European Journal of Human Genetics - 2017

219 articles | Last updated: 2025-12-03 14:12:56
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Article Title Cauc. White Euro. Other Phrases Used
T A T A T A T A
From Mendel to Medical Genetics
Activities and initiatives of the renewed European Society of Human Genetics (ESHG) (1992–2017)
Those wonderful school days in Sestri Levante!
Secretary Generals on recent ESHG presidents (2003–2015)
Read's Recall: Shuffling abstracts - and foundations
The annual meeting 1988–2017
Commemoration of 15 years ESHG SPC member and chair from 2009 to 2016
Involving the European National Human Genetics Societies European National Human Genetics Societies
The European Board of Medical Genetics: development of a professional registration system in Europe
The creation of the International Federation of Human Genetics Societies in 1995–1996
The growth of the IFHGS after 2000
Recognition of clinical genetics in Europe
The development of the public and professional policy committee
The ESHG’s second quarter century: consolidation and growth—the period covering 1992–2017
The early years of the ESHG leading to the reform of 1988 and the spirit of the Sestri Levante school
The inner life and structure of ESHG
The development and growth of EJHG 1995–2017
EuroGentest NoE, the ESHG, and genetic services
Dysmorphology and the ESHG
Regulatory variants of FOXG1 in the context of its topological domain organisation
CHARGE syndrome: a recurrent hotspot of mutations in CHD7 IVS25 analyzed by bioinformatic tools and minigene assays
Congenital embryonal rhabdomyosarcoma caused by heterozygous concomitant PTCH1 and PTCH2 germline mutations
A genotype-first approach identifies an intellectual disability-overweight syndrome caused by PHIP haploinsufficiency
Molecular and cellular issues of KMT2A variants involved in Wiedemann-Steiner syndrome
Bayesian analysis of genome-wide inflammatory bowel disease data sets reveals new risk loci
NMNAT1 variants cause cone and cone-rod dystrophy
Mucopolysaccharidosis at play?
19p13 microduplications encompassing NFIX are responsible for intellectual disability, short stature and small head circumference
Points to consider for laboratories reporting results from diagnostic genomic sequencing
Recent developments in genetics and medically assisted reproduction: from research to clinical applications
Mitochondrial genomes uncover the maternal history of the Pamir populations
The role of CNVs in the etiology of rare autosomal recessive disorders: the example of TRAPPC9-associated intellectual disability
Rules for processing genetic data for research purposes in view of the new EU General Data Protection Regulation
HUWE1 variants cause dominant X-linked intellectual disability: a clinical study of 21 patients
One small edit for humans, one giant edit for humankind? Points and questions to consider for a responsible way forward for gene editing in humans
Early speech development in Koolen de Vries syndrome limited by oral praxis and hypotonia
Expanding the phenotype of metabolic cutis laxa with an additional disorder of N-linked protein glycosylation
Common origin of the gelsolin gene variant in 62 Finnish AGel amyloidosis families Finnish
The International Rare Diseases Research Consortium: Policies and Guidelines to maximize impact
Care and cost consequences of pediatric whole genome sequencing compared to chromosome microarray
Exome Pool-Seq in neurodevelopmental disorders
Decision making and experiences of young adults undergoing presymptomatic genetic testing for familial cancer: a longitudinal grounded theory study
The many “I” inside of “Me”
Deep characterization of a common D4Z4 variant identifies biallelic DUX4 expression as a modifier for disease penetrance in FSHD2 European population
A novel homozygous ARL13B variant in patients with Joubert syndrome impairs its guanine nucleotide-exchange factor activity
Next-generation sequencing of patients with congenital anosmia
Patients with a Kabuki syndrome phenotype demonstrate DNA methylation abnormalities
WISExome: a within-sample comparison approach to detect copy number variations in whole exome sequencing data
Contribution of germline deleterious variants in the RAD51 paralogs to breast and ovarian cancers
Immunoglobulin therapy ameliorates the phenotype and increases lifespan in the severely affected dystrophin–utrophin double knockout mice
Repeat genetic testing with targeted capture sequencing in primary arrhythmia syndrome and cardiomyopathy
Erratum: Responsible implementation of expanded carrier screening
The TAD-pathway for GWAS signals
Erratum: 22 Years of predictive testing for Huntington's disease: the experience of the UK Huntington's Prediction Consortium
Erratum: Glaucoma spectrum and age-related prevalence of individuals with FOXC1 and PITX2 variants
Erratum: Reconstructing the population history of the largest tribe of India: the Dravidian speaking Gond
Population-specific genetic variation in large sequencing data sets: why more data is still better
Mutations in RARS cause a hypomyelination disorder akin to Pelizaeus–Merzbacher disease
Lysenko and Russian genetics: Reply to Wang & Liu
Identification of causative variants in TXNL4A in Burn-McKeown syndrome and isolated choanal atresia
Detecting splicing patterns in genes involved in hereditary breast and ovarian cancer
Lysenko and Russian genetics: an alternative view
Acceptable applications of preimplantation genetic diagnosis (PGD) among Israeli PGD users
Identification of ASAH1 as a susceptibility gene for familial keloids
Unraveling genetic predisposition to familial or early onset gastric cancer using germline whole-exome sequencing
Autosomal recessive congenital ichthyosis: CERS3 mutations identified by a next generation sequencing panel targeting ichthyosis genes
Propionic acidemia as a cause of adult-onset dilated cardiomyopathy
Genetic correlations between intraocular pressure, blood pressure and primary open-angle glaucoma: a multi-cohort analysis
NDUFAF4 variants are associated with Leigh syndrome and cause a specific mitochondrial complex I assembly defect
The wide spectrum of POT1 gene variants correlates with multiple cancer types
The Y chromosome: a blueprint for men’s health?
