Human Mutation - 2022

176 articles | Last updated: 2025-12-03 14:12:57
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Issue Information
Variant effect on splicing regulatory elements, branchpoint usage, and pseudoexonization: Strategies to enhance bioinformatic prediction using hereditary cancer genes as exemplars
<i>KBTBD13</i> is a novel cardiomyopathy gene
Germline selection of <i>PTPN11</i> (HGNC:9644) variants make a major contribution to both Noonan syndrome's high birth rate and the transmission of sporadic cancer variants re
<i>HOGA1</i> gene pathogenic variants in primary hyperoxaluria type III: Spectrum of pathogenic sequence variants, and phenotypic association
Comprehensive analysis of the <i>PRPF31</i> gene in retinitis pigmentosa patients: Four novel <i>Alu</i> ‐mediated copy number variations at the
The pathogenic c.1171A&gt;G (p.Arg391Gly) and c.2359G&gt;A (p.Val787Ile) ABCC6 variants display incomplete penetrance causing pseudoxanthoma elasticum in a subset of individuals
New clinical and molecular evidence linking mutations in <i>ARSG</i> to Usher syndrome type IV
A recurrent single‐exon deletion in <i>TBCK</i> might be under‐recognized in patients with infantile hypotonia and psychomotor delay
VariantAlert: A web‐based tool to notify updates in genetic variant annotations
Genetic characterization of 1210 Japanese pedigrees with inherited retinal diseases by whole‐exome sequencing
SPiP: Splicing Prediction Pipeline, a machine learning tool for massive detection of exonic and intronic variant effects on mRNA splicing
TFIIH stabilization recovers the DNA repair and transcription dysfunctions in thermo‐sensitive trichothiodystrophy
Using single molecule Molecular Inversion Probes as a cost‐effective, high‐throughput sequencing approach to target all genes and loci associated with macular diseases
Multiple endocrine neoplasia type 2 (MEN2) and <i>RET</i> specific modifications of the ACMG/AMP variant classification guidelines and impact on the MEN2 <i>RET</i>
High‐yield identification of pathogenic <i>NF1</i> variants by skin fibroblast transcriptome screening after apparently normal diagnostic DNA testing
Biallelic <i>ADAMTSL4</i> variants in a Chinese cohort of congenital ectopia lentis: Implications for genotype–phenotype relationships Chinese
Front Cover, Volume 43, Issue 11
MicroRNA binding site variation is enriched in psychiatric disorders
Specifications of the ACMG/AMP variant curation guidelines for myocilin: Recommendations from the clingen glaucoma expert panel
Revealing the functions of clonal driver gene mutations in patients based on evolutionary dependencies
Issue Information
Exome sequencing unravels genetic variants associated with chronic kidney disease in Saudi Arabian patients
Juvenile mucopolysaccharidosis plus disease caused by a missense mutation in <i>VPS33A</i>
ENPP1 deficiency: A clinical update on the relevance of individual variants using a locus‐specific patient database
Biallelic loss‐of‐function variants in <i>EXOC6B</i> are associated with impaired primary ciliogenesis and cause spondylo‐epi‐metaphyseal dysplasia with joint laxity type 3
Rare surfactant‐related variants in familial and sporadic pulmonary fibrosis
Biallelic loss‐of‐function mutations in <i>SEPTIN4</i> ( <i>C17ORF47</i> ), encoding a conserved annulus protein, cause thin midpiece spermatozoa and ma
Mutation update for the <i>ACTN2</i> gene
<i>Human Mutation</i> special issue on innovations in genomic diagnostics
Next‐generation sequencing errors due to genetic variation in <i>WRAP53</i> encoding TCAB1 on chromosome 17
Functional characterization of a novel <i>TP53RK</i> mutation identified in a family with Galloway–Mowat syndrome
Destabilization of mutated human PUS3 protein causes intellectual disability
Mutations in the TBX15‐ADAMTS2 pathway associate with a novel soft palate dysplasia
Next‐generation phenotyping contributing to the identification of a 4.7 kb deletion in <i>KANSL1</i> causing Koolen‐de Vries syndrome
Long‐read sequencing for molecular diagnostics in constitutional genetic disorders
Rapid genome sequencing for pediatrics
Analysis of hereditary cancer gene variant classifications from ClinVar indicates a need for regular reassessment of clinical assertions
Long‐read HiFi sequencing of <i>NUDT15</i> : Phased full‐gene haplotyping and pharmacogenomic allele discovery
Issue Information
Cancer‐causing <i>MAP2K1</i> mutation in a mosaic patient with cardio‐facio‐cutaneous syndrome and immunodeficiency
