The American Journal of Human Genetics - 2022

194 articles | Last updated: 2025-12-03 14:12:57
Caucasian
0
White
0
European
2
Other
1
Article Title Cauc. White Euro. Other Phrases Used
T A T A T A T A
A minimal role for synonymous variation in human disease
Genomic Medicine Year in Review: 2022
Consequences of chromosome gain: A new view on trisomy syndromes
This month in The Journal
Addressing the challenges of polygenic scores in human genetic research
Functional characterization of 5p15.33 risk locus in uveal melanoma reveals rs452384 as a functional variant and NKX2.4 as an allele-specific interactor
Massively parallel reporter assays and variant scoring identified functional variants and target genes for melanoma loci and highlighted cell-type specificity
Understanding changes in genetic literacy over time and in genetic research participants
Calibration of computational tools for missense variant pathogenicity classification and ClinGen recommendations for PP3/BP4 criteria
A recurrent de novo splice site variant involving DNM1 exon 10a causes developmental and epileptic encephalopathy through a dominant-negative mechanism
A scoping review of guidelines for the use of race, ethnicity, and ancestry reveals widespread consensus but also points of ongoing disagreement
TCEAL1 loss-of-function results in an X-linked dominant neurodevelopmental syndrome and drives the neurological disease trait in Xq22.2 deletions
Estimating the genome-wide mutation rate from thousands of unrelated individuals
Genome- and transcriptome-wide association studies of 386,000 Asian and European-ancestry women provide new insights into breast cancer genetics European-ancestry; Asian
EMILIN1 deficiency causes arterial tortuosity with osteopenia and connects impaired elastogenesis with defective collagen fibrillogenesis
Systematic comparison of family history and polygenic risk across 24 common diseases
Shariant platform: Enabling evidence sharing across Australian clinical genetic-testing laboratories to support variant interpretation
Response to Eura et al.
RILPL1-related OPDM is absent in a Japanese cohort
Care4Rare Canada: Outcomes from a decade of network science for rare disease gene discovery
This month in The Journal
The recurrent de novo c.2011C>T missense variant in MTSS2 causes syndromic intellectual disability
A founder event causing a dominant childhood epilepsy survives 800 years through weak selective pressure
Bi-allelic CAMSAP1 variants cause a clinically recognizable neuronal migration disorder
Transcriptional and functional consequences of alterations to MEF2C and its topological organization in neuronal models
Liability-scale heritability estimation for biobank studies of low-prevalence disease
Reduced penetrance of MODY-associated HNF1A/HNF4A variants but not GCK variants in clinically unselected cohorts
The construction of cross-population polygenic risk scores using transfer learning
Multi-omics approach dissects cis-regulatory mechanisms underlying North Carolina macular dystrophy, a retinal enhanceropathy
Pleiotropic modifiers of age-related diabetes and neonatal intestinal obstruction in cystic fibrosis
De novo variants in FRMD5 are associated with developmental delay, intellectual disability, ataxia, and abnormalities of eye movement
FastRNA: An efficient solution for PCA of single-cell RNA-sequencing data based on a batch-accounting count model
This month in The Journal
Segregation analysis of 17,425 population-based breast cancer families: Evidence for genetic susceptibility and risk prediction
MagicalRsq: Machine-learning-based genotype imputation quality calibration
A cross-species approach using an in vivo evaluation platform in mice demonstrates that sequence variation in human RABEP2 modulates ischemic stroke outcomes
Tissue- and cell-type-specific molecular and functional signatures of 16p11.2 reciprocal genomic disorder across mouse brain and human neuronal models
ExPRSweb: An online repository with polygenic risk scores for common health-related exposures
The piRNA-pathway factor FKBP6 is essential for spermatogenesis but dispensable for control of meiotic LINE-1 expression in humans
KnockoffTrio: A knockoff framework for the identification of putative causal variants in genome-wide association studies with trio design
An HNRNPK-specific DNA methylation signature makes sense of missense variants and expands the phenotypic spectrum of Au-Kline syndrome
GABBR1 monoallelic de novo variants linked to neurodevelopmental delay and epilepsy
Mutations in SCNM1 cause orofaciodigital syndrome due to minor intron splicing defects affecting primary cilia
The recurrent de novo c.2011C>T missense variant in MTSS2 causes syndromic intellectual disability
Social and scientific motivations to move beyond groups in allele frequencies: The TOPMed experience
Genetics-informed precision treatment formulation in schizophrenia and bipolar disorder