Viewing the male-specific chromosome Y in a new light
Analysis of centrosome and DNA damage response in PLK4 associated Seckel syndrome
Participation in interdisciplinary meetings on genetic diagnostics (NGS)
Uptake of genetic testing by the children of Lynch syndrome variant carriers across three generations
A clinically driven variant prioritization framework outperforms purely computational approaches for the diagnostic analysis of singleton WES data
Massive sequencing of 70 genes reveals a myriad of missing genes or mechanisms to be uncovered in hereditary spastic paraplegias
SweGen: a whole-genome data resource of genetic variability in a cross-section of the Swedish population
Haplotype-based stratification of Huntington's disease
Third party interpretation of raw genetic data: an ethical exploration
Accurate and comprehensive analysis of single nucleotide variants and large deletions of the human mitochondrial genome in DNA and single cells
Decreased male reproductive success in association with mitochondrial dysfunction
Autosomal recessive cone-rod dystrophy can be caused by mutations in the ATF6 gene
Implicating candidate genes at GWAS signals by leveraging topologically associating domains
Mutation in the COX4I1 gene is associated with short stature, poor weight gain and increased chromosomal breaks, simulating Fanconi anemia
Recontacting in clinical practice: the views and expectations of patients in the United Kingdom
Deep intronic hotspot variant explaining rhabdoid tumor predisposition syndrome in two patients with atypical teratoid and rhabdoid tumor
Co-inheritance of mutations associated with arrhythmogenic cardiomyopathy and hypertrophic cardiomyopathy
Wilms tumour in Beckwith–Wiedemann Syndrome and loss of methylation at imprinting centre 2: revisiting tumour surveillance guidelines
Transparency of genetic testing services for ‘health, wellness and lifestyle’: analysis of online prepurchase information for UK consumers
Japan’s initiative on rare and undiagnosed diseases (IRUD): towards an end to the diagnostic odyssey
Clinical Utility Gene Card for hereditary angioedema with normal C1 inhibitor (HAEnC1)
Sexual dimorphism in the genetic influence on human childlessness
Genetically predicted high body mass index is associated with increased gastric cancer risk
New insights into the imprinted MEG8-DMR in 14q32 and clinical and molecular description of novel patients with Temple syndrome
Non-coding variants contribute to the clinical heterogeneity of TTR amyloidosis
Phenotypic extremes of BICD2-opathies: from lethal, congenital muscular atrophy with arthrogryposis to asymptomatic with subclinical features
Yunis-Varón syndrome caused by biallelic VAC14 mutations
Rare RNF213 variants in the C-terminal region encompassing the RING-finger domain are associated with moyamoya angiopathy in Caucasians
A novel de novo mutation in MYT1, the unique OAVS gene identified so far
Sex chromosome aneuploidies and copy-number variants: a further explanation for neurodevelopmental prognosis variability?
Coexisting variants in OSTM1 and MANEAL cause a complex neurodegenerative disorder with NBIA-like brain abnormalities
Variant in the X-chromosome spliceosomal gene GPKOW causes male-lethal microcephaly with intrauterine growth restriction
‘Absolute clarity’ and how to achieve it
Attitudes, knowledge and consequences of uncertain genetic findings in hypertrophic cardiomyopathy
Thorough discussion of cancer research—thoughts against the main stream
Be prepared for prenatal diagnosis
Expanded carrier screening: what determines intended participation and can this be influenced by message framing and narrative information?
