in Ireland and Britain
Chadic languages and Y haplogroups
A single nucleotide polymorphism in APOA5 determines triglyceride levels in Hong Kong and Guangzhou Chinese
A fourth locus for autosomal dominant hypercholesterolemia maps at 16q22.1
Chiari I malformation, delayed gross motor skills, severe speech delay, and epileptiform discharges in a child with FOXP1 haploinsufficiency
Reply to Lancaster
Carrier screening for Beta-thalassaemia: a review of international practice
Genes predict village of origin in rural Europe
Genome-wide gene expression profiling of the Angelman syndrome mice with Ube3a mutation
Paternally inherited microdeletion at 15q11.2 confirms a significant role for the SNORD116 C/D box snoRNA cluster in Prader–Willi syndrome
Genome-wide scan identifies a quantitative trait locus at 4p15.3 for serum urate
Dengue hemorrhagic fever is associated with polymorphisms in JAK1
The medical geneticist as expert in the transgenerational and developmental aspects of diseases
Erratum: Corrigendum to: Common genetic variation and performance on standardized cognitive tests
Erratum: Corrigendum to: Human Y chromosome haplogroup R-V88: a paternal genetic record of early mid Holocene trans-Saharan connections and the spread of Chadic languages
5q11.2 deletion in a patient with tracheal agenesis
Familial neonatal isolated cardiomyopathy caused by a mutation in the flavoprotein subunit of succinate dehydrogenase
Co-expression of FBN1 with mesenchyme-specific genes in mouse cell lines: implications for phenotypic variability in Marfan syndrome
CAMOS, a nonprogressive, autosomal recessive, congenital cerebellar ataxia, is caused by a mutant zinc-finger protein, ZNF592
A qualitative study exploring genetic counsellors’ experiences of counselling children
The unfolding clinical spectrum of holoprosencephaly due to mutations in SHH, ZIC2, SIX3 and TGIF genes
Reply to Lévy et al
A novel recessive GUCY2D mutation causing cone–rod dystrophy and not Leber's congenital amaurosis
Dietary anaplerotic therapy improves peripheral tissue energy metabolism in patients with Huntington's disease
Therapeutic exon ‘switching’ for dysferlinopathies?
Clinical utility gene card for: HMSN/HNPP HMSN types 1, 2, 3, 6 (CMT1,2,4, DSN, CHN, GAN, CCFDN, HNA); HNPP
Clinical utility gene card for: MUTYH-associated polyposis (MAP), autosomal recessive colorectal adenomatous polyposis
Notable contribution of large CFTR gene rearrangements to the diagnosis of cystic fibrosis in fetuses with bowel anomalies
The obesity-associated SNPs in intron 1 of the FTO gene affect primary transcript levels
Uptake of carrier testing in families after cystic fibrosis diagnosis through newborn screening
New RAB3GAP1 mutations in patients with Warburg Micro Syndrome from different ethnic backgrounds and a possible founder effect in the Danish different ethnic backgrounds; Danish
Deletion of MAOA and MAOB in a male patient causes severe developmental delay, intermittent hypotonia and stereotypical hand movements
A powerful score test to detect positive selection in genome-wide scans
Different distribution of the genetic subtypes of the Prader–Willi syndrome in the elderly
A total of 220 patients with autosomal dominant spastic paraplegia do not display mutations in the SLC33A1 gene (SPG42)
High frequency of COH1 intragenic deletions and duplications detected by MLPA in patients with Cohen syndrome
Genomic profile of copy number variants on the short arm of human chromosome 8
A screening methodology based on Random Forests to improve the detection of gene–gene interactions
Evidence for both copy number and allelic (NA1/NA2) risk at the FCGR3B locus in systemic lupus erythematosus
Mutation R184Q of connexin 26 in hearing loss patients has a dominant-negative effect on connexin 26 and connexin 30
