The American Journal of Human Genetics - 2021

211 articles | Last updated: 2025-12-03 14:12:57
Caucasian
0
White
0
European
1
Other
2
Article Title Cauc. White Euro. Other Phrases Used
T A T A T A T A
Distinguishing pedigree relationships via multi-way identity by descent sharing and sex-specific genetic maps
Massively parallel functional testing of MSH2 missense variants conferring Lynch syndrome risk
UBR7 functions with UBR5 in the Notch signaling pathway and is involved in a neurodevelopmental syndrome with epilepsy, ptosis, and hypothyroidism
PLEIO: a method to map and interpret pleiotropic loci with GWAS summary statistics
Rare and de novo coding variants in chromodomain genes in Chiari I malformation
Host genetic effects in pneumonia
Leveraging phenotypic variability to identify genetic interactions in human phenotypes
So many Nigerians: why is Nigeria overrepresented as the ancestral genetic homeland of Legacy African North Americans?
Response to Jackson
Multi-parametric analysis of 57 SYNGAP1 variants reveal impacts on GTPase signaling, localization, and protein stability
Identification of susceptibility loci for Takayasu arteritis through a large multi-ancestral genome-wide association study
Probabilistic colocalization of genetic variants from complex and molecular traits: promise and limitations
SCUBE3 loss-of-function causes a recognizable recessive developmental disorder due to defective bone morphogenetic protein signaling
Failure to recombine is a common feature of human oogenesis
Missense variants in the N-terminal domain of the A isoform of FHF2/FGF13 cause an X-linked developmental and epileptic encephalopathy
Evolving use of ancestry, ethnicity, and race in genetics research—A survey spanning seven decades
This month in The Journal
Welch-weighted Egger regression reduces false positives due to correlated pleiotropy in Mendelian randomization
Phenome risk classification enables phenotypic imputation and gene discovery in developmental stuttering
Genomic medicine year in review: 2021
Describing human populations: An evolving picture in human genetics research
Bi-allelic premature truncating variants in LTBP1 cause cutis laxa syndrome
Improved pathogenicity prediction for rare human missense variants
Biallelic Mutations in ADPRHL2, Encoding ADP-Ribosylhydrolase 3, Lead to a Degenerative Pediatric Stress-Induced Epileptic Ataxia Syndrome
H3K27ac HiChIP in prostate cell lines identifies risk genes for prostate cancer susceptibility
Bayesian model comparison for rare-variant association studies
Bi-allelic variants in OGDHL cause a neurodevelopmental spectrum disease featuring epilepsy, hearing loss, visual impairment, and ataxia
Mosaic human preimplantation embryos and their developmental potential in a prospective, non-selection clinical trial
Closing the gap: Systematic integration of multiplexed functional data resolves variants of uncertain significance in BRCA1, TP53, and PTEN
Genome-wide analysis of common and rare variants via multiple knockoffs at biobank scale, with an application to Alzheimer disease genetics
Identification of discriminative gene-level and protein-level features associated with pathogenic gain-of-function and loss-of-function variants
Returning actionable genomic results in a research biobank: Analytic validity, clinical implementation, and resource utilization
Integrative multiomics analysis highlights immune-cell regulatory mechanisms and shared genetic architecture for 14 immune-associated diseases and cancer outcomes
Bonsai: An efficient method for inferring large human pedigrees from genotype data
This month in The Journal
Interpretable prioritization of splice variants in diagnostic next-generation sequencing
Clustered mutations in the GRIK2 kainate receptor subunit gene underlie diverse neurodevelopmental disorders
Bi-allelic variants in the mitochondrial RNase P subunit PRORP cause mitochondrial tRNA processing defects and pleiotropic multisystem presentations
Leveraging health systems data to characterize a large effect variant conferring risk for liver disease in Puerto Ricans
Dystonia-specific mutations in THAP1 alter transcription of genes associated with neurodevelopment and myelin
