Human Genetics - 2021

167 articles | Last updated: 2025-12-03 14:12:56
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T A T A T A T A
5q35 duplication presents with psychiatric and undergrowth phenotypes mediated by NSD1 overexpression and mTOR signaling downregulation
Bi-allelic BRWD1 variants cause male infertility with asthenoteratozoospermia and likely primary ciliary dyskinesia
Loss of PIGK function causes severe infantile encephalopathy and extensive neuronal apoptosis
A missense allele of PEX5 is responsible for the defective import of PTS2 cargo proteins into peroxisomes
PIGF deficiency causes a phenotype overlapping with DOORS syndrome
Embeddings from protein language models predict conservation and variant effects
Challenges in the diagnosis of neurofibromatosis type 1 (NF1) in young children facilitated by means of revised diagnostic criteria including genetic testing for pathogenic NF1 gene variants
Differential fates of introns in gene expression due to global alternative splicing
Variant-specific effects define the phenotypic spectrum of HNRNPH2-associated neurodevelopmental disorders in males
Retraction Note to: Revisiting the male genetic landscape of China: a multi-center study of almost 38,000 Y-STR haplotypes
Common, low-frequency, rare, and ultra-rare coding variants contribute to COVID-19 severity
In the family: access to, and communication of, familial information in clinical practice
What makes a good prediction? Feature importance and beginning to open the black box of machine learning in genetics
Genome-wide analysis of mitochondrial DNA copy number reveals loci implicated in nucleotide metabolism, platelet activation, and megakaryocyte proliferation
Prenatal exome sequencing and chromosomal microarray analysis in fetal structural anomalies in a highly consanguineous population reveals a propensity of ciliopathy genes causing multisystem phenotype
A common founder effect of the splice site variant c.-23 + 1G > A in GJB2 gene causing autosomal recessive deafness 1A (DFNB1A) in Eurasia
A novel complex genomic rearrangement affecting the KCNJ2 regulatory region causes a variant of Cooks syndrome
Evaluation of copy number variants for genetic hearing loss: a review of current approaches and recent findings
Genetic therapies for neurological disorders
SLC gene mutations and pediatric neurological disorders: diverse clinical phenotypes in a Saudi Arabian population
Spectrum and characterization of bi-allelic variants in MMAB causing cblB-type methylmalonic aciduria
Insight into ALKBH8-related intellectual developmental disability based on the first pathogenic missense variant
Disease-associated human genetic variation through the lens of precursor and mature RNA structure
MYT1L-associated neurodevelopmental disorder: description of 40 new cases and literature review of clinical and molecular aspects
Mitochondrial “dysmorphology” in variant classification
Correction to: Detailed clinical features and genotype–phenotype correlation in an OTOF-related hearing loss cohort in Japan
PRNCR1: a long non-coding RNA with a pivotal oncogenic role in cancer
The population genetics characteristics of a 90 locus panel of microhaplotypes
The potential application of organoids in breast cancer research and treatment
The promise of automated machine learning for the genetic analysis of complex traits
ASTL is mutated in female infertility
A review of migraine genetics: gathering genomic and transcriptomic factors
Interpretable machine learning for genomics
TUBB3 Arg262His causes a recognizable syndrome including CFEOM3, facial palsy, joint contractures, and early-onset peripheral neuropathy
Genetic etiology of non-syndromic hearing loss in Latin America
Population pharmacogenomics: an update on ethnogeographic differences and opportunities for precision public health individuals of European descent
Ocular manifestations in patients with inborn errors of intracellular cobalamin metabolism: a systematic review
How to fix a broken protein: restoring function to mutant human cystathionine β-synthase
WDR37 syndrome: identification of a distinct new cluster of disease-associated variants and functional analyses of mutant proteins
A recessive variant in TFAM causes mtDNA depletion associated with primary ovarian insufficiency, seizures, intellectual disability and hearing loss
A pathogenic deletion in Forkhead Box L1 (FOXL1) identifies the first otosclerosis (OTSC) gene
Correction to: Exploring the missing heritability in subjects with hearing loss, enlarged vestibular aqueducts, and a single or no pathogenic SLC26A4 variant
