European Journal of Human Genetics - 2020

222 articles | Last updated: 2025-12-03 14:12:56
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T A T A T A T A
In cis TP53 and RAD51C pathogenic variants may predispose to sebaceous gland carcinomas
GertJan B. van Ommen 28 September 1947–7 November 2020
Examining the uptake of predictive BRCA testing in the UK; findings and implications
A human case of GIMAP6 deficiency: a novel primary immune deficiency
Chromosomes in the genomic age. Preserving cytogenomic competence of diagnostic genome laboratories
Informing about genetic risk in families with Huntington disease: comparison of attitudes across two decades
Speech and language phenotype in Phelan-McDermid (22q13.3) syndrome
SVA retrotransposon insertion in exon of MMR genes results in aberrant RNA splicing and causes Lynch syndrome
Correction: Immunity and mental illness: findings from a Danish population-based immunogenetic study of seven psychiatric and neurodevelopmental disorder
Correction: MobiDetails: online DNA variants interpretation
Atrial fibrillation—a complex polygenetic disease
Acquired somatic MMR deficiency is a major cause of MSI tumor in patients suspected for “Lynch-like syndrome” including young patients
Benefits of clinical criteria and high-throughput sequencing for diagnosing children with syndromic craniosynostosis
Autosomal genetics and Y-chromosome haplogroup L1b-M317 reveal Mount Lebanon Maronites as a persistently non-emigrating population
High-resolution population-specific recombination rates and their effect on phasing and genotype imputation non-Finnish Europeans (NFE)
Reflections on dynamic consent in biomedical research: the story so far
Whole-exome sequencing of Finnish patients with vascular cognitive impairment
Copy neutral loss of heterozygosity (cnLOH) patterns in synchronous colorectal cancer
Abstracts from the 53rd European Society of Human Genetics (ESHG) Conference: Interactive e-Posters
Abstracts from the 53rd European Society of Human Genetics (ESHG) Conference: Oral Presentations
Regarding the estimations of people affected by rare diseases
Abstracts from the 53rd European Society of Human Genetics (ESHG) Conference: e-Posters
Reply to E. Vicente et al.
Long-read trio sequencing of individuals with unsolved intellectual disability
Noninvasive prenatal test of single-gene disorders by linked-read direct haplotyping: application in various diseases
Parental exome analysis identifies shared carrier status for a second recessive disorder in couples with an affected child
Opportunistic genomic screening. Recommendations of the European Society of Human Genetics
Pathogenic variants causing ABL1 malformation syndrome cluster in a myristoyl-binding pocket and increase tyrosine kinase activity
Genotype-first approach to the detection of hereditary breast and ovarian cancer risk, and effects of risk disclosure to biobank participants
Biallelic PADI6 variants cause multilocus imprinting disturbances and miscarriages in the same family
The genetics of rod-cone dystrophy in Arab countries: a systematic review
A paternally inherited 1.4 kb deletion of the 11p15.5 imprinting center 2 is associated with a mild familial Silver–Russell syndrome phenotype
Enzymatic diagnosis of Pompe disease: lessons from 28 years of experience Asian pseudodeficiency
MobiDetails: online DNA variants interpretation
Pontocerebellar hypoplasia due to bi-allelic variants in MINPP1
Correction: Exome sequencing in infants with congenital hearing impairment: a population-based cohort study
A generic assay for the identification of splicing variants that induce nonsense-mediated decay in Pompe disease Asian
Novel FARS2 variants in patients with early onset encephalopathy with or without epilepsy associated with long survival
Subdividing Y-chromosome haplogroup R1a1 reveals Norse Viking dispersal lineages in Britain Western-European; Europe; European; peninsular Scandinavia; Norway; Sweden; British Isles; Britain; Norse Viking; Vikings
Estimation of the number of people with Down syndrome in Europe
An expanded analysis framework for multivariate GWAS connects inflammatory biomarkers to functional variants and disease
Measuring clinical utility in the context of genetic testing: a scoping review
Dissecting the paternal founders of Mundari (Austroasiatic) speakers associated with the language dispersal in South Asia
The clinical significance of A2ML1 variants in Noonan syndrome has to be reconsidered
X Chromosome inactivation: a modifier of factor VIII and IX plasma levels and bleeding phenotype in Haemophilia carriers
Regulatory landscape of providing information on newborn screening to parents across Europe
