Journal of Medical Genetics - 2020

178 articles | Last updated: 2025-12-03 14:12:57
Caucasian
1
White
0
European
3
Other
14
Article Title Cauc. White Euro. Other Phrases Used
T A T A T A T A
Adult phenotype in Koolen-de Vries/<i>KANSL1</i> haploinsufficiency syndrome
Calibration of polygenic risk scores is required prior to clinical implementation: results of three common cancers in UKB
Deep exploration of a<i>CDKN1C</i>mutation causing a mixture of Beckwith-Wiedemann and IMAGe syndromes revealed a novel transcript associated with developmental delay
Adopted individuals’ interest in elective genomic testing
Phenotype and genotype spectra of a Chinese cohort with nephronophthisis-related ciliopathy Chinese
Functional analysis of <i>TLK2</i> variants and their proximal interactomes implicates impaired kinase activity and chromatin maintenance defects in their pathogenesis
Dysfunction of VIPR2 leads to myopia in humans and mice Chinese Han
Sensitivity and specificity of loss of heterozygosity analysis for the classification of rare germline variants in<i>BRCA1/2</i>: results of the observational AGO-TR1 study (NCT02222883)
Linkage analysis identifies an isolated strabismus locus at 14q12 overlapping with FOXG1 syndrome region
Characteristics of germline mutations in Korean patients with pheochromocytoma/paraganglioma Korean; Asian
Screening of CNVs using NGS data improves mutation detection yield and decreases costs in genetic testing for hereditary cancer
A complex DICER1 syndrome phenotype associated with a germline pathogenic variant affecting the RNase IIIa domain of DICER1
Combining evidence for and against pathogenicity for variants in cancer susceptibility genes: CanVIG-UK consensus recommendations
MELAS-associated m.5541C&gt;T mutation caused instability of mitochondrial tRNA<sup>Trp</sup> and remarkable mitochondrial dysfunction
Heterogeneity of PNPT1 neuroimaging: mitochondriopathy, interferonopathy or both?
Canadian College of Medical Geneticists (CCMG) points to consider: resuming genetic services in a pandemic—a summary
Inhibition of G-protein signalling in cardiac dysfunction of intellectual developmental disorder with cardiac arrhythmia (IDDCA) syndrome
Implementing gene curation for hereditary cancer susceptibility in Australia: achieving consensus on genes with clinical utility
Using chatbots to screen for heritable cancer syndromes in patients undergoing routine colonoscopy
Genetic characterisation of sarcomatoid carcinomas reveals multiple novel actionable mutations and identifies <i>KRAS</i> mutation as a biomarker of poor prognosis
Prenatal features in Beckwith-Wiedemann syndrome and indications for prenatal testing
Phenotypes in adult patients with Rett syndrome: results of a 13-year experience and insights into healthcare transition
Dilated cardiomyopathy caused by truncating titin variants: long-term outcomes, arrhythmias, response to treatment and sex differences
Biallelic cGMP-dependent type II protein kinase gene (<i>PRKG2</i>) variants cause a novel acromesomelic dysplasia
CRISPR–Cas9/long-read sequencing approach to identify cryptic mutations in <i>BRCA1</i> and other tumour suppressor genes
Neurogenetic fetal akinesia and arthrogryposis: genetics, expanding genotype-phenotypes and functional genomics
Targeted deep sequencing of DNA from multiple tissue types improves the diagnostic rate and reveals a highly diverse phenotype of mosaic neurofibromatosis type 2
A germline 1;3 translocation disrupting the <i>VHL</i> gene: a novel genetic cause for von Hippel-Lindau
Stay at home: implementation and impact of virtualising cancer genetic services during COVID-19
Blood functional assay for rapid clinical interpretation of germline <i>TP53</i> variants
Association between ARID2 and RAS-MAPK pathway in intellectual disability and short stature
Association between a 46-SNP Polygenic Risk Score and melanoma risk in Dutch patients with familial melanoma
Molecular landscape of CAPN3 mutations in limb-girdle muscular dystrophy type R1: from a Chinese multicentre analysis to a worldwide perspective Chinese population; Europe; Finns; South Asians; 'ethnicity'; 'population'