Hearing loss in Africa: current genetic profile Uses terms referring to populations: 'Europeans and Asians', 'African', 'North Africa', 'sub-Saharan
Molecular and genetic characterization of a large Brazilian cohort presenting hearing loss
Special issue on companion animal genetics: Novel variants discovered in wide variety of diseases in dogs, identification and further characterization of traits in dogs and cats, and the use of microa
Novel gene discovery for hearing loss and other routes to increased diagnostic rates
Clinical implementation of drug metabolizing gene-based therapeutic interventions worldwide
The genetic etiology of hearing loss in Japan revealed by the social health insurance-based genetic testing of 10K patients
Identification of a novel missense mutation in the fibroblast growth factor 5 gene associated with longhair in the Maine Coon Cat
Prioritizing de novo autism risk variants with calibrated gene- and variant-scoring models
Detailed clinical features and genotype–phenotype correlation in an OTOF-related hearing loss cohort in Japan
Classification of NF1 microdeletions and its importance for establishing genotype/phenotype correlations in patients with NF1 microdeletions
Patient and public preferences for being recontacted with updated genomic results: a mixed methods study
Clinical impact of rare variants associated with inherited channelopathies: a 5-year update
Biallelic variants in YRDC cause a developmental disorder with progeroid features
COCH-related autosomal dominant nonsyndromic hearing loss: a phenotype–genotype study
Prevalence and clinical features of autosomal dominant and recessive TMC1-associated hearing loss Japanese
Differential genetic diagnoses of adult post-lingual hearing loss according to the audiogram pattern and novel candidate gene evaluation
ATR-X syndrome: genetics, clinical spectrum, and management
Racial and ethnic disparities in diagnostic efficacy of comprehensive genetic testing for sensorineural hearing loss
Genetics of otosclerosis: finally catching up with other complex traits?
Diagnostic yield of clinical exome sequencing as a first-tier genetic test for the diagnosis of genetic disorders in pediatric patients: results from a referral center study
Revealing modifier variations characterizations for elucidating the genetic basis of human phenotypic variations
Gene expression levels modulate germline mutation rates through the compound effects of transcription-coupled repair and damage
The noncoding genome and hearing loss
Racial and ethnic disparities in genetic testing for hearing loss: a systematic review and synthesis subjects of European and Asian ancestry; European ancestry; Asian ancestry; Latino American, African, and indigenous North American ancestry
Genetic hearing loss: the audiologist’s perspective
Discovery of structural deletions in breast cancer predisposition genes using whole genome sequencing data from > 2000 women of African-ancestry African-ancestry
An ethical analysis of divergent clinical approaches to the application of genetic testing for autism and schizophrenia
Recontacting in medical genetics: the implications of a broadening knowledge base
Retraction Note: EDAR, LYPLAL1, PRDM16, PAX3, DKK1, TNFSF12, CACNA2D3, and SUPT3H gene variants influence facial morphology in a Eurasian population Eurasian population
Complete lung agenesis caused by complex genomic rearrangements with neo-TAD formation at the SHH locus
NGS and phenotypic ontology-based approaches increase the diagnostic yield in syndromic retinal diseases
Central auditory deficits associated with genetic forms of peripheral deafness
Predicting functional consequences of mutations using molecular interaction network features
ASL expression in ALDH1A1+ neurons in the substantia nigra metabolically contributes to neurodegenerative phenotype
Ethical considerations in gene selection for reproductive carrier screening
The natural history of OTOF-related auditory neuropathy spectrum disorders: a multicenter study
Dissecting the genetic history of the Roman Catholic populations of West Coast India
The fetus in the age of the genome
Through 40,000 years of human presence in Southern Europe: the Italian case study post-LGM re-peopling of Europe; pre-Villabruna Europeans; Southern European samples
A wide range of protective and predisposing variants in aggrecan influence the susceptibility for otosclerosis
Exploring the missing heritability in subjects with hearing loss, enlarged vestibular aqueducts, and a single or no pathogenic SLC26A4 variant
Restriction enzyme selection dictates detection range sensitivity in chromatin conformation capture-based variant-to-gene mapping approaches
