Human Mutation - 2018

214 articles | Last updated: 2025-12-03 14:12:57
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T A T A T A T A
Cover Image, Volume 40, Issue 1
Autosomal recessive primary microcephaly due to <i>ASPM</i> mutations: An update
Issue Information
A recurrent mutation in <i>KCNQ4</i> in Korean families with nonsyndromic hearing loss and rescue of the channel activity by KCNQ activators
Biallelic loss of function variants in <i>PPP1R21</i> cause a neurodevelopmental syndrome with impaired endocytic function
A de novo pathogenic <i>CSNK1E</i> mutation identified by exome sequencing in family trios with epileptic encephalopathy
Lamin A mutation impairs interaction with nucleoporin NUP155 and disrupts nucleocytoplasmic transport in atrial fibrillation
The effect of premature termination codon mutations on <i>CFTR</i> mRNA abundance in human nasal epithelium and intestinal organoids: a basis for read‐through therapies in cyst
The homozygous variant c.797G&gt;A/p.(Cys266Tyr) in<i>PISD</i>is associated with a Spondyloepimetaphyseal dysplasia with large epiphyses and disturbed mitochondrial function
Mutational and phenotypic spectra of <i>KCNE1</i> deficiency in Jervell and Lange‐Nielsen Syndrome and Romano‐Ward Syndrome
<i>EPCAM</i>mutation update: Variants associated with congenital tufting enteropathy and Lynch syndrome
Back Cover, Volume 39, Issue 12
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Cover Image, Volume 39, Issue 12
Evidence of predisposing epimutation in retinoblastoma
A novel autosomal recessive<i>GJB2</i>-associated disorder: Ichthyosis follicularis, bilateral severe sensorineural hearing loss, and punctate palmoplantar keratoderma
Small supernumerary marker chromosomes: A legacy of trisomy rescue?
Disease-causing variants of the conserved +2T of 5′ splice sites can be rescued by engineered U1snRNAs
Construction of cloning-friendly minigenes for mammalian expression of full-length human NF1 isoforms
In silico and in vivo models for Qatari-specific classical homocystinuria as basis for development of novel therapies
Genotype and phenotype variability in Sjögren-Larsson syndrome
The role of translation elongation factor eEF1 subunits in neurodevelopmental disorders
Recessive mutations in the neuronal isoforms of <i>DST</i> , encoding dystonin, lead to abnormal actin cytoskeleton organization and HSAN type VI
Early infantile-onset epileptic encephalopathy 28 due to a homozygous microdeletion involving the <i>WWOX</i> gene in a region of uniparental disomy
Novel<i>POLE</i>pathogenic germline variant in a family with multiple primary tumors results in distinct mutational signatures
<i>NR5A1</i>gene variants repress the ovarian-specific WNT signaling pathway in 46,XX disorders of sex development patients
Allele balance bias identifies systematic genotyping errors and false disease associations
Variable population prevalence estimates of germline <i>TP53</i> variants: A gnomAD-based analysis
Pathogenicity of novel atypical variants leading to choroideremia as determined by functional analyses
Analysis of the <i>TTR</i> gene in the investigation of amyloidosis: A 25-year single UK center experience
<i>IQSEC2</i>mutation update and review of the female-specific phenotype spectrum including intellectual disability and epilepsy
The progression of the ClinGen gene clinical validity classification over time
Evidence‐based assessments of clinical actionability in the context of secondary findings: Updates from ClinGen's Actionability Working Group
The ClinGen Epilepsy Gene Curation Expert Panel—Bridging the divide between clinical domain knowledge and formal gene curation criteria
On the verge of diagnosis: Detection, reporting, and investigation of de novo variants in novel genes identified by clinical sequencing
Adapting crowdsourced clinical cancer curation in CIViC to the ClinGen minimum variant level data community‐driven standards
Cover Image, Volume 39, Issue 11
Issue Information
Assessing the gene–disease association of 19 genes with the RASopathies using the ClinGen gene curation framework
ClinGen advancing genomic data‐sharing standards as a GA4GH driver project
Expert specification of the ACMG/AMP variant interpretation guidelines for genetic hearing loss