PAX6 missense variants in two families with isolated foveal hypoplasia and nystagmus: evidence of paternal postzygotic mosaicism
Perceptions of best practices for return of results in an international survey of psychiatric genetics researchers
Assessment and visualization of phenome-wide causal relationships using genetic data: an application to dental caries and periodontitis
Pleiotropic genomic variants at 17q21.31 associated with bone mineral density and body fat mass: a bivariate genome-wide association analysis
Secondary research use of personal medical data: attitudes from patient and population surveys in The Netherlands and Germany Dutch, German (nationalities)
Taste perception and lifestyle: insights from phenotype and genome data among Africans and Asians Africans and Asians
NAA10 p.(N101K) disrupts N-terminal acetyltransferase complex NatA and is associated with developmental delay and hemihypertrophy
Founder effect of the TTTCA repeat insertions in SAMD12 causing BAFME1
Hypophosphatasia: a genetic-based nosology and new insights in genotype-phenotype correlation
Correction: Intrafamilial communication of hereditary breast and ovarian cancer genetic information in Italian women: towards a personalised approach
A powerful new method for rare-variant analysis of quantitative traits in families
Homozygote loss-of-function variants in the human COCH gene underlie hearing loss
An epigenetic, transgenerational model of increased mental health disorders in children, adolescents and young adults
Effects of agalsidase-β administration on vascular function and blood pressure in familial Anderson–Fabry disease
Barriers and facilitators for cascade testing in genetic conditions: a systematic review
Identification of UBAP1 mutations in juvenile hereditary spastic paraplegia in the 100,000 Genomes Project
What is the meaning of a ‘genomic result’ in the context of pregnancy?
Intrafamilial communication of hereditary breast and ovarian cancer genetic information in Italian women: towards a personalised approach
A relatively common homozygous TRAPPC4 splicing variant is associated with an early-infantile neurodegenerative syndrome
Wilms tumor in patients with osteopathia striata with cranial sclerosis
Identification of disease-causing variants by comprehensive genetic testing with exome sequencing in adults with suspicion of hereditary FSGS
NGS-based spinal muscular atrophy carrier screening of 10,585 diverse couples in China: a pan-ethnic study
Sex-specific genetic effects across biomarkers
Genetic modifiers in rare disorders: the case of fragile X syndrome
Pathogenic deep intronic MTM1 variant activates a pseudo-exon encoding a nonsense codon resulting in severe X-linked myotubular myopathy
Successful application of genome sequencing in a diagnostic setting: 1007 index cases from a clinically heterogeneous cohort
Cancer surveillance for individuals with Li-Fraumeni syndrome
Reply to Kratz et al.
Perceptions of genetic testing in patients with hereditary chronic pancreatitis and their families: a qualitative triangulation
Correction: gr/gr deletion predisposes to testicular germ cell tumour independently from altered spermatogenesis: results from the largest European study European study
Variants of SOS2 are a rare cause of Noonan syndrome with particular predisposition for lymphatic complications
How will new genetic technologies, such as gene editing, change reproductive decision-making? Views of high-risk couples
Population prevalence and inheritance pattern of recurrent CNVs associated with neurodevelopmental disorders in 12,252 newborns and their parents
Non-invasive prenatal testing (NIPT): societal pressure or freedom of choice? A vignette study of Dutch citizens’ attitudes
A quantitative trait rare variant nonparametric linkage method with application to age-at-onset of Alzheimer’s disease
Improved HLA-based prediction of coeliac disease identifies two novel genetic interactions
National external quality assessment for next-generation sequencing-based diagnostics of primary immunodeficiencies
Rare heterozygous GDF6 variants in patients with renal anomalies
Confirmation of FZD5 implication in a cohort of 50 patients with ocular coloboma
Demographic and prosocial intrapersonal characteristics of biobank participants and refusers: the findings of a survey in the Netherlands
Accurate fetal variant calling in the presence of maternal cell contamination
Contributions of de novo variants to systemic lupus erythematosus
Differences in local population history at the finest level: the case of the Estonian population
Cultural variation impacts paternal and maternal genetic lineages of the Hmong-Mien and Sino-Tibetan groups from Thailand
COVID-19 and Down’s syndrome: are we heading for a disaster?