Clinical phenotype and loss of the slow skeletal muscle troponin T in three new patients with recessive TNNT1 nemaline myopathy
Practical approach to the genetic diagnosis of unsolved dystrophinopathies: a stepwise strategy in the genomic era
Ethnic-specific <i>BRCA1/2</i> variation within Asia population: evidence from over 78 000 cancer and 40 000 non-cancer cases of Indian, Chinese, Korean and Japanese populations
Globotriaosylsphingosine (lyso-Gb<sub>3</sub>) and analogues in plasma and urine of patients with Fabry disease and correlations with long-term treatment and genotypes in a nationwide female Danish co
Biallelic variant in cyclin B3 is associated with failure of maternal meiosis II and recurrent digynic triploidy
De novo mutations of <i>SCN1A</i> are responsible for arthrogryposis broadening the <i>SCN1A</i>-related phenotypes
Systematic evaluation of olfaction in patients with hereditary cystic kidney diseases/renal ciliopathies
Assessment of mismatch repair deficiency in ovarian cancer
Constitutional de novo deletion CNV encompassing <i>REST</i> predisposes to diffuse hyperplastic perilobar nephroblastomatosis (HPLN)
Low tolerance for transcriptional variation at cohesin genes is accompanied by functional links to disease-relevant pathways
Loss-of-function variants in<i>POT1</i>predispose to uveal melanoma
Convergent lines of evidence support <i>NOTCH4</i> as a schizophrenia risk gene
Bi-allelic <i>SHOC1</i> loss-of-function mutations cause meiotic arrest and non-obstructive azoospermia Two Chinese families
Delineation of a new fibrillin-2-opathy with evidence for a role of <i>FBN2</i> in the pathogenesis of carpal tunnel syndrome
<i>RASA1</i> phenotype overlaps with hereditary haemorrhagic telangiectasia: two case reports
Assessing performance of pathogenicity predictors using clinically relevant variant datasets
A novel homozygous variant in <i>TRAPPC2L</i> results in a neurodevelopmental disorder and disrupts TRAPP complex function Ashkenazi Jewish
Biallelic variants in <i>BRCA1</i> gene cause a recognisable phenotype within chromosomal instability syndromes reframed as BRCA1 deficiency
Determinants of quality of life in Rett syndrome: new findings on associations with genotype
Association between genetic polymorphisms and endometrial cancer risk: a systematic review
De novo missense variants in <i>LMBRD2</i> are associated with developmental and motor delays, brain structure abnormalities and dysmorphic features
<i>CIC de novo</i> loss of function variants contribute to cerebral folate deficiency by downregulating <i>FOLR1</i> expression
Frameshift mutation of <i>Timm8a1</i> gene in mouse leads to an abnormal mitochondrial structure in the brain, correlating with hearing and memory impairment
Telangiectasia-ectodermal dysplasia-brachydactyly-cardiac anomaly syndrome is caused by de novo mutations in <i>protein kinase D1</i>
Pathogenic variants in<i>IMPG1</i>cause autosomal dominant and autosomal recessive retinitis pigmentosa
Can population <i>BRCA</i> screening be applied in non-Ashkenazi Jewish populations? Experience in Macau population
Biallelic mutations in the<i>TOGARAM1</i>gene cause a novel primary ciliopathy
Genotype-first in a cohort of 95 fetuses with multiple congenital abnormalities: when exome sequencing reveals unexpected fetal phenotype-genotype correlations
Data-driven modelling of mutational hotspots and in silico predictors in hypertrophic cardiomyopathy
Prenatal clinical manifestations in individuals with<i>COL4A1/2</i>variants
Heterozygous <i>KIF1A</i> variants underlie a wide spectrum of neurodevelopmental and neurodegenerative disorders
Biallelic loss-of-function<i>ZFYVE19</i>mutations are associated with congenital hepatic fibrosis, sclerosing cholangiopathy and high-GGT cholestasis Han Chinese
Congenital sensorineural hearing loss as the initial presentation of<i>PTPN11</i>-associated Noonan syndrome with multiple lentigines or Noonan syndrome: clinical features and underlying mechanisms