ClinGen's GenomeConnect registry enables patient‐centered data sharing
ClinVar database of global familial hypercholesterolemia‐associated DNA variants
ClinGen Allele Registry links information about genetic variants
Integrating somatic variant data and biomarkers for germline variant classification in cancer predisposition genes
The clinical imperative for inclusivity: Race, ethnicity, and ancestry (REA) in genomics
ClinGen Variant Curation Expert Panel experiences and standardized processes for disease and gene‐level specification of the ACMG/AMP guidelines for sequence variant interpretation
Unique aspects of sequence variant interpretation for inborn errors of metabolism (IEM): The ClinGen IEM Working Group and the Phenylalanine Hydroxylase Gene
Specifications of the ACMG/AMP variant curation guidelines for the analysis of germline <i>CDH1</i> sequence variants
ClinGen and ClinVar – Enabling Genomics in Precision Medicine
Gene‐specific criteria for <i>PTEN</i> variant curation: Recommendations from the ClinGen PTEN Expert Panel
Scaling resolution of variant classification differences in ClinVar between 41 clinical laboratories through an outlier approach
The value of genomic variant ClinVar submissions from clinical providers: Beyond the addition of novel variants
Genetic database software as medical devices
ClinVar at five years: Delivering on the promise
Updated recommendation for the benign stand‐alone ACMG/AMP criterion
Framework for microRNA variant annotation and prioritization using human population and disease datasets
Global genetic insight contributed by consanguineous Pakistani families segregating hearing loss
Functional evidence implicating <i>NOTCH2</i> missense mutations in primary ovarian insufficiency etiology
CardioVAI: An automatic implementation of ACMG-AMP variant interpretation guidelines in the diagnosis of cardiovascular diseases
<i>In vitro</i>functional characterization of the novel<i>DHH</i>mutations p.(Asn337Lysfs*24) and p.(Glu212Lys) associated with gonadal dysgenesis
Is evolutionary loss our gain? The role of <i>ACTN3</i> p.Arg577Ter (R577X) genotype in athletic performance, ageing, and disease
The mutation-dependent pathogenicity of<i>NPHS2</i>p.R229Q: A guide for clinical assessment
Antisense oligonucleotides modulate dopa decarboxylase function in aromatic <scp>l</scp> -amino acid decarboxylase deficiency
Novel pathogenic variants in filamin C identified in pediatric restrictive cardiomyopathy
Concurrent <i>AFG3L2</i> and <i>SPG7</i> mutations associated with syndromic parkinsonism and optic atrophy with aberrant OPA1 processing and mitochondrial network fragmentation
Inhibition of mitochondrial translation in fibroblasts from a patient expressing the KARS p.(Pro228Leu) variant and presenting with sensorineural deafness, developmental delay, and lactic acidosis
<i>matchbox</i>: An open-source tool for patient matching via the Matchmaker Exchange
Current review of<i>TP53</i>pathogenic germline variants in breast cancer patients outside Li-Fraumeni syndrome
Cover Image, Volume 39, Issue 10
Issue Information
Diagnosis of Li-Fraumeni Syndrome: Differentiating <i>TP53</i> germline mutations from clonal hematopoiesis
Mutation update for the GPC3 gene involved in Simpson-Golabi-Behmel syndrome and review of the literature
<i>BRCA1</i> and <i>BRCA2</i> 5′ noncoding region variants identified in breast cancer patients alter promoter activity and protein binding
Analysis of novel missense ATR mutations reveals new splicing defects underlying Seckel syndrome
Recommendations for interpreting the loss of function PVS1 ACMG/AMP variant criterion
Insights into the genotype-phenotype correlation and molecular function of SLC25A46
The intellectual disability-associated CAMK2G p.Arg292Pro mutation acts as a pathogenic gain-of-function
Hyaline fibromatosis syndrome: Clinical update and phenotype-genotype correlations
Issue Information
Erratum
<i>STAC3</i>variants cause a congenital myopathy with distinctive dysmorphic features and malignant hyperthermia susceptibility
Back Cover, Volume 39, Issue 9
Cover Image, Volume 39, Issue 9
Association analysis of exome variants and refraction, axial length, and corneal curvature in a European-American population European-American population