Molecular characterization of SMN copy number derived from carrier screening and from core families with SMA in a Chinese population
The power of the Transmission Disequilibrium Test in the presence of population stratification
Pelizaeus–Merzbacher-like disease is caused not only by a loss of connexin47 function but also by a hemichannel dysfunction
DNA methylation profiling of human placentas reveals promoter hypomethylation of multiple genes in early-onset preeclampsia
Genetic education and the challenge of genomic medicine: development of core competences to support preparation of health professionals in Europe
Linkage and candidate gene studies of autism spectrum disorders in European populations European populations
Follow-up of potential novel Graves' disease susceptibility loci, identified in the UK WTCCC genome-wide nonsynonymous SNP study
Genome-wide gene and pathway analysis
Recurrent 70.8 Mb 4q22.2q32.3 duplication due to ovarian germinal mosaicism
A pure likelihood approach to the analysis of genetic association data: an alternative to Bayesian and frequentist analysis
A simple Bayesian mixture model with a hybrid procedure for genome-wide association studies
Risk of breast and prostate cancer is not associated with increased homozygosity in outbred populations
Genome-wide linkage analyses of hereditary prostate cancer families with colon cancer provide further evidence for a susceptibility locus on 15q11–q14
A genome-wide linkage scan reveals CD53 as an important regulator of innate TNF-α levels
Leena Palotie-Peltonen, 1952–2010, visionary and role model
Erratum: Corrigendum to: CASK mutations are frequent in males and cause X-linked nystagmus and variable XLMR phenotypes
Altered TGFβ signaling and cardiovascular manifestations in patients with autosomal recessive cutis laxa type I caused by fibulin-4 deficiency
Linking LINGO1 to essential tremor
Replication of the LINGO1 gene association with essential tremor in a North American population
A sequence variant on 17q21 is associated with age at onset and severity of asthma
Clinical utility gene card for: Marfan syndrome type 1 and related phenotypes [FBN1]
Early onset and slow progression of SCA28, a rare dominant ataxia in a large four-generation family with a novel AFG3L2 mutation
Linkage disequilibrium and age of HLA region SNPs in relation to classic HLA gene alleles within Europe within Europe
Detection of susceptibility genes as modifiers due to subgroup differences in complex disease
Usefulness of factor V Leiden mutation testing in clinical practice
Retrospective access to data: the ENGAGE consent experience
Erratum: Drawing the history of the Hutterite population on a genetic landscape: inference from Y-chromosome and mtDNA genotypes
Erratum: Differential MSH2 promoter methylation in blood cells of Neurofibromatosis type 1 (NF1) patients
Heterozygous deletion of a 2-Mb region including the dystroglycan gene in a patient with mild myopathy, facial hypotonia, oral-motor dyspraxia and white matter abnormalities
What is a meaningful result? Disclosing the results of genomic research in autism to research participants
Linking the sub-Saharan and West Eurasian gene pools: maternal and paternal heritage of the Tuareg nomads from the African Sahel West Eurasian; sub-Saharan
Replication of past candidate loci for common diseases and phenotypes in 100 genome-wide association studies
The BRCAPRO 5.0 model is a useful tool in genetic counseling and clinical management of male breast cancer cases
Quality standards in Biobanking: authentication by genetic profiling of blood spots from donor's original sample
GWAS: heritability missing in action?
TTR familial amyloid polyneuropathy: does a mitochondrial polymorphism entirely explain the parent-of-origin difference in penetrance?
Italian appeal court: a genetic predisposition to commit murder?