Secondary findings in inherited heart conditions: a genotype-first feasibility study to assess phenotype, behavioural and psychosocial outcomes
ACE2 gene variants may underlie interindividual variability and susceptibility to COVID-19 in the Italian population European country; Italian population; Asian countries
Molecular tumor testing in patients with Lynch-like syndrome reveals a de novo mosaic variant of a mismatch repair gene transmitted to offspring
Gene selection for the Australian Reproductive Genetic Carrier Screening Project (“Mackenzie’s Mission”)
Comparisons of screening strategies for identifying Lynch syndrome among patients with MLH1-deficient colorectal cancer
Identification of distinct transcriptome signatures of human adipose tissue from fifteen depots
Setting a new standard in cystic fibrosis newborn screening illustrates controversial issues as new data emerge
Expanding the phenotype associated to KMT2A variants: overlapping clinical signs between Wiedemann–Steiner and Rubinstein–Taybi syndromes
Harmful vimentin manifests itself as multiorgan failure
Novel candidate genes in esophageal atresia/tracheoesophageal fistula identified by exome sequencing
Determination of the phylogenetic origins of the Árpád Dynasty based on Y chromosome sequencing of Béla the Third Caucasus; Bashkirs; Hungarian; haplogroups (R-Z2125, R-Z2123, R-SUR51, R-ARP); Northern Afghanistan;
Overrepresentation of genetic variation in the AnkyrinG interactome is related to a range of neurodevelopmental disorders
Identification of genetic variants controlling RNA editing and their effect on RNA structure stabilization
Mainstreaming germline BRCA1/2 testing in non-mucinous epithelial ovarian cancer in the North West of England
Variants in the 5′UTR reduce SHOX expression and contribute to SHOX haploinsufficiency
Switch from enzyme replacement therapy to oral chaperone migalastat for treating fabry disease: real-life data
EMQN best practice guidelines for molecular genetic testing and reporting of 21-hydroxylase deficiency
Detection of copy-number variations from NGS data using read depth information: a diagnostic performance evaluation
Diphthamide-deficiency syndrome: a novel human developmental disorder and ribosomopathy
Taurine newborn screening to prevent one form of retinal degeneration and cardiomyopathy
Biallelic MFSD2A variants associated with congenital microcephaly, developmental delay, and recognizable neuroimaging features
SKOR1 has a transcriptional regulatory role on genes involved in pathways related to restless legs syndrome
Genetic study of the Arctic CPT1A variant suggests that its effect on fatty acid levels is modulated by traditional Inuit diet
Evaluation of CNV detection tools for NGS panel data in genetic diagnostics
Delivering genome sequencing for rapid genetic diagnosis in critically ill children: parent and professional views, experiences and challenges
Unique combination and in silico modeling of biallelic POLR3A variants as a cause of Wiedemann–Rautenstrauch syndrome
AAV-mediated FOXG1 gene editing in human Rett primary cells
Cancer Surveillance Guideline for individuals with PTEN hamartoma tumour syndrome
Web-based return of BRCA2 research results: one-year genetic counselling experience in Iceland
Digenic inheritance of subclinical variants in Noonan Syndrome patients: an alternative pathogenic model?
The effect of sample size on polygenic hazard models for prostate cancer
Is HSPG2 a modifier gene for Marfan syndrome?