Evaluation of a whole‐exome sequencing pipeline and benchmarking of causal germline variant prioritizers
de novo variant calling identifies cancer mutation signatures in the 1000 Genomes Project
<i>CIC</i> missense variants contribute to susceptibility for spina bifida
A calibrated cell‐based functional assay to aid classification of <i>MLH1</i> DNA mismatch repair gene variants
Front Cover, Volume 43, Issue 10
Kyphoscoliotic Ehlers‐Danlos syndrome caused by pathogenic variants in <i>FKBP14</i> : Further insights into the phenotypic spectrum and pathogenic mechanisms
Long read sequencing and expression studies of<i>AHDC1</i>deletions in Xia‐Gibbs syndrome reveal a novel genetic regulatory mechanism
Phasing of de novo mutations using a scaled‐up multiple amplicon long‐read sequencing approach
A survey of current methods to detect and genotype inversions
Mosaicism in tuberous sclerosis complex: Lowering the threshold for clinical reporting
Biallelic pathogenic variants in <i>COX11</i> are associated with an infantile‐onset mitochondrial encephalopathy
A loss‐of‐function cysteine mutant in fibulin‐3 (EFEMP1) forms aberrant extracellular disulfide‐linked homodimers and alters extracellular matrix composition
AutoCaSc: Prioritizing candidate genes for neurodevelopmental disorders
Clinical, splicing, and functional analysis to classify <i>BRCA2</i> exon 3 variants: Application of a points‐based ACMG/AMP approach
Mutations in <i>OOEP</i> and <i>NLRP5</i> identified in infertile patients with early embryonic arrest
Type II Alexander disease caused by splicing errors and aberrant overexpression of an uncharacterized GFAP isoform
Issue Information
Front Cover, Volume 43, Issue 9
De novo putative loss‐of‐function variants in <i>TAF4</i> are associated with a neuro‐developmental disorder
Expanding the phenotypic variability of <i>MORC2</i> gene mutations: From Charcot‐Marie‐Tooth disease to late‐onset pure motor neuropathy
Functional correlation of genome‐wide DNA methylation profiles in genetic neurodevelopmental disorders
Characterization of a possible founder synonymous variant in <i>TECTA</i> in multiple individuals with autosomal recessive hearing loss
Biallelic <i>NLRP7</i> variants in patients with recurrent hydatidiform mole: A review and expert consensus
Clinical and pathophysiological delineation of musculocontractural Ehlers—Danlos syndrome caused by dermatan sulfate epimerase deficiency (mcEDS‐ <i>DSE</i> ): A detailed and c
Deep intronic <i>NIPBL de novo</i> mutations and differential diagnoses revealed by whole genome and RNA sequencing in Cornelia de Lange syndrome patients
Multi‐omics analysis reveals multiple mechanisms causing Prader–Willi like syndrome in a family with a X;15 translocation
Multiple mechanisms contribute to the phenotypic effects of synonymous variants
Issue Information
Human Mutation special issue on “Variant Effect Prediction"
Front Cover, Volume 43, Issue 8
Isolated aneurysmal disease as an underestimated finding in individuals with <i>JAG1</i> pathogenic variants
Burden of rare variants in arrhythmogenic cardiomyopathy with right dominant form‐associated genes provides new insights for molecular diagnosis and clinical management
Mutation update: The spectra of <i>PLEC</i> sequence variants and related plectinopathies
Biallelic variants in <i>WARS1</i> cause a highly variable neurodevelopmental syndrome and implicate a critical exon for normal auditory function
Dominant TP63 missense variants lead to constitutive activation and premature ovarian insufficiency
<i>WARS1</i> and <i>SARS1</i> : Two tRNA synthetases implicated in autosomal recessive microcephaly
A functional platform for the selection of pathogenic variants of <i>PMM2</i> amenable to rescue via the use of pharmacological chaperones
Rare germline variants in <i>PALB2</i> and <i>BRCA2</i> in familial and sporadic chordoma
Polarized trafficking and copper transport activity of ATP7B: A mutational approach to establish genotype–phenotype correlation in Wilson disease
First splicing variant in <i>HECW2</i> with an autosomal recessive pattern of inheritance and associated with NDHSAL
<i>CAPN3</i> c.1746‐20C&gt;G variant is hypomorphic for LGMD R1 calpain 3‐related
The <i>MAP3K7</i> gene: Further delineation of clinical characteristics and genotype/phenotype correlations
Better and faster is cheaper
Clinical presentation and genetic analyses of neurofibromatosis type 1 in independent patients with monoallelic double de novo closely spaced mutations in the <i>NF1</i> gene