Establishing risk of vision loss in Leber hereditary optic neuropathy
Human iPSC-derived neurons reveal early developmental alteration of neurite outgrowth in the late-occurring neurodegenerative Wolfram syndrome
Allele-specific epigenetic activity in prostate cancer and normal prostate tissue implicates prostate cancer risk mechanisms
Conceptualization of utility in translational clinical genomics research
Active site variants in STT3A cause a dominant type I congenital disorder of glycosylation with neuromusculoskeletal findings
Genetic overlap and causality between blood metabolites and migraine
The National Academies’ Roundtable on Genomics and Precision Health: Where we have been and where we are heading
Bi-allelic variants in SPATA5L1 lead to intellectual disability, spastic-dystonic cerebral palsy, epilepsy, and hearing loss
The risks of using unapproved gene symbols
Revisiting the out of Africa event with a deep-learning approach
Sub-genic intolerance, ClinVar, and the epilepsies: A whole-exome sequencing study of 29,165 individuals
Comparative single-cell analysis of biopsies clarifies pathogenic mechanisms in Klinefelter syndrome
SPRED2 loss-of-function causes a recessive Noonan syndrome-like phenotype
Detecting cryptic clinically relevant structural variation in exome-sequencing data increases diagnostic yield for developmental disorders
This month in The Journal
Classification of 101 BRCA1 and BRCA2 variants of uncertain significance by cosegregation study: A powerful approach
High prevalence of multilocus pathogenic variation in neurodevelopmental disorders in the Turkish population Turkish population
ABHD16A deficiency causes a complicated form of hereditary spastic paraplegia associated with intellectual disability and cerebral anomalies
Whole-genome sequencing in diverse subjects identifies genetic correlates of leukocyte traits: The NHLBI TOPMed program
An integrated approach to identify environmental modulators of genetic risk factors for complex traits
A 584 bp deletion in CTRB2 inhibits chymotrypsin B2 activity and secretion and confers risk of pancreatic cancer
Improved pathogenicity prediction for rare human missense variants
Rare and de novo variants in 827 congenital diaphragmatic hernia probands implicate LONP1 as candidate risk gene
Identifying digenic disease genes via machine learning in the Undiagnosed Diseases Network
This month in The Journal
Response to Hamosh et al.
Fast two-stage phasing of large-scale sequence data
Response to Biesecker et al.
Genetic determinants of blood-cell traits influence susceptibility to childhood acute lymphoblastic leukemia
COPB2 loss of function causes a coatopathy with osteoporosis and developmental delay
Stratification of risk of progression to colectomy in ulcerative colitis via measured and predicted gene expression
Position effects at the FGF8 locus are associated with femoral hypoplasia
Brain-trait-associated variants impact cell-type-specific gene regulation during neurogenesis
Robust genetic nurture effects on education: A systematic review and meta-analysis based on 38,654 families across 8 cohorts
Patterns of genetic connectedness between modern and medieval Estonian genomes reveal the origins of a major ancestry component of the Finnish population
Altered regulation of DPF3, a member of the SWI/SNF complexes, underlies the 14q24 renal cancer susceptibility locus
Clustered mutations in the GRIK2 kainate receptor subunit gene underlie diverse neurodevelopmental disorders
A polygenic-score-based approach for identification of gene-drug interactions stratifying breast cancer risk
This month in The Journal
A UVB-responsive common variant at chromosome band 7p21.1 confers tanning response and melanoma risk via regulation of the aryl hydrocarbon receptor, AHR
The omnigenic model and polygenic prediction of complex traits
Scaling national and international improvement in virtual gene panel curation via a collaborative approach to discordance resolution
Massively parallel characterization of CYP2C9 variant enzyme activity and abundance
TNPO2 variants associate with human developmental delays, neurologic deficits, and dysmorphic features and alter TNPO2 activity in Drosophila
Anatomy of DNA methylation signatures: Emerging insights and applications