A single-center study on 140 patients with cerebral cavernous malformations: 28 new pathogenic variants and functional characterization of a <i>PDCD10</i> large deletion
Corrigendum
Variable cardiovascular phenotypes associated with <i>SMAD2</i> pathogenic variants
Functionally significant, novel<i>GATA4</i>variants are frequently associated with Tetralogy of Fallot
Relationship of electrophysiological dysfunction and clinical severity in<i>SCN2A</i>-related epilepsies
hgvs: A Python package for manipulating sequence variants using HGVS nomenclature: 2018 Update
Characterization of mutation spectrum and identification of novel mutations in <i>ATP7B</i> gene from a cohort of Wilson disease patients: Functional and therapeutic implications
Mucopolysaccharidosis type VI (MPS VI) and molecular analysis: Review and classification of published variants in the<i>ARSB</i>gene
RheoScale: A tool to aggregate and quantify experimentally determined substitution outcomes for multiple variants at individual protein positions
Exploring genetic modifiers of Gaucher disease: The next horizon
Quantifying the potential of functional evidence to reclassify variants of uncertain significance in the categorical and Bayesian interpretation frameworks
Copy number variant discrepancy resolution using the ClinGen dosage sensitivity map results in updated clinical interpretations in ClinVar
Mutations in the gene <i>PDE6C</i> encoding the catalytic subunit of the cone photoreceptor phosphodiesterase in patients with achromatopsia
LINE- and<i>Alu</i>-containing genomic instability hotspot at 16q24.1 associated with recurrent and nonrecurrent CNV deletions causative for ACDMPV
Motor protein binding and mitochondrial transport are altered by pathogenic TUBB4A variants
<i>Alu-Alu</i> mediated intragenic duplications in <i>IFT81</i> and <i>MATN3</i> are associated with skeletal dysplasias
Mutant NR5A1/SF-1 in patients with disorders of sex development shows defective activation of the<i>SOX9</i>TESCO enhancer
<i>LAMA2</i>gene mutation update: Toward a more comprehensive picture of the laminin-α2 variome and its related phenotypes
<i>STUB1</i>polyadenylation signal variant AACAAA does not affect polyadenylation but decreases<i>STUB1</i>translation causing SCAR16
Detection of novel germline mutations in six breast cancer predisposition genes by targeted next-generation sequencing
Disruption of<i>TWIST1</i>translation by 5′ UTR variants in Saethre-Chotzen syndrome
Next generation sequencing identifies double homozygous mutations in two distinct genes (<i>EXPH5</i> and <i>COL17A1</i> ) in a patient with concomitant simplex and junctional epidermolysis bullosa
Homozygosity for <i>FARSB</i> mutation leads to Phe-tRNA synthetase-related disease of growth restriction, brain calcification, and interstitial lung disease
Heterozygous missense mutations in<i>NFATC1</i>are associated with atrioventricular septal defect
Mutation update for <i>CYP4F22</i> variants associated with autosomal recessive congenital ichthyosis
Missense mutations have unexpected consequences: The McArdle disease paradigm
Issue Information
Cover Image, Volume 39, Issue 8
Common genetic causes of holoprosencephaly are limited to a small set of evolutionarily conserved driver genes of midline development coordinated by TGF-β, hedgehog, and FGF signaling
A mutation of<i>SCN1B</i>associated with GEFS+ causes functional and maturation defects of the voltage-dependent sodium channel
A characterization of postzygotic mutations identified in monozygotic twins
Characterization of spliceogenic variants located in regions linked to high levels of alternative splicing:<i>BRCA2</i>c.7976+5G &gt; T as a case study
Rare<i>RELN</i>variants affect Reelin-DAB1 signal transduction in autism spectrum disorder
Whole genome sequencing and mutation rate analysis of trios with paternal dioxin exposure
The analysis of myotonia congenita mutations discloses functional clusters of amino acids within the CBS2 domain and the C-terminal peptide of the ClC-1 channel
Detailed analysis of <i>HTT</i> repeat elements in human blood using targeted amplification-free long-read sequencing
Elucidating the genetic architecture of Adams-Oliver syndrome in a large European cohort European cohort