Screening of potential novel candidate genes in schwannomatosis patients
Issue Information
Front Cover, Volume 43, Issue 7
Back Cover, Volume 43, Issue 7
The human ATP‐binding cassette (ABC) transporter superfamily
Guidelines for clinical interpretation of variant pathogenicity using RNA phenotypes
Benchmarking of univariate pleiotropy detection methods applied to epilepsy
De novo truncating <i>NOVA2</i> variants affect alternative splicing and lead to heterogeneous neurodevelopmental phenotypes
Three novel <i>FHL1</i> variants cause a mild phenotype of Emery‐Dreifuss muscular dystrophy
MiRLog and dbmiR: Prioritization and functional annotation tools to study human microRNA sequence variants
DOCKopathies: A systematic review of the clinical pathologies associated with human <i>DOCK</i> pathogenic variants
Seven years since the launch of the Matchmaker Exchange: The evolution of genomic matchmaking
Altered closed state inactivation gating in Kv4.2 channels results in developmental and epileptic encephalopathies in human patients
Autosomal dominantly inherited myopathy likely caused by the <i>TNNT1</i> variant p.(Asp65Ala)
Transcriptome analysis provides critical answers to the “variants of uncertain significance” conundrum
EFEMP1 rare variants cause familial juvenile‐onset open‐angle glaucoma
Partial loss‐of‐function variant in neuregulin 1 identified in family with heritable peripheral neuropathy
Short amplicon reverse transcription‐polymerase chain reaction detects aberrant splicing in genes with low expression in blood missed by ribonucleic acid sequencing analysis for clinical diagnosis
Mutation update: Variants of the <i>ENPP1</i> gene in pathologic calcification, hypophosphatemic rickets, and cutaneous hypopigmentation with punctate keratoderma
Large scale genotype‐ and phenotype‐driven machine learning in Von Hippel‐Lindau disease
Clinical whole‐genome sequencing in cancer diagnosis
Computational analysis of neurodevelopmental phenotypes: Harmonization empowers clinical discovery
The transmission of human mitochondrial DNA in four‐generation pedigrees
uORF‐introducing variants in the 5′UTR of the <i>NIPBL</i> gene as a cause of Cornelia de Lange syndrome
Cas9‐guided haplotyping of three truncation variants in autosomal recessive disease
The TALE never ends: A comprehensive overview of the role of PBX1, a TALE transcription factor, in human developmental defects
Predictive functional assay‐based classification of PMS2 variants in Lynch syndrome
<i>DNAH14</i> variants are associated with neurodevelopmental disorders
Issue Information
Feasibility and limitations of cultured skin fibroblasts for germline genetic testing in hematologic disorders
The fibrillinopathies: New insights with focus on the paradigm of opposing phenotypes for both <i>FBN1</i> and <i>FBN2</i>
STRipy: A graphical application for enhanced genotyping of pathogenic short tandem repeats in sequencing data
Comparison of the frequency of loss‐of‐function <i>LZTR1</i> variants between schwannomatosis patients and the general population
Bioinformatics detection of modulators controlling splicing factor‐dependent intron retention in the human brain
Effects of 14 <i>F9</i> synonymous codon variants on hemophilia B expression: Alteration of splicing along with protein expression
Phenotype‐driven approaches to enhance variant prioritization and diagnosis of rare disease
In Memoriam: Haig H. Kazazian, Jr. (1937–2022)
Issue Information
Phenotypic and mutational spectrum of <i>ROR2</i> ‐related Robinow syndrome
Re‐evaluation of missense variant classifications in <i>NF2</i>
Pathogenic missense variants altering codon 336 of <i>GARS1</i> lead to divergent dominant phenotypes
Comprehensive variant spectrum of the <i>CNGA3</i> gene in patients affected by achromatopsia
Beacon v2 and Beacon networks: A “lingua franca” for federated data discovery in biomedical genomics, and beyond
Tissue‐specific genotype–phenotype correlations among USH2A‐related disorders in the RUSH2A study
A homozygous hypomorphic<i>BNIP1</i>variant causes an increase in autophagosomes and reduced autophagic flux and results in a spondylo‐epiphyseal dysplasia
<i>seqr</i> : A web‐based analysis and collaboration tool for rare disease genomics
Gene–disease relationship evidence: A clinical perspective focusing on ultra‐rare diseases
An expanded phenotype centric benchmark of variant prioritisation tools