Identification and single-base gene-editing functional validation of a cis-EPO variant as a genetic predictor for EPO-increasing therapies
Integrating transcriptomics, metabolomics, and GWAS helps reveal molecular mechanisms for metabolite levels and disease risk
Statement on storage and use of genetic materials
This month in The Journal
Bi-allelic LETM1 variants perturb mitochondrial ion homeostasis leading to a clinical spectrum with predominant nervous system involvement
On the reproductive capabilities of aneuploid human preimplantation embryos
Genetic structure correlates with ethnolinguistic diversity in eastern and southern Africa
Addressing underrepresentation in genomics research through community engagement
Bi-allelic loss-of-function variants in TMEM147 cause moderate to profound intellectual disability with facial dysmorphism and pseudo-Pelger-Huët anomaly
A genome sequencing system for universal newborn screening, diagnosis, and precision medicine for severe genetic diseases
Variation in CFHR3 determines susceptibility to meningococcal disease by controlling factor H concentrations
OARD: Open annotations for rare diseases and their phenotypes based on real-world data
Extent to which array genotyping and imputation with large reference panels approximate deep whole-genome sequencing
A reverse genetics and genomics approach to gene paralog function and disease: Myokymia and the juxtaparanode
Exome sequencing reveals a distinct somatic genomic landscape in breast cancer from women with germline PTEN variants
A multi-layer functional genomic analysis to understand noncoding genetic variation in lipids
“Choice of law” in precision medicine research
Natural Selection Shapes Codon Usage in the Human Genome
Loss of function of OTUD7A in the schizophrenia- associated 15q13.3 deletion impairs synapse development and function in human neurons
Multi-ancestry fine-mapping improves precision to identify causal genes in transcriptome-wide association studies
Germline thymidylate synthase deficiency impacts nucleotide metabolism and causes dyskeratosis congenita
Neurodevelopmental copy-number variants: A roadmap to improving outcomes by uniting patient advocates, researchers, and clinicians for collective impact
This month in The Journal
ADGRL1 haploinsufficiency causes a variable spectrum of neurodevelopmental disorders in humans and alters synaptic activity and behavior in a mouse model
Simultaneous inference of parental admixture proportions and admixture times from unphased local ancestry calls
Rescue of a familial dysautonomia mouse model by AAV9-Exon-specific U1 snRNA
Monoallelic pathogenic ALG5 variants cause atypical polycystic kidney disease and interstitial fibrosis
Bi-allelic variants in DOHH, catalyzing the last step of hypusine biosynthesis, are associated with a neurodevelopmental disorder
Bi-allelic loss-of-function variants in PPFIBP1 cause a neurodevelopmental disorder with microcephaly, epilepsy, and periventricular calcifications
Large-scale analyses of the X chromosome in 2,354 infertile men discover recurrently affected genes associated with spermatogenic failure
Response to Lee et al.
Investigating the shared genetic architecture of uterine leiomyoma and breast cancer: A genome-wide cross-trait analysis
Inherited HTT CAG repeat length does not have a major impact on Huntington disease duration
Clinical impact of additional findings detected by genome-wide non-invasive prenatal testing: Follow-up results of the TRIDENT-2 study
This month in The Journal
C. Thomas Caskey, M.D. (1938–2022): A remembrance
Digital health-enabled genomics: Opportunities and challenges
Homozygous mutations in C14orf39/SIX6OS1 cause non-obstructive azoospermia and premature ovarian insufficiency in humans
Genetic interactions drive heterogeneity in causal variant effect sizes for gene expression and complex traits
Leveraging the local genetic structure for trans-ancestry association mapping
The impact of anorexia nervosa and BMI polygenic risk on childhood growth: A 20-year longitudinal population-based study
Rare genetic variants in genes and loci linked to dominant monogenic developmental disorders cause milder related phenotypes in the general population
A massively parallel assay accurately discriminates between functionally normal and abnormal variants in a hotspot domain of KCNH2
A calibrated functional patch-clamp assay to enhance clinical variant interpretation in KCNH2-related long QT syndrome
Identification and functional evaluation of GRIA1 missense and truncation variants in individuals with ID: An emerging neurodevelopmental syndrome
An epigenome-wide view of osteoarthritis in primary tissues
Genotype error biases trio-based estimates of haplotype phase accuracy
This month in The Journal
Is there a way to reduce the inequity in variant interpretation on the basis of ancestry?