A comparison of Cox and logistic regression for use in genome-wide association studies of cohort and case-cohort design
Genotype and phenotype spectrum of NRAS germline variants
Cerebellar hypoplasia with endosteal sclerosis is a POLR3-related disorder
Maternal heterozygous NLRP7 variant results in recurrent reproductive failure and imprinting disturbances in the offspring
England uses a competency-based approach to consent for health interventions
Reply to C Harling
A rare-variant test for high-dimensional data
Clinical utility gene card for: Tangier disease
Characterization of pathogenic SORL1 genetic variants for association with Alzheimer’s disease: a clinical interpretation strategy
The last sea nomads of the Indonesian archipelago: genomic origins and dispersal
Defects in the acid phosphatase ACPT cause recessive hypoplastic amelogenesis imperfecta
Recurrent somatic JAK-STAT pathway variants within a RUNX1-mutated pedigree
The perceived impact of the European registration system for genetic counsellors and nurses
Glaucoma spectrum and age-related prevalence of individuals with FOXC1 and PITX2 variants
Insertion of Alu elements at a PTEN hotspot in Cowden syndrome
Application of rare variant transmission disequilibrium tests to epileptic encephalopathy trio sequence data
Exome sequencing of two Italian pedigrees with non-isolated Chiari malformation type I reveals candidate genes for cranio-facial development
Complementarity between medical geneticists and genetic counsellors: its added value in genetic services in Europe
Variants in TTC25 affect autistic trait in patients with autism spectrum disorder and general population
Haploinsufficiency of ZNF462 is associated with craniofacial anomalies, corpus callosum dysgenesis, ptosis, and developmental delay
‘Inherited Metabolic Disease in Adults: A Clinical Guide’
A missense variant in ITPR1 provides evidence for autosomal recessive SCA29 with asymptomatic cerebellar hypoplasia in carriers
Progressive hereditary spastic paraplegia caused by a homozygous KY mutation
Exome sequencing revealed a splice site variant in the IQCE gene underlying post-axial polydactyly type A restricted to lower limb
The European Society of Human Genetics: beginnings, early history and development over its first 25 years
Uninformed consent in nutrigenomic research
Some pioneers of European human genetics
Comparison of the efficiency of colorectal cancer screening programs based on age and genetic risk for reduction of colorectal cancer mortality
Practices and views of neurologists regarding the use of whole-genome sequencing in clinical settings: a web-based survey
Introduction to the first ESHG/EJHG Anniversary issue
UK National Screening Committee Criteria: clarification of two misunderstandings
Novel SLC25A32 mutation in a patient with a severe neuromuscular phenotype
Reply to A Mackie
The RUDY study: using digital technologies to enable a research partnership
Haploinsufficiency for ANKRD11-flanking genes makes the difference between KBG and 16q24.3 microdeletion syndromes: 12 new cases
Variants in CPLX1 in two families with autosomal-recessive severe infantile myoclonic epilepsy and ID
Gene-set analysis shows association between FMRP targets and autism spectrum disorder
Erratum: Legal approaches regarding health-care decisions involving minors: implications for next-generation sequencing
Selected advances in genetics—cream of the crop
How to manage large-scale collaborative genomics research projects?
A unique haplotype of RCCX copy number variation: from the clinics of congenital adrenal hyperplasia to evolutionary genetics
Missing heritability: is the gap closing? An analysis of 32 complex traits in the Lifelines Cohort Study
An efficient and flexible test for rare variant effects
Improved imputation accuracy of rare and low-frequency variants using population-specific high-coverage WGS-based imputation reference panel
Development and clinical utility of a novel diagnostic nystagmus gene panel using targeted next-generation sequencing
Genetic variants associated with type 2 diabetes and adiposity and risk of intracranial and abdominal aortic aneurysms
Severe neurodegeneration, progressive cerebral volume loss and diffuse hypomyelination associated with a homozygous frameshift mutation in CSTB
Validation of copy number variation analysis for next-generation sequencing diagnostics
Upstream SLC2A1 translation initiation causes GLUT1 deficiency syndrome
Clinical utility gene card for: Fabry disease – update 2016
Analysis of exome data for 4293 trios suggests GPI-anchor biogenesis defects are a rare cause of developmental disorders
From older to younger: intergenerational promotion of health behaviours in Portuguese families affected by familial amyloid polyneuropathy
Genotypic spectrum and phenotype correlations of ABCA4-associated disease in patients of south Asian descent
PUF60 variants cause a syndrome of ID, short stature, microcephaly, coloboma, craniofacial, cardiac, renal and spinal features
Insights from early experience of a Rare Disease Genomic Medicine Multidisciplinary Team: a qualitative study
The role of genetic counsellors in genomic healthcare in the United Kingdom: a statement by the Association of Genetic Nurses and Counsellors
Personal utility in genomic testing: a systematic literature review
A mutation in the TMEM65 gene results in mitochondrial myopathy with severe neurological manifestations