Radiographical Survey of Osteochondrodysplasia in Scottish Fold Cats caused by the TRPV4 gene variant
First glance at the molecular etiology of hearing loss in French-Canadian families from Saguenay-Lac-Saint-Jean’s founder population French-Canadian families from Saguenay-Lac-Saint-Jean’s founder population
The clinical utility of exome and genome sequencing across clinical indications: a systematic review
The PMEL gene and merle (dapple) in the dachshund: cryptic, hidden, and mosaic variants demonstrate the need for genetic testing prior to breeding
Genetic etiology of hearing loss in Russia
Correction to: Novel loci and mapuche genetic ancestry are associated with pubertal growth traits in Chilean boys Mapuche genetic ancestry
Genetic architecture and phenotypic landscape of SLC26A4-related hearing loss
Deafness-in-a-dish: modeling hereditary deafness with inner ear organoids
New insights into Perrault syndrome, a clinically and genetically heterogeneous disorder
Genetics, pathogenesis and therapeutic developments for Usher syndrome type 2
Genetic etiology of hereditary hearing loss in the Gulf Cooperation Council countries
Molecular genetic landscape of hereditary hearing loss in Pakistan
ZNF668 deficiency causes a recognizable disorder of DNA damage repair
Hearing loss and tinnitus: association studies for complex-hearing disorders in mouse and man
Evidence that geographic variation in genetic ancestry associates with uterine fibroids
Identification of autosomal recessive nonsyndromic hearing impairment genes through the study of consanguineous and non-consanguineous families: past, present, and future
Identification of aneuploidy in dogs screened by a SNP microarray
An overview of germline variations in genes of primary immunodeficiences through integrative analysis of ClinVar, HGMD® and dbSNP databases
Genetic analyses of gynecological disease identify genetic relationships between uterine fibroids and endometrial cancer, and a novel endometrial cancer genetic risk region at the WNT4 1p36.12 locus
Genetics and meta-analysis of recessive non-syndromic hearing impairment and Usher syndrome in Maghreb population: lessons from the past, contemporary actualities and future challenges
Bi-allelic truncating variants in CFAP206 cause male infertility in human and mouse
Correction to: Causal influences of neuroticism on mental health and cardiovascular disease
Genetic architecture and phenotypic landscape of deafness and onychodystrophy syndromes
Human deafness-associated variants alter the dynamics of key molecules in hair cell stereocilia F-actin cores
Highly pleiotropic variants of human traits are enriched in genomic regions with strong background selection
Use of whole genome analysis to identify shared genomic variants across breeds in canine mitral valve disease
IFIH1 loss-of-function variants contribute to very early-onset inflammatory bowel disease
Compensatory epistasis explored by molecular dynamics simulations
Collagen transport and related pathways in Osteogenesis Imperfecta
Genome sequencing in families with congenital limb malformations
Unraveling the genetic complexities of combined retinal dystrophy and hearing impairment
Analysis of genotype–phenotype relationships in 90 Chinese probands with Waardenburg syndrome
Association of CXCR6 with COVID-19 severity: delineating the host genetic factors in transcriptomic regulation
Evaluating marginal genetic correlation of associated loci for complex diseases and traits between European and East Asian populations European and East Asian populations
Current challenges and opportunities for pharmacogenomics: perspective of the Industry Pharmacogenomics Working Group (I-PWG)
Whole-genome sequencing identifies functional noncoding variation in SEMA3C that cosegregates with dyslexia in a multigenerational family
Chromosomal translocations inactivating CDKN2A support a single path for malignant peripheral nerve sheath tumor initiation
Phenome-wide screening of GWAS data reveals the complex causal architecture of obesity
Novel loci and Mapuche genetic ancestry are associated with pubertal growth traits in Chilean boys European and Mapuche Native American ancestries; European adolescents; European compared with Mapuch; Mapuche Native American ancestries; Mapuche adolescents
Genotype imputation in case-only studies of gene-environment interaction: validity and power
What is so complicated about prenatal testing for Down syndrome? A personal view
Missense variant in LOXHD1 is associated with canine nonsyndromic hearing loss
Should we respect parents’ views about which results to return from genomic sequencing?