Biallelic variants in <i>ADARB1</i>, encoding a dsRNA-specific adenosine deaminase, cause a severe developmental and epileptic encephalopathy
Comprehensive characteristics of somatic mutations in the normal tissues of patients with cancer and existence of somatic mutant clones linked to cancer development
Otitis media susceptibility and shifts in the head and neck microbiome due to <i>SPINK5</i> variants
A<i>RIPOR2</i>in-frame deletion is a frequent and highly penetrant cause of adult-onset hearing loss
<i>DLG5</i> variants are associated with multiple congenital anomalies including ciliopathy phenotypes
Novel form of rhizomelic skeletal dysplasia associated with a homozygous variant in<i>GNPNAT1</i> Pakistani family
Variant type and position predict two distinct limb phenotypes in patients with GLI3-mediated polydactyly syndromes
Retinal nerve fibre layer thickness associates with phenylketonuria control and cognition
Heritable genetic variants in key cancer genes link cancer risk with anthropometric traits
Polygenic and multifactorial scores for pancreatic ductal adenocarcinoma risk prediction European origin
Exome and genome sequencing in adults with undiagnosed disease: a prospective cohort study
Loss of imprinting of the human-specific imprinted gene <i>ZNF597</i> causes prenatal growth retardation and dysmorphic features: implications for phenotypic overlap with Silver-Russell syndrome
Sporadic vestibular schwannoma: a molecular testing summary
Co-occurrence of germline <i>BRCA1</i> and <i>CDH1</i> pathogenic variants
Wide range of reduced penetrance alleles in spinal and bulbar muscular atrophy: a model-based approach
Clinical spectrum and genetic variations of <i>LMNA</i>-related muscular dystrophies in a large cohort of Chinese patients
Defining the phenotypical spectrum associated with variants in <i>TUBB2A</i>
Novel loss-of-function mutation in <i>HERC2</i> is associated with severe developmental delay and paediatric lethality
UKCGG Consensus Group guidelines for the management of patients with constitutional<i>TP53</i>pathogenic variants
Haploinsufficiency of the<i>NF1</i>gene is associated with protection against diabetes
Genetic screening for monogenic hypertension in hypertensive individuals in a clinical setting Chinese Han population
<i>SETD1B</i>-associated neurodevelopmental disorder
Population-based targeted sequencing of 54 candidate genes identifies<i>PALB2</i>as a susceptibility gene for high-grade serous ovarian cancer controls of broad European ancestry
Absence of SCAPER causes male infertility in humans and<i>Drosophila</i>by modulating microtubule dynamics during meiosis across ethnicities
Treatment switch in Fabry disease- a matter of dose?
Rare and<i>de novo</i>duplications containing<i>SHOX</i>in clubfoot
<i>NUBPL</i> mitochondrial disease: new patients and review of the genetic and clinical spectrum
Genetic and functional insights into CDA-I prevalence and pathogenesis
Mutation in<i>CATIP</i>(C2orf62) causes oligoteratoasthenozoospermia by affecting actin dynamics Bedouin
TMEM16A deficiency: a potentially fatal neonatal disease resulting from impaired chloride currents
Genotype-phenotype correlations for pancreatic cancer risk in Dutch melanoma families with pathogenic <i>CDKN2A</i> variants
Homozygous hypomorphic<i>BRCA2</i>variant in primary ovarian insufficiency without cancer or Fanconi anaemia trait Turkish
Analysis of genotype–phenotype correlations in <i>PAX6</i>-associated aniridia
Arterial complications in classical Ehlers-Danlos syndrome: a case series
Prevalence of<i>BRCA1</i>/<i>BRCA2</i>pathogenic variation in Chinese Han population
Integrated case-control and somatic-germline interaction analyses of soft-tissue sarcoma European ancestry
Paternal 132 bp deletion affecting <i>KCNQ1OT1</i> in 11p15.5 is associated with growth retardation but does not affect imprinting
Hypomethylation of a centromeric block of ICR1 is sufficient to cause Silver-Russell syndrome
Six-year prospective follow-up study in 151 carriers of the mitochondrial DNA 3243 A&gt;G variant