Professional duties are now considered legal duties of care within genomic medicine
Inflammasome genetics and complex diseases: a comprehensive review
Characterisation of a second gain of function EDAR variant, encoding EDAR380R, in East Asia
The novel duplication HRAS c.186_206dup p.(Glu62_Arg68dup): clinical and functional aspects
Molecular characterization of a 1p36 chromosomal duplication and in utero interference define ENO1 as a candidate gene for polymicrogyria
Attitudes of relatives of mucopolysaccharidosis type III patients toward preconception expanded carrier screening
A second cohort of CHD3 patients expands the molecular mechanisms known to cause Snijders Blok-Campeau syndrome
National Newborn Screening for cystic fibrosis in the Republic of Ireland: genetic data from the first 6.5 years
Genetic aspects of congenital nephrotic syndrome: a consensus statement from the ERKNet–ESPN inherited glomerulopathy working group
Biallelic PDE2A variants: a new cause of syndromic paroxysmal dyskinesia
The genetic architecture of Stargardt macular dystrophy (STGD1): a longitudinal 40-year study in a genetic isolate
Judging in the genomic era: judges’ genetic knowledge, confidence and need for training
Guidelines for the Li–Fraumeni and heritable TP53-related cancer syndromes
Clinical and genetic characterization of nephropathy in patients with nail-patella syndrome
Medium-coverage DNA sequencing in the design of the genetic association study
Development and validation of a measure of comprehension of genomic screening—negative results (CoG-NR)
BBMRI-ERIC’s contributions to research and knowledge exchange on COVID-19
Editorial: Cutting Covid no slack
Quality of life drives patients’ preferences for secondary findings from genomic sequencing
Future-proofing biobanks’ governance
EMQN best practice guidelines for genetic testing in dystrophinopathies
Forming and ending marital or cohabiting relationships in a Danish population-based cohort of individuals with neurofibromatosis 1
SRD5A3 defective congenital disorder of glycosylation: clinical utility gene card
Connecting data, tools and people across Europe: ELIXIR’s response to the COVID-19 pandemic
The exhaustive genomic scan approach, with an application to rare-variant association analysis
Who ever heard of 16p11.2 deletion syndrome? Parents’ perspectives on a susceptibility copy number variation syndrome
The COVID-19 Host Genetics Initiative, a global initiative to elucidate the role of host genetic factors in susceptibility and severity of the SARS-CoV-2 virus pandemic
Predictive genetic testing in Huntington’s disease: should a neurologist be involved?
The VODAN IN: support of a FAIR-based infrastructure for COVID-19
Functional biology of the Steel syndrome founder allele and evidence for clan genomics derivation of COL27A1 pathogenic alleles worldwide Puerto Rico; Puerto Rican descent; Puerto Rican; Turkish kindreds; consanguineous Turkish kindreds;
Communicating genetic information to family members: analysis of consent forms for diagnostic genomic sequencing
What do we do and how do we do it? Assessing genetic counselling in the modern era
Cognitive and affective outcomes of genetic counselling in the Netherlands at group and individual level: a personalized approach seems necessary
Management of information within Portuguese families with Huntington disease: a transgenerational process for putting the puzzle together
Assessing the stability of biobank donor preferences regarding sample use: evidence supporting the value of dynamic consent
Association of congenital cardiovascular malformation and neuropsychiatric phenotypes with 15q11.2 (BP1–BP2) deletion in the UK Biobank
High rate of HDR in gene editing of p.(Thr158Met) MECP2 mutational hotspot
Using an integrative machine learning approach utilising homology modelling to clinically interpret genetic variants: CACNA1F as an exemplar
The uptake of predictive DNA testing in 40 families with a pathogenic BRCA1/BRCA2 variant. An evaluation of the proband-mediated procedure
The evolutionarily conserved function of TBR1 in controlling the size of anterior commissure in human and mouse brains
Reply to Hsueh YP et al.
The GDPR, secondary research purposes and global data sharing—one-wheel too many
CUGC for hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome
CUGC for lysinuric protein intolerance (LPI)
Transcript-specific regulation in T-cells in multiple sclerosis susceptibility
Towards a fine-scale picture of European genetic diversity European genetic diversity
A comparison of genomic diagnostics in adults and children with epilepsy and comorbid intellectual disability
Willingness to donate genomic and other medical data: results from Germany
Whole exome sequencing in ADHD trios from single and multi-incident families implicates new candidate genes and highlights polygenic transmission
First-line exome sequencing in Palestinian and Israeli Arabs with neurological disorders is efficient and facilitates disease gene discovery Palestinian and Israeli Arabs; Arab communities; German control cohort
Gene therapy regulation: could in-body editing fall through the net?