Pathogenic STX3 variants affecting the retinal and intestinal transcripts cause an early-onset severe retinal dystrophy in microvillus inclusion disease subjects
Causal influences of neuroticism on mental health and cardiovascular disease
Characterization of intermediate-sized insertions using whole-genome sequencing data and analysis of their functional impact on gene expression
Variants in GCNA, X-linked germ-cell genome integrity gene, identified in men with primary spermatogenic failure
Biallelic mutations in l-dopachrome tautomerase (DCT) cause infantile nystagmus and oculocutaneous albinism
Missense and truncating variants in CHD5 in a dominant neurodevelopmental disorder with intellectual disability, behavioral disturbances, and epilepsy
Okur-Chung neurodevelopmental syndrome-linked CK2α variants have reduced kinase activity
Geographic variation in the polygenic score of height in Japan
Addressing the dark matter of gene therapy: technical and ethical barriers to clinical application
In-frame deletion in canine PITRM1 is associated with a severe early-onset epilepsy, mitochondrial dysfunction and neurodegeneration
Pathogenic variants in CDH11 impair cell adhesion and cause Teebi hypertelorism syndrome
The human microbiome and genetic disease: towards the integration of metagenomic and multi-omics data
Correction to: A missense mutation in IFT74, encoding for an essential component for intraflagellar transport of Tubulin, causes asthenozoospermia and male infertility without clinical signs of Bardet
Genome-first approach to rare EYA4 variants and cardio-auditory phenotypes in adults
Peter S. Harper: obituary
The promise of public health ethics for precision medicine: the case of newborn preventive genomic sequencing
A missense variant in NUF2, a component of the kinetochore NDC80 complex, causes impaired chromosome segregation and aneuploidy associated with microcephaly and short stature
Exome sequencing reveals predominantly de novo variants in disorders with intellectual disability (ID) in the founder population of Finland European populations
A missense mutation in IFT74, encoding for an essential component for intraflagellar transport of Tubulin, causes asthenozoospermia and male infertility without clinical signs of Bardet–Biedl syndrome
High-throughput STELA provides a rapid test for the diagnosis of telomere biology disorders
A knockout mutation associated with juvenile paroxysmal dyskinesia in Markiesje dogs indicates SOD1 pleiotropy
Insights into the pathogenicity of missense variants in the forkhead domain of FOX proteins underlying Mendelian disorders
Multi-omics highlights ABO plasma protein as a causal risk factor for COVID-19
Canine DVL2 variant contributes to brachycephalic phenotype and caudal vertebral anomalies
A missense variant in IFT122 associated with a canine model of retinitis pigmentosa
Admixture mapping identifies African and Amerindigenous local ancestry loci associated with fetal growth
Do we measure or compute polygenic risk scores? Why language matters
Pharmacogenetics in developing countries and low resource environments
Intronic variant in POU1F1 associated with canine pituitary dwarfism
Web resource on available DNA variant tests for hereditary diseases and genetic predispositions in dogs and cats: An Update
Correction to: Deciphering the genetic and epidemiological landscape of mitochondrial DNA abundance
Missense3D-DB web catalogue: an atom-based analysis and repository of 4M human protein-coding genetic variants
A biallelic variant in CLRN2 causes non-syndromic hearing loss in humans
A recurrent missense variant in EYA3 gene is associated with oculo-auriculo-vertebral spectrum
Meta-analysis of sample-level dbGaP data reveals novel shared genetic link between body height and Crohn’s disease
Genetics of agenesis/hypoplasia of the uterus and vagina: narrowing down the number of candidate genes for Mayer–Rokitansky–Küster–Hauser Syndrome
Alternative splicing in normal and pathological human placentas is correlated to genetic variants
RET compound inheritance in Chinese patients with Hirschsprung disease: lack of penetrance from insufficient gene dysfunction Chinese
Haploinsufficiency of the HIRA gene located in the 22q11 deletion syndrome region is associated with abnormal neurodevelopment and impaired dendritic outgrowth
A network-based machine-learning framework to identify both functional modules and disease genes