Bi-allelic<i>TTC5</i>variants cause delayed developmental milestones and intellectual disability
Mutation in the MICOS subunit gene <i>APOO</i> (MIC26) associated with an X-linked recessive mitochondrial myopathy, lactic acidosis, cognitive impairment and autistic features
‘Kinesinopathies’: emerging role of the kinesin family member genes in birth defects
Improved molecular detection of mosaicism in Beckwith-Wiedemann Syndrome
De novo variants in <i>SIAH1,</i> encoding an E3 ubiquitin ligase, are associated with developmental delay, hypotonia and dysmorphic features
Description of a multidisciplinary model of care in a French cohort of adult patients with tuberous sclerosis complex
Update and audit of the St George’s classification algorithm of primary lymphatic anomalies: a clinical and molecular approach to diagnosis
<i>De novo</i>mutations in the X-linked<i>TFE3</i>gene cause intellectual disability with pigmentary mosaicism and storage disorder-like features
Evidence for polygenic and oligogenic basis of Australian sporadic amyotrophic lateral sclerosis
Prevalence and associated phenotypes of DUSP6, IL17RD and SPRY4 variants in a large Chinese cohort with isolated hypogonadotropic hypogonadism Chinese
Autosomal dominant polycystic kidney disease in absence of renal cyst formation illustrates genetic interaction between<i>WT1</i>and<i>PKD1</i>
Diagnostic yield and clinical impact of exome sequencing in early-onset scoliosis (EOS)
Exome sequencing analysis identifies frequent oligogenic involvement and<i>FLNB</i>variants in adolescent idiopathic scoliosis
Human X chromosome exome sequencing identifies<i>BCORL1</i>as contributor to spermatogenesis
Comprehensive genetic sequence and copy number analysis for Charcot-Marie-Tooth disease in a Canadian cohort of 2517 patients
Understanding polygenic models, their development and the potential application of polygenic scores in healthcare
15q11.2 deletion is enriched in patients with total anomalous pulmonary venous connection
Uptake of pre-symptomatic testing for<i>BRCA1</i>and<i>BRCA2</i>is age, gender, offspring and time-dependent
TRAPγ-CDG shows asymmetric glycosylation and an effect on processing of proteins required in higher organisms
Novel genetic characterisation and phenotype correlation in von Hippel-Lindau (VHL) disease based on the Elongin C binding site: a large retrospective study
Under-reported aspects of diagnosis and treatment addressed in the Dutch-Flemish guideline for comprehensive diagnostics in disorders/differences of sex development
Homozygous variants in <i>SYCP2L</i> cause premature ovarian insufficiency
Low grade mosaicism in hereditary haemorrhagic telangiectasia identified by bidirectional whole genome sequencing reads through the 100,000 Genomes Project clinical diagnostic pipeline
Identifying novel oncogenic<i>RET</i>mutations and characterising their sensitivity to RET-specific inhibitors
Identification of known and unknown genes associated with mitral valve prolapse using an exome slice methodology
Excess of de novo variants in genes involved in chromatin remodelling in patients with marfanoid habitus and intellectual disability
Correction:<i>Reasons for and time to retraction of genetics articles published between 1970 and 2018</i>
Variable expression of subclinical phenotypes instead of reduced penetrance in families with mild triphalangeal thumb phenotypes
Germline <i>RET</i> variants underlie a subset of paediatric osteosarcoma
A homozygous<i>UBA5</i>pathogenic variant causes a fatal congenital neuropathy
Colorectal cancer genetic variants are also associated with serrated polyposis syndrome susceptibility
Diverse types of genomic evidence converge on alcohol use disorder risk genes
Heterogeneity of MSI-H gastric cancer identifies a subtype with worse survival Asian
Clinical and genetic features of somatic mosaicism in facioscapulohumeral dystrophy
Customised next-generation sequencing multigene panel to screen a large cohort of individuals with chromatin-related disorder
Cancer Variant Interpretation Group UK (CanVIG-UK): an exemplar national subspecialty multidisciplinary network