An algorithm for optimal testing in co‐segregation analysis
ModelMatcher: A scientist‐centric online platform to facilitate collaborations between stakeholders of rare and undiagnosed disease research
Estimating the proportion of pathogenic variants from breast cancer case–control data: Application to calibration of ACMG/AMP variant classification criteria
Issue Information
PatientMatcher: A customizable Python‐based open‐source tool for matching undiagnosed rare disease patients via the Matchmaker Exchange network
Cover, Volume 43, Issue 3
Clinical exome sequencing—Mistakes and caveats
Variant‐level matching for diagnosis and discovery: Challenges and opportunities
The heterozygous mutations of <i>SLC26A8</i> are not the main actors for male infertility
A clinical laboratory's experience using GeneMatcher—Building stronger gene–disease relationships
The RD‐Connect Genome‐Phenome Analysis Platform: Accelerating diagnosis, research, and gene discovery for rare diseases
Novel <i>CIC</i> variants identified in individuals with neurodevelopmental phenotypes
Intron mutations and early transcription termination in Duchenne and Becker muscular dystrophy
PhenomeCentral: 7 years of rare disease matchmaking
Mutation profiling of the c.1521_1523delCTT (p.Phe508del, F508del) cystic fibrosis transmembrane conductance regulator allele using haplotype‐resolved long‐read next generation sequencing
Discovery of over 200 new and expanded genetic conditions using GeneMatcher
The impact of GeneMatcher on international data sharing and collaboration
Further delineation of auriculocondylar syndrome based on 14 novel cases and reassessment of 25 published cases
Machine learning models for accurate prioritization of variants of uncertain significance
Diagnostic testing laboratories are valuable partners for disease gene discovery: 5‐year experience with GeneMatcher
DECIPHER: Supporting the interpretation and sharing of rare disease phenotype‐linked variant data to advance diagnosis and research
Advances in the development of PubCaseFinder, including the new application programming interface and matching algorithm
Diagnosis and follow‐up of glycogen storage disease (GSD) type VI from the largest GSD center in China
Genome sequencing demonstrates high diagnostic yield in children with undiagnosed global developmental delay/intellectual disability: A prospective study
Qatar genome: Insights on genomics from the Middle East
A novel nonsense variant in the NFE2L1 transcription factor in a patient with developmental delay, hypotonia, genital anomalies, and failure to thrive
Identification of a novel homozygous <i>synthesis of cytochrome c oxidase 2</i> variant in siblings with early‐onset axonal Charcot‐Marie‐Tooth disease
Issue Information
Cover, Volume 43, Issue 2
Variant interpretation: UCSC Genome Browser Recommended Track Sets
Recessive variants in <i>COL25A1</i> gene as novel cause of arthrogryposis multiplex congenita with ocular congenital cranial dysinnervation disorder
Detection of revertant mosaicism in epidermolysis bullosa through Cas9‐targeted long‐read sequencing
Delineation of a novel neurodevelopmental syndrome associated with <i>PAX5</i> haploinsufficiency
Expanding the phenotypic and molecular spectrum of <i>NFS1</i> ‐related disorders that cause functional deficiencies in mitochondrial and cytosolic iron–sulfur cluster containi
Novel biallelic loss of <i>EEF1B2</i> function links to autosomal recessive intellectual disability
Early onset X‐linked female limited high myopia in three multigenerational families caused by novel mutations in the <i>ARR3</i> gene
Molecular characterization of a large cohort of mucopolysaccharidosis patients: Iran Mucopolysaccharidosis RE‐diagnosis study (IMPRESsion)
Targeted next‐generation sequencing in a large series of fetuses with severe renal diseases
Clinico‐radiological features, molecular spectrum, and identification of prognostic factors in developmental and epileptic encephalopathy due to inosine triphosphate pyrophosphatase (ITPase) deficienc
Genomic variants reducing expression of two endocytic receptors in 46,XY differences of sex development
Functionally impaired <i>RPL8</i> variants associated with Diamond–Blackfan anemia and a Diamond–Blackfan anemia‐like phenotype
Distinct sequence features underlie microdeletions and gross deletions in the human genome
Novel germline <i>MET pathogenic variants in French patients with papillary renal cell carcinomas type I</i>