Interpretable prioritization of splice variants in diagnostic next-generation sequencing
Cell-type-specific meQTLs extend melanoma GWAS annotation beyond eQTLs and inform melanocyte gene-regulatory mechanisms
High-throughput splicing assays identify missense and silent splice-disruptive POU1F1 variants underlying pituitary hormone deficiency
Regulatory variants in TCF7L2 are associated with thoracic aortic aneurysm
Beyond factor H: The impact of genetic-risk variants for age-related macular degeneration on circulating factor-H-like 1 and factor-H-related protein concentrations
Common haplotypes at the CFH locus and low-frequency variants in CFHR2 and CFHR5 associate with systemic FHR concentrations and age-related macular degeneration
Genome-wide association study reveals an association between the HLA-DPB1∗02:01:02 allele and wheat-dependent exercise-induced anaphylaxis
New approaches to predict the effect of co-occurring variants on protein characteristics
Dissection of pleiotropic effects of variants in and adjacent to F8 exon 19 and rescue of mRNA splicing and protein function
Optical genome mapping enables constitutional chromosomal aberration detection
Bi-allelic mutations of DNAH10 cause primary male infertility with asthenoteratozoospermia in humans and mice
Next-generation cytogenetics: Comprehensive assessment of 52 hematological malignancy genomes by optical genome mapping
Targeted long-read sequencing identifies missing disease-causing variation
Nonsense-mediated decay is highly stable across individuals and tissues
Genomic partitioning of inbreeding depression in humans
Shifting landscapes of human MTHFR missense-variant effects
Loss of C2orf69 defines a fatal autoinflammatory syndrome in humans and zebrafish that evokes a glycogen-storage-associated mitochondriopathy
This month in The Journal
Constrained maximum likelihood-based Mendelian randomization robust to both correlated and uncorrelated pleiotropic effects
Whole-exome sequencing reveals common and rare variants in immunologic and neurological genes implicated in achalasia
Unique variants in CLCN3, encoding an endosomal anion/proton exchanger, underlie a spectrum of neurodevelopmental disorders
Functional annotation of the 2q35 breast cancer risk locus implicates a structural variant in influencing activity of a long-range enhancer element
Bi-allelic variants in the ER quality-control mannosidase gene EDEM3 cause a congenital disorder of glycosylation
Summix: A method for detecting and adjusting for population structure in genetic summary data
Exome variant discrepancies due to reference-genome differences
De novo and bi-allelic variants in AP1G1 cause neurodevelopmental disorder with developmental delay, intellectual disability, and epilepsy
Evaluation of DNA Methylation Episignatures for Diagnosis and Phenotype Correlations in 42 Mendelian Neurodevelopmental Disorders
Pan-ancestry exome-wide association analyses of COVID-19 outcomes in 586,157 individuals
Whole-genome sequencing association analysis of quantitative red blood cell phenotypes: The NHLBI TOPMed program
A form of muscular dystrophy associated with pathogenic variants in JAG2
This month in The Journal
Project Baby Bear: Rapid precision care incorporating rWGS in 5 California children’s hospitals demonstrates improved clinical outcomes and reduced costs of care
Large-scale machine-learning-based phenotyping significantly improves genomic discovery for optic nerve head morphology
Automated AI labeling of optic nerve head enables insights into cross-ancestry glaucoma risk and genetic discovery in >280,000 images from UKB and CLSA
Mutations in TP73 cause impaired mucociliary clearance and lissencephaly
Genetic effects on liver chromatin accessibility identify disease regulatory variants
Loss of C2orf69 defines a fatal autoinflammatory syndrome in humans and zebrafish that evokes a glycogen-storage-associated mitochondriopathy
Non-coding region variants upstream of MEF2C cause severe developmental disorder through three distinct loss-of-function mechanisms
Bi-allelic loss-of-function variants in BCAS3 cause a syndromic neurodevelopmental disorder
A computational approach for detecting physiological homogeneity in the midst of genetic heterogeneity