Clinical, pathological and genetic spectrum in 89 cases of mitochondrial progressive external ophthalmoplegia
Biallelic variants in <i>MAATS1</i> encoding CFAP91, a calmodulin-associated and spoke-associated complex protein, cause severe astheno-teratozoospermia and male infertility
Use of a rare disease registry for establishing phenotypic classification of previously unassigned <i>GLA</i> variants: a consensus classification system by a multispecialty Fabry disease genotype–phe
Diurnal variation in autonomic regulation among patients with genotyped Rett syndrome
Medical and neurobehavioural phenotypes in carriers of X-linked ichthyosis-associated genetic deletions in the UK Biobank
Severity of oro-dental anomalies in Loeys-Dietz syndrome segregates by gene mutation
Pathogenic variants in <i>TNRC6B</i> cause a genetic disorder characterised by developmental delay/intellectual disability and a spectrum of neurobehavioural phenotypes including autism and ADHD
Gene discoveries in autism are biased towards comorbidity with intellectual disability
Variants of uncertain clinical significance in hereditary breast and ovarian cancer genes: best practices in functional analysis for clinical annotation
Diagnostic exome sequencing in non-acquired focal epilepsies highlights a major role of GATOR1 complex genes
MicroRNA-4516-mediated regulation of <i>MAPK10</i> relies on 3′ UTR <i>cis</i>-acting variants and contributes to the altered risk of Hirschsprung disease
Mendelian randomisation study of the effects of known and putative risk factors on pancreatic cancer
Redefinition of familial intestinal gastric cancer: clinical and genetic perspectives
Investigating the genetic susceptibility to exertional heat illness
Excess of singleton loss-of-function variants in Parkinson’s disease contributes to genetic risk
Phenotype of genetically confirmed Silver-Russell syndrome beyond childhood
Homozygous mutations in <i>DZIP1</i> can induce asthenoteratospermia with severe MMAF
Variants in <i>LRRC34</i> reveal distinct mechanisms for predisposition to papillary thyroid carcinoma
Contribution of de novo and inherited rare CNVs to very preterm birth
<i>CDK5RAP2</i>primary microcephaly is associated with hypothalamic, retinal and cochlear developmental defects
Whole genome sequence-based haplotypes reveal a single origin of the 1393 bp <i>HBB</i> deletion
Clinical exome sequencing as the first-tier test for diagnosing developmental disorders covering both CNV and SNV: a Chinese cohort
ATR-16 syndrome: mechanisms linking monosomy to phenotype
Germline mutations in the new E1’ cryptic exon of the <i>VHL</i> gene in patients with tumours of von Hippel-Lindau disease spectrum or with paraganglioma
Characterisation of heterozygous <i>PMS2</i> variants in French patients with Lynch syndrome
Detecting mosaic variants in patients with somatic overgrowth syndromes using cell-free circulating DNA and deep sequencing
Pseudodiastrophic dysplasia expands the known phenotypic spectrum of defects in proteoglycan biosynthesis
Impacts of genomics on the health and social costs of intellectual disability
Higher burden of rare frameshift indels in genes related to synaptic transmission separate familial hemiplegic migraine from common types of migraine
Cancer patients’ views and understanding of genome sequencing: a qualitative study
Molecular characterisation of oncogenic urothelial mosaic mutations in patients with extensive keratinocytic epidermal naevi
Novel CCM2 missense variants abrogating the CCM1–CCM2 interaction cause cerebral cavernous malformations
<i>POT1</i> mutation spectrum in tumour types commonly diagnosed among <i>POT1</i>-associated hereditary cancer syndrome families
The clinical relevance of intragenic <i>NRXN1</i> deletions
<i>FLNC</i> truncations cause arrhythmogenic right ventricular cardiomyopathy
De novo variants in <i>SUPT16H</i> cause neurodevelopmental disorders associated with corpus callosum abnormalities
Xq28 copy number gain causing moyamoya disease and a novel moyamoya syndrome