The p.A897KfsX4 frameshift variation in desmocollin-2 is not a causative mutation in arrhythmogenic right ventricular cardiomyopathy
Reconsidering reproductive benefit through newborn screening: a systematic review of guidelines on preconception, prenatal and newborn screening
The inhibition of mevalonate pathway induces upregulation of NALP3 expression: new insight in the pathogenesis of mevalonate kinase deficiency
RNA splicing meets genetic testing: detection and interpretation of splicing defects in genetic diseases
Evidence for interaction between 5-hydroxytryptamine (serotonin) receptor 2A and MHC type II molecules in the development of rheumatoid arthritis
Polymorphisms in the IL2, IL2RA and IL2RB genes in multiple sclerosis risk
A new set of markers for human identification based on 32 polymorphic Alu insertions
Bayes factors in complex genetics
EMQN Best Practice Guidelines for molecular genetic testing of SCAs
Consensus and controversies in best practices for molecular genetic testing of spinocerebellar ataxias
LTBP2 null mutations in an autosomal recessive ocular syndrome with megalocornea, spherophakia, and secondary glaucoma
Paternal origin of the de novo constitutional t(11;22)(q23;q11)
Stickler syndrome caused by COL2A1 mutations: genotype–phenotype correlation in a series of 100 patients
Research and applied medical genetics: filling the gap
‘Me, Myself, and Why. Understanding your own genome and evolutionary history’
Clinical utility gene card for: Ehlers–Danlos syndrome types I–VII
The USH2A c.2299delG mutation: dating its common origin in a Southern European population Southern European population
Evidence for CTLA4 as a susceptibility gene for dilated cardiomyopathy
Therapeutic exon skipping for dysferlinopathies?
Mammary-digital-nail (MDN) syndrome: a novel phenotype maps to human chromosome 22q12.3–13.1
Common genetic variation and performance on standardized cognitive tests
High frequency of copy number imbalances in Rubinstein–Taybi patients negative to CREBBP mutational analysis
Clinical utility gene card for: Haemochromatosis [HFE]
Clinical utility gene card for: DiGeorge syndrome, velocardiofacial syndrome, Shprintzen syndrome, chromosome 22q11.2 deletion syndrome (22q11.2, TBX1)
Clinical utility gene card for: Lynch syndrome (MLH1, MSH2, MSH6, PMS2)
Methylation analysis of 79 patients with growth restriction reveals novel patterns of methylation change at imprinted loci
Genotype–phenotype correlations in nonlethal osteogenesis imperfecta caused by mutations in the helical domain of collagen type I
An omnibus test for family-based association studies with multiple SNPs and multiple phenotypes
Two novel missense mutations in HAIRY-AND-ENHANCER-OF-SPLIT-7 in a family with spondylocostal dysostosis
Additional evidence to support the role of a common variant near the complement factor I gene in susceptibility to age-related macular degeneration
Association between genes on chromosome 4p16 and non-syndromic oral clefts in four populations
Genetic variants in the BDNF gene and therapeutic response to risperidone in schizophrenia patients: a pharmacogenetic study
Polymorphisms in the sialic acid-binding immunoglobulin-like lectin-8 (Siglec-8) gene are associated with susceptibility to asthma
Tag-SNP analysis of the GFI1-EVI5-RPL5-FAM69 risk locus for multiple sclerosis
An unbalanced translocation unmasks a recessive mutation in the follicle-stimulating hormone receptor (FSHR) gene and causes FSH resistance
Migrations are of all times
Erratum: Corrigendum to: The 2q23.1 microdeletion syndrome: clinical and behavioural phenotype
Erratum: Spectrum of CLCN1 mutations in patients with myotonia congenita in Northern Scandinavia
Preaxial polydactyly/triphalangeal thumb is associated with changed transcription factor-binding affinity in a family with a novel point mutation in the long-range cis-regulatory element ZRS
Use of a modeling framework to evaluate the effect of a modifier gene (MBL2) on variation in cystic fibrosis
A genome-wide association study for age-related hearing impairment in the Saami
Meta-analysis of 20 genome-wide linkage studies evidenced new regions linked to asthma and atopy
A new locus on 3p23–p25 for an autosomal-dominant limb-girdle muscular dystrophy, LGMD1H
Dyslexia and DCDC2: normal variation in reading and spelling is associated with DCDC2 polymorphisms in an Australian population sample
Differential decay of parent-of-origin-specific genomic sharing in cystic fibrosis-affected sib pairs maps a paternally imprinted locus to 7q34
Human Y chromosome haplogroup R-V88: a paternal genetic record of early mid Holocene trans-Saharan connections and the spread of Chadic languages
Frequent genetic differences between matched primary and metastatic breast cancer provide an approach to identification of biomarkers for disease progression