Targeted resequencing reveals genetic risks in patients with sporadic idiopathic pulmonary fibrosis
Results of next-generation sequencing gene panel diagnostics including copy-number variation analysis in 810 patients suspected of heritable thoracic aortic disorders
<i>DMD</i> genotype correlations from the Duchenne Registry: Endogenous exon skipping is a factor in prolonged ambulation for individuals with a defined mutation subtype
Further delineation of Malan syndrome
Germline variation in the oxidative DNA repair genes<i>NUDT1</i>and<i>OGG1</i>is not associated with hereditary colorectal cancer or polyposis
Cover Image, Volume 39, Issue 7
Issue Information
Real-world clinical applicability of pathogenicity predictors assessed on<i>SERPINA1</i>mutations in alpha-1-antitrypsin deficiency
Targeted sequencing with expanded gene profile enables high diagnostic yield in non-5q-spinal muscular atrophies
De novo mutations in <i>FLNC</i> leading to early-onset restrictive cardiomyopathy and congenital myopathy
Issue Information
Cover Image, Volume 39, Issue 6
Severe neurocognitive and growth disorders due to variation in <i>THOC2</i> , an essential component of nuclear mRNA export machinery
A novel <i>CLCN5</i> pathogenic mutation supports Dent disease with normal endosomal acidification
ClinVar Miner: Demonstrating utility of a Web-based tool for viewing and filtering ClinVar data
Autosomal-dominant early-onset spastic paraparesis with brain calcification due to <i>IFIH1</i> gain-of-function
De novo variants in <i>RHOBTB2</i> , an atypical Rho GTPase gene, cause epileptic encephalopathy
A functional assay for the clinical annotation of genetic variants of uncertain significance in Diamond-Blackfan anemia
Analysis of intratumor heterogeneity in Neurofibromatosis type 1 plexiform neurofibromas and neurofibromas with atypical features: Correlating histological and genomic findings
Improved, ACMG-compliant, in silico prediction of pathogenicity for missense substitutions encoded by <i>TP53</i> variants
Evidence for <i>GALNT12</i> as a moderate penetrance gene for colorectal cancer
Clinical and functional characterization of two novel <i>ZBTB20</i> mutations causing Primrose syndrome
Asthma and allergic rhinitis associate with the <i>rs2229542</i> variant that induces a p.Lys90Glu mutation and compromises AKR1B1 protein levels
Partial loss-of-function of sodium channel <i>SCN8A</i> in familial isolated myoclonus
Mutations and common variants in the human arginase 1 (<i>ARG1</i>) gene: Impact on patients, diagnostics, and protein structure considerations
<i>MECP2</i> variation in Rett syndrome-An overview of current coverage of genetic and phenotype data within existing databases
Marker chromosome genomic structure and temporal origin implicate a chromoanasynthesis event in a family with pleiotropic psychiatric phenotypes
Seshat: A Web service for accurate annotation, validation, and analysis of<i>TP53</i>variants generated by conventional and next-generation sequencing
NGS testing for cardiomyopathy: Utility of adding RASopathy-associated genes
Novel<i>CASK</i>mutations in cases with syndromic microcephaly
Genome sequencing reveals a novel genetic mechanism underlying dihydropyrimidine dehydrogenase deficiency: A novel missense variant c.1700G&gt;A and a large intragenic inversion in <i>DPYD</i> spanni
Whole-genome sequencing in patients with ciliopathies uncovers a novel recurrent tandem duplication in <i>IFT140</i>
De novo mutations in the<i>SET</i>nuclear proto-oncogene, encoding a component of the inhibitor of histone acetyltransferases (INHAT) complex in patients with nonsyndromic intellectual disability
Insight into vitamin B<sub>6</sub>-dependent epilepsy due to<i>PLPBP</i>(previously<i>PROSC</i>) missense mutations
Genotype-specific progression of hereditary medullary thyroid cancer
Cover Image, Volume 39, Issue 5
Abnormal function of the UBA5 protein in a case of early developmental and epileptic encephalopathy with suppression-burst
Issue Information
Unusual association of a unique CAG interruption in 5′ of DM1 CTG repeats with intergenerational contractions and low somatic mosaicism
<i>MERTK</i> mutation update in inherited retinal diseases
Yet another database?