The importance of universal ethical standards in science
Closing the loop: Editors' feedback on the ASHG readership survey
Comprehensive evaluation and efficient classification of BRCA1 RING domain missense substitutions
Clinical impact of additional findings detected by genome-wide non-invasive prenatal testing: Follow-up results of the TRIDENT-2 study
A spectrum of recessiveness among Mendelian disease variants in UK Biobank
A phenome-wide association study identifies effects of copy-number variation of VNTRs and multicopy genes on multiple human traits
Clotting factor genes are associated with preeclampsia in high-altitude pregnant women in the Peruvian Andes
Integration of rare expression outlier-associated variants improves polygenic risk prediction
Genome-wide association meta-analysis identifies 48 risk variants and highlights the role of the stria vascularis in hearing loss
Analysis of MRI-derived spleen iron in the UK Biobank identifies genetic variation linked to iron homeostasis and hemolysis
Whole-exome sequencing identifies rare genetic variants associated with human plasma metabolites
The immunogenetics of viral antigen response is associated with subtype-specific glioma risk and survival
Response to Mörseburg et al.
Analyzing and reconciling colocalization and transcriptome-wide association studies from the perspective of inferential reproducibility
This month in The Journal
Response to Wyckelsma et al.: Loss of α-actinin-3 during human evolution provides superior cold resilience and muscle heat generation
Germline predisposition to pediatric Ewing sarcoma is characterized by inherited pathogenic variants in DNA damage repair genes
Meta-imputation: An efficient method to combine genotype data after imputation with multiple reference panels
TOP-LD: A tool to explore linkage disequilibrium with TOPMed whole-genome sequence data
Shared components of heritability across genetically correlated traits
Combining evidence from Mendelian randomization and colocalization: Review and comparison of approaches
Germline MBD4 deficiency causes a multi-tumor predisposition syndrome
Powerful and robust inference of complex phenotypes' causal genes with dependent expression quantitative loci by a median-based Mendelian randomization
Leveraging LD eigenvalue regression to improve the estimation of SNP heritability and confounding inflation
A genealogical estimate of genetic relationships
Neither cardiac mitochondrial DNA variation nor copy number contribute to congenital heart disease risk
Progressive liver, kidney, and heart degeneration in children and adults affected by TULP3 mutations
Polygenic transcriptome risk scores for COPD and lung function improve cross-ethnic portability of prediction in the NHLBI TOPMed program
Walter Elmore Nance (1933–2021)
Germline variants in tumor suppressor FBXW7 lead to impaired ubiquitination and a neurodevelopmental syndrome
De novo variants in POLR3B cause ataxia, spasticity, and demyelinating neuropathy
This month in The Journal
Recessive PRDM13 mutations cause fatal perinatal brainstem dysfunction with cerebellar hypoplasia and disrupt Purkinje cell differentiation
Polygenic risk for prostate cancer: Decreasing relative risk with age but little impact on absolute risk
De novo variants in ATP2B1 lead to neurodevelopmental delay
METRO: Multi-ancestry transcriptome-wide association studies for powerful gene-trait association detection
Enrichment analyses identify shared associations for 25 quantitative traits in over 600,000 individuals from seven diverse ancestries
Genetic modifiers of Huntington disease differentially influence motor and cognitive domains
Family history aggregation unit-based tests to detect rare genetic variant associations with application to the Framingham Heart Study
Inferring population structure in biobank-scale genomic data
RAREsim: A simulation method for very rare genetic variants
Familial long-read sequencing increases yield of de novo mutations
Partitioning gene-level contributions to complex-trait heritability by allele frequency identifies disease-relevant genes
Predicted gene expression in ancestrally diverse populations leads to discovery of susceptibility loci for lifestyle and cardiometabolic traits
The genomic signatures of natural selection in admixed human populations
2021 Allan Award
This month in The Journal
Monoallelic and bi-allelic variants in NCDN cause neurodevelopmental delay, intellectual disability, and epilepsy
2021 McKusick Leadership Award: Learning from communities
2021 ASHG awards and addresses
2021 Curt Stern Award: Studying the biology of “junk”
2021 ASHG presidential address—Imagination and daring: Past, present, and future
The annual ASHG dinner