High prevalence of SMARCB1 constitutional abnormalities including mosaicism in malignant rhabdoid tumors
Self-reported hearing loss questions provide a good measure for genetic studies: a polygenic risk score analysis from UK Biobank "European and non-European samples"; "Europeans but not non-European subpopulations"; "ethnic background"
Co-existence of ABCB11 and DCDC2 disease: Infantile cholestasis requires both next-generation sequencing and clinical-histopathologic correlation
The intronic BRCA1 c.5407-25T>A variant causing partly skipping of exon 23—a likely pathogenic variant with reduced penetrance? non-Finnish Europeans
Correction: Comparison of methods for transcriptome imputation through application to two common complex diseases
How the “control-fate continuum” helps explain the genetic testing decision-making process: a grounded theory study
Familial adult myoclonic epilepsy type 1 SAMD12 TTTCA repeat expansion arose 17,000 years ago and is present in Sri Lankan and Indian families
Parental repeat length instability in myotonic dystrophy type 1 pre- and protomutations
Correction: The genetic history of France
De novo variants in CAMTA1 cause a syndrome variably associated with spasticity, ataxia, and intellectual disability
Common genetic variation in the Angelman syndrome imprinting centre affects the imprinting of chromosome 15
Second case of Bardet–Biedl syndrome caused by biallelic variants in IFT74
Detection and protection mechanisms against vulnerabilities are needed in blockchain applications
Disruptive and avoidable: GDPR challenges to secondary research uses of data
Inferring the population history of Tai-Kadai-speaking people and southernmost Han Chinese on Hainan Island by genome-wide array genotyping
Reply to Y. Takefuji
An intellectual disability syndrome with single-nucleotide variants in O-GlcNAc transferase
Incorporating information from markers in LD with test locus for detecting imprinting and maternal effects
Reviewer recognition
Primrose syndrome: a phenotypic comparison of patients with a ZBTB20 missense variant versus a 3q13.31 microdeletion including ZBTB20
Correction: Congenital adrenal hyperplasia due to 11-beta-hydroxylase deficiency: functional consequences of four CYP11B1 mutations
Broad consent in practice: lessons learned from a hospital-based biobank for prospective research on genomic and medical data
A dominant vimentin variant causes a rare syndrome with premature aging
Reply to C.D. Richter
Response to “Continental drift? Do European clinical genetic testing laboratories have a patent problem?”
Relationships between patient- and session-related variables and outcomes of psychiatric genetic counseling
Smith–Lemli–Opitz syndrome: what is the actual risk for couples carriers of the DHCR7:c.964-1G>C variant?
Evaluation of current genetic testing reports in German-speaking countries with regard to secondary use and future electronic implementation
Alternate approach to stroke phenotyping identifies a genetic risk locus for small vessel stroke
Cryptic exon activation causes dystrophinopathy in two Chinese families
The genetic history of France
Recommendations for designing genetic test reports to be understood by patients and non-specialists
Clinical genomic testing: what matters to key stakeholders?
Parents’ motivations, concerns and understanding of genome sequencing: a qualitative interview study
Identification of RELN variant p.(Ser2486Gly) in an Iranian family with ankylosing spondylitis; the first association of RELN and AS
De novo TBR1 variants cause a neurocognitive phenotype with ID and autistic traits: report of 25 new individuals and review of the literature
Genotype phasing in pedigrees using whole-genome sequence data
A newly recognized multiple malformation syndrome with caudal regression associated with a biallelic c.402G>A variant in TBX4
Evidence for penetrance in patients without a family history of disease: a systematic review
Transcript specific regulation of expression influences susceptibility to multiple sclerosis
High-resolution inference of genetic relationships among Jewish populations
Autosomal-dominant adult neuronal ceroid lipofuscinosis caused by duplication in DNAJC5 initially missed by Sanger and whole-exome sequencing
Exploring broad consent in the context of the 100,000 Genomes Project: a mixed methods study
CUGC for syndromic microphthalmia including next-generation sequencing-based approaches
Searching for parent-of-origin effects on cardiometabolic traits in imprinted genomic regions
Unraveling the genetic cause of hereditary ophthalmic disorders in Arab societies from Israel and the Palestinian Authority
TCTEX1D1 is a genetic modifier of disease progression in Duchenne muscular dystrophy CINRG cohort of European ancestry
Development and mixed-methods evaluation of an online animation for young people about genome sequencing