Deciphering the mechanism of Q145H SFTPC mutation unmasks a splicing defect and explains the severity of the phenotype
Exome sequencing identifies primary carnitine deficiency in a family with cardiomyopathy and sudden death
Gonadal mosaicism of a novel IQSEC2 variant causing female limited intellectual disability and epilepsy
A novel recessive TTN founder variant is a common cause of distal myopathy in the Serbian population
Mutations in the PCYT1A gene are responsible for isolated forms of retinal dystrophy
Ancient mitochondrial lineages support the prehistoric maternal root of Basques in Northern Iberian Peninsula
European registration process for Clinical Laboratory Geneticists in genetic healthcare
Paternity testing under the cloak of recreational genetics
Prediction of years of life after diagnosis of breast cancer using omics and omic-by-treatment interactions
Choices for return of primary and secondary genomic research results of 790 members of families with Mendelian disease
Genetics of the peloponnesean populations and the theory of extinction of the medieval peloponnesean Greeks
Non-invasive prenatal diagnosis of beta-thalassemia by semiconductor sequencing: a feasibility study in the sardinian population Sardinian population
Partial duplication of the CRYBB1-CRYBA4 locus is associated with autosomal dominant congenital cataract
tRNA N6-adenosine threonylcarbamoyltransferase defect due to KAE1/TCS3 (OSGEP) mutation manifest by neurodegeneration and renal tubulopathy
Diagnostic exome sequencing in 266 Dutch patients with visual impairment
Prevalence of congenital amusia
MCM5: a new actor in the link between DNA replication and Meier-Gorlin syndrome
Coffee consumption is associated with DNA methylation levels of human blood
Erratum: Mitochondrial encephalomyopathy and retinoblastoma explained by compound heterozygosity of SUCLA2 point mutation and 13q14 deletion
Erratum: Clinical exome sequencing for cerebellar ataxia and spastic paraplegia uncovers novel gene–disease associations and unanticipated rare disorders
A fast and accurate method for detection of IBD shared haplotypes in genome-wide SNP data
Intragenic FMR1 disease-causing variants: a significant mutational mechanism leading to Fragile-X syndrome
Recontacting in clinical genetics and genomic medicine? We need to talk about it
Securing the use of existing sample collections for future human genetic research
Two novel BMP-2 variants identified in patients with thoracic ossification of the ligamentum flavum
Whole-genome view of the consequences of a population bottleneck using 2926 genome sequences from Finland and United Kingdom
Collagen synthesis disruption and downregulation of core elements of TGF-β, Hippo, and Wnt pathways in keratoconus corneas
Variability in assigning pathogenicity to incidental findings: insights from LDLR sequence linked to the electronic health record in 1013 individuals
Reconstructing the population history of the largest tribe of India: the Dravidian speaking Gond
Evaluation of copy-number variants as modifiers of breast and ovarian cancer risk for BRCA1 pathogenic variant carriers
Loss of Function of KCNC1 is associated with intellectual disability without seizures
Non-invasive prenatal diagnosis of spinal muscular atrophy by relative haplotype dosage
Psychosocial effects in parents and children 12 years after newborn genetic screening for type 1 diabetes
From exomes to genomes: challenges and solutions in population-based genetic association studies
Signatures of human European Palaeolithic expansion shown by resequencing of non-recombining X-chromosome segments human European Palaeolithic expansion
Suggestive association between variants in IL1RAPL and asthma symptoms in Latin American children
Characterising private and shared signatures of positive selection in 37 Asian populations
A survey of sub-Saharan gene flow into the Mediterranean at risk loci for coronary artery disease
Clinical utility gene card for: Non-Syndromic Microphthalmia Including Next-Generation Sequencing-Based Approaches
Juvenile myelomonocytic leukemia-associated variants are associated with neo-natal lethal Noonan syndrome
Of dogs and men
Association analysis of dyslexia candidate genes in a Dutch longitudinal sample
Post-mortem whole-exome analysis in a large sudden infant death syndrome cohort with a focus on cardiovascular and metabolic genetic diseases
Clinical utility gene card for: Cantú syndrome
Recontacting in clinical practice: an investigation of the views of healthcare professionals and clinical scientists in the United Kingdom
Identification and population history of CYP4V2 mutations in patients with Bietti crystalline corneoretinal dystrophy
Reply to Walsh et al
FIG4 variants in central European patients with amyotrophic lateral sclerosis: a whole-exome and targeted sequencing study central European
Genome-wide association scan of neuropathic pain symptoms post total joint replacement highlights a variant in the protein-kinase C gene
Update on a previously reported male with a FLNA missense mutation
Novel homozygous missense variant of GRIN1 in two sibs with intellectual disability and autistic features without epilepsy
Psychological and behavioural impact of returning personal results from whole-genome sequencing: the HealthSeq project
Severe neurodegenerative disease in brothers with homozygous mutation in POLR1A