Loss-of-function variants in TIAM1 are associated with developmental delay, intellectual disability, and seizures
The individual and global impact of copy-number variants on complex human traits
A multi-dimensional integrative scoring framework for predicting functional variants in the human genome
Family secrets: Experiences and outcomes of participating in direct-to-consumer genetic relative-finder services
Recurrent de novo missense variants across multiple histone H4 genes underlie a neurodevelopmental syndrome
Duplications disrupt chromatin architecture and rewire GPR101-enhancer communication in X-linked acrogigantism
THUMPD1 bi-allelic variants cause loss of tRNA acetylation and a syndromic neurodevelopmental disorder
The functional impact of BRCA1 BRCT domain variants using multiplexed DNA double-strand break repair assays
GWAS of longitudinal trajectories at biobank scale
Whole-exome sequencing improves the diagnosis and care of men with non-obstructive azoospermia
A functional genomics approach in Tanzanian population identifies distinct genetic regulators of cytokine production compared to European population European population
The CGG repeat expansion in RILPL1 is associated with oculopharyngodistal myopathy type 4
Partitioning gene-mediated disease heritability without eQTLs
Accounting for age of onset and family history improves power in genome-wide association studies
Analysis of missense variants in the human genome reveals widespread gene-specific clustering and improves prediction of pathogenicity
Personalized genetic counseling for Stargardt disease: Offspring risk estimates based on variant severity
This month in The Journal
Portability of 245 polygenic scores when derived from the UK Biobank and applied to 9 ancestry groups from the same cohort
Leveraging gene co-regulation to identify gene sets enriched for disease heritability
Bi-allelic variants in neuronal cell adhesion molecule cause a neurodevelopmental disorder characterized by developmental delay, hypotonia, neuropathy/spasticity
Redefining tissue specificity of genetic regulation of gene expression in the presence of allelic heterogeneity
Harnessing tissue-specific genetic variation to dissect putative causal pathways between body mass index and cardiometabolic phenotypes
Trans-ancestral fine-mapping of MHC reveals key amino acids associated with spontaneous clearance of hepatitis C in HLA-DQβ1
Midbrain organoids mimic early embryonic neurodevelopment and recapitulate LRRK2-p.Gly2019Ser-associated gene expression
A transchromosomic rat model with human chromosome 21 shows robust Down syndrome features
Impaired catabolism of free oligosaccharides due to MAN2C1 variants causes a neurodevelopmental disorder
A flexible summary statistics-based colocalization method with application to the mucin cystic fibrosis lung disease modifier locus
MRSD: A quantitative approach for assessing suitability of RNA-seq in the investigation of mis-splicing in Mendelian disease
Rare germline heterozygous missense variants in BRCA1-associated protein 1, BAP1, cause a syndromic neurodevelopmental disorder
PhenoApt leverages clinical expertise to prioritize candidate genes via machine learning
StrVCTVRE: A supervised learning method to predict the pathogenicity of human genome structural variants
The genetic architecture of pediatric cardiomyopathy
This month in The Journal
Stephen T. Warren, Ph.D. (1953–2021): A remembrance
Subcutaneous adipose tissue splice quantitative trait loci reveal differences in isoform usage associated with cardiometabolic traits
Portability of 245 polygenic scores when derived from the UK Biobank and applied to 9 ancestry groups from the same cohort
Accurate long-read sequencing allows assembly of the duplicated RHD and RHCE genes harboring variants relevant to blood transfusion
Functional analysis of the 1p34.3 risk locus implicates GNL2 in high-grade serous ovarian cancer
Longer CAG repeat length is associated with shorter survival after disease onset in Huntington disease
Overcoming constraints on the detection of recessive selection in human genes from population frequency data
Rare coding variants in 35 genes associate with circulating lipid levels—A multi-ancestry analysis of 170,000 exomes
Bi-allelic variants in DNHD1 cause flagellar axoneme defects and asthenoteratozoospermia in humans and mice
Genetic architecture of gene regulation in Indonesian populations identifies QTLs associated with global and local ancestries
Estrogen receptor alpha and NFATc1 bind to a bone mineral density-associated SNP to repress WNT5B in osteoblasts
Monoallelic IFT140 pathogenic variants are an important cause of the autosomal dominant polycystic kidney-spectrum phenotype
Fast estimation of genetic correlation for biobank-scale data