A human importin-β-related disorder: Syndromic thoracic aortic aneurysm caused by bi-allelic loss-of-function variants in IPO8
Bi-allelic variants in IPO8 cause a connective tissue disorder associated with cardiovascular defects, skeletal abnormalities, and immune dysregulation
Altered replication stress response due to CARD14 mutations promotes recombination-induced revertant mosaicism
Bi-allelic premature truncating variants in LTBP1 cause cutis laxa syndrome
Impaired glucose-1,6-biphosphate production due to bi-allelic PGM2L1 mutations is associated with a neurodevelopmental disorder
Leveraging both individual-level genetic data and GWAS summary statistics increases polygenic prediction
A mutation in SLC37A4 causes a dominantly inherited congenital disorder of glycosylation characterized by liver dysfunction
2020 McKusick Award address
Estimation of non-additive genetic variance in human complex traits from a large sample of unrelated individuals
Variants in the degron of AFF3 are associated with intellectual disability, mesomelic dysplasia, horseshoe kidney, and epileptic encephalopathy
This month in The Journal
EPISPOT: An epigenome-driven approach for detecting and interpreting hotspots in molecular QTL studies
Sub-genic intolerance, ClinVar, and the epilepsies: A whole-exome sequencing study of 29,165 individuals
Heterozygous ANKRD17 loss-of-function variants cause a syndrome with intellectual disability, speech delay, and dysmorphism
Truncating SRCAP variants outside the Floating-Harbor syndrome locus cause a distinct neurodevelopmental disorder with a specific DNA methylation signature
Impaired cholesterol efflux in retinal pigment epithelium of individuals with juvenile macular degeneration
Missense variants in DPYSL5 cause a neurodevelopmental disorder with corpus callosum agenesis and cerebellar abnormalities
Somatic MAP3K3 mutation defines a subclass of cerebral cavernous malformation
Engagement and return of results preferences among a primarily African American genomic sequencing research cohort
Whole-genome sequencing association analysis of quantitative red blood cell phenotypes: The NHLBI TOPMed program
A form of muscular dystrophy associated with pathogenic variants in JAG2
Efficient mixed model approach for large-scale genome-wide association studies of ordinal categorical phenotypes
Estimation of non-additive genetic variance in human complex traits from a large sample of unrelated individuals
Haploinsufficiency of the Sin3/HDAC corepressor complex member SIN3B causes a syndromic intellectual disability/autism spectrum disorder
30 years of repeat expansion disorders: What have we learned and what are the remaining challenges?
Quantifying the contribution of dominance deviation effects to complex trait variation in biobank-scale data
Loss-of-function myeloperoxidase mutations are associated with increased neutrophil counts and pustular skin disease
Opportunities and challenges for the computational interpretation of rare variation in clinically important genes
Association of structural variation with cardiometabolic traits in Finns
This month in The Journal
A catalog of GWAS fine-mapping efforts in autoimmune disease
Progressive myoclonus epilepsies—Residual unsolved cases have marked genetic heterogeneity including dolichol-dependent protein glycosylation pathway genes
Expectations and blind spots for structural variation detection from long-read assemblies and short-read genome sequencing technologies
Pervasive cis effects of variation in copy number of large tandem repeats on local DNA methylation and gene expression
A unified framework for cross-population trait prediction by leveraging the genetic correlation of polygenic traits
Low-coverage sequencing cost-effectively detects known and novel variation in underrepresented populations
Machine learning-based reclassification of germline variants of unknown significance: The RENOVO algorithm
The landscape of autosomal-recessive pathogenic variants in European populations reveals phenotype-specific effects European populations
Spectrum of splicing variants in disease genes and the ability of RNA analysis to reduce uncertainty in clinical interpretation
A DNA repair disorder caused by de novo monoallelic DDB1 variants is associated with a neurodevelopmental syndrome
Epigenetic inactivation of ERF reactivates γ-globin expression in β-thalassemia
A powerful subset-based method identifies gene set associations and improves interpretation in UK Biobank
Discovery and fine-mapping of height loci via high-density imputation of GWASs in individuals of African ancestry African ancestry
Monoallelic and bi-allelic variants in NCDN cause neurodevelopmental delay, intellectual disability, and epilepsy
Negative selection on complex traits limits phenotype prediction accuracy between populations
De novo structural mutation rates and gamete-of-origin biases revealed through genome sequencing of 2,396 families
Rare and de novo coding variants in chromodomain genes in Chiari I malformation
Age dependency of the polygenic risk score for colorectal cancer
2020 William Allan Award address: genetics as a way of thinking—cultural inheritance from our teachers
2020 Curt Stern Award address: a more perfect clinical genome—how consanguineous populations contribute to the medical annotation of the human genome
2020 William Allan Award introduction: Mary-Claire King
How science will help us move forward in 2021
This month in The Journal
2020 ASHG awards and addresses
ASHG 2020 Curt Stern Award introduction: Fowzan Sami Alkuraya
Human ancient DNA analyses reveal the high burden of tuberculosis in Europeans over the last 2,000 years
Response to Li and Hopper
2020 ASHG presidential address: the ‘BIG TENT’ of genetics/genomics and our world
Mendelian pathway analysis of laboratory traits reveals distinct roles for ciliary subcompartments in common disease pathogenesis
Integrative analysis of liver-specific non-coding regulatory SNPs associated with the risk of coronary artery disease
Homozygous pathogenic variants in ACTL9 cause fertilization failure and male infertility in humans and mice
Strong functional data for pathogenicity or neutrality classify BRCA2 DNA-binding-domain variants of uncertain significance
SPEN haploinsufficiency causes a neurodevelopmental disorder overlapping proximal 1p36 deletion syndrome with an episignature of X chromosomes in females
Loss of α-actinin-3 during human evolution provides superior cold resilience and muscle heat generation
Whole-genome sequencing of African Americans implicates differential genetic architecture in inflammatory bowel disease
Genetic control of the human brain proteome
Topologically associating domain boundaries that are stable across diverse cell types are evolutionarily constrained and enriched for heritability
Albert de la Chapelle (1933–2020)
Rare and de novo coding variants in chromodomain genes in Chiari I malformation
The diagnostic odyssey: our family’s story
Recognizing those who deal with rare disease every day
HiC-ACT: improved detection of chromatin interactions from Hi-C data via aggregated Cauchy test
This month in The Journal
Mutation-specific pathophysiological mechanisms define different neurodevelopmental disorders associated with SATB1 dysfunction
Allele-specific editing ameliorates dominant retinitis pigmentosa in a transgenic mouse model
De novo TRIM8 variants impair its protein localization to nuclear bodies and cause developmental delay, epilepsy, and focal segmental glomerulosclerosis
Homozygous mutations in C14orf39/SIX6OS1 cause non-obstructive azoospermia and premature ovarian insufficiency in humans
Deleterious variants in X-linked CFAP47 induce asthenoteratozoospermia and primary male infertility
Perturbations of genes essential for Müllerian duct and Wölffian duct development in Mayer-Rokitansky-Küster-Hauser syndrome
Disentangling selection on genetically correlated polygenic traits via whole-genome genealogies
Multi-trait transcriptome-wide association studies with probabilistic Mendelian randomization
Genome-wide association study identifies novel susceptibility loci for KIT D816V positive mastocytosis
This month in The Journal
A dyadic approach to the delineation of diagnostic entities in clinical genomics
De novo variants in POLR3B cause ataxia, spasticity, and demyelinating neuropathy
The genomics workforce must become more diverse: a strategic imperative