Mutation update for the<i>GPC3</i>gene involved in Simpson-Golabi-Behmel syndrome and review of the literature
Inferring the effect of genomic variation in the new era of genomics
A<i>PIGH</i>mutation leading to GPI deficiency is associated with developmental delay and autism
A homozygous variant disrupting the <i>PIGH</i> start-codon is associated with developmental delay, epilepsy, and microcephaly
Compound heterozygosity for loss-of-function <i>FARSB</i> variants in a patient with classic features of recessive aminoacyl-tRNA synthetase-related disease
Novel approach to functional SNPs discovery from genome-wide data reveals promising variants for colon cancer risk
<i>STX2</i>is a causative gene for nonobstructive azoospermia
MSeqDR mvTool: A mitochondrial DNA Web and API resource for comprehensive variant annotation, universal nomenclature collation, and reference genome conversion
Mutations in the fourth β-propeller domain of LRP4 are associated with isolated syndactyly with fusion of the third and fourth fingers
Tricho-Hepato-Enteric Syndrome mutation update: Mutations spectrum of<i>TTC37</i>and<i>SKIV2L</i>, clinical analysis and future prospects
Robust identification of deletions in exome and genome sequence data based on clustering of Mendelian errors
Neurofibromin (<i>NF1</i> ) genetic variant structure-function analyses using a full-length mouse cDNA
Issue Information
Cover Image, Volume 39, Issue 4
ABCA3 missense mutations causing surfactant dysfunction disorders have distinct cellular phenotypes
A functional polymorphism in the <i>pre-miR-146a</i> gene is associated with the risk of nonsyndromic orofacial cleft
Erratum
Variant pathogenicity evaluation in the community-driven Inherited Neuropathy Variant Browser
The <i>BRCA2</i> c.68-7T &gt; A variant is not pathogenic: A model for clinical calibration of spliceogenicity
Text-mined phenotype annotation and vector-based similarity to improve identification of similar phenotypes and causative genes in monogenic disease patients
Mutational spectrum in a worldwide study of 29,700 families with<i>BRCA1</i>or<i>BRCA2</i>mutations
MeCP2 AT-Hook1 mutations in patients with intellectual disability and/or schizophrenia disrupt DNA binding and chromatin compaction in vitro
Issue Information
Cover Image, Volume 39, Issue 3
Very short DNA segments can be detected and handled by the repair machinery during germline chromothriptic chromosome reassembly
A mutation update on the LDS-associated genes<i>TGFB2/3</i>and<i>SMAD2/3</i>
Secretion of wild-type factor IX upon readthrough over<i>F9</i>pre-peptide nonsense mutations causing hemophilia B
Diagnostic yield of a targeted gene panel in primary ciliary dyskinesia patients
Issue Information
Genotype-phenotype correlations in individuals with pathogenic<i>RERE</i>variants
Mitogenomic differences between the normal and tumor cells of colorectal cancer patients
Cover Image, Volume 39, Issue 2
Mutation update of transcription factor genes<i>FOXE3</i>,<i>HSF4</i>,<i>MAF</i>, and<i>PITX3</i>causing cataracts and other developmental ocular defects
Molecular phenotype of SLC4A11 missense mutants: Setting the stage for personalized medicine in corneal dystrophies
Somatic mutations activating Wiskott-Aldrich syndrome protein concomitant with RAS pathway mutations in juvenile myelomonocytic leukemia patients
Clinical, biochemical, and genetic features associated with <i>VARS2</i> -related mitochondrial disease
Genetic contribution of retinoid-related genes to neural tube defects
Loss-of-function mutations in<i>ISCA2</i>disrupt 4Fe-4S cluster machinery and cause a fatal leukodystrophy with hyperglycinemia and mtDNA depletion
Alu element insertion in<i>PKLR</i>gene as a novel cause of pyruvate kinase deficiency in Middle Eastern patients
Targeted copy number screening highlights an intragenic deletion of <i>WDR63</i> as the likely cause of human occipital encephalocele and abnormal CNS development in zebrafish
<i>MPV17</i>-related mitochondrial DNA maintenance defect: New cases and review of clinical, biochemical, and molecular aspects
Disease-causing mutations in the promoter and enhancer of the ornithine transcarbamylase gene
Methods and tools for assessing the impact of genetic variations: The 2017 Annual Scientific Meeting of the Human Genome Variation Society
Thorough in silico and in vitro cDNA analysis of 21 putative<i>BRCA1</i>and<i>BRCA2</i>splice variants and a complex tandem duplication in<i>BRCA2</i>allowing the identification of activated cryptic s
Exonic mutations and exon skipping: Lessons learned from <i>DFNA5</i>
Nemaline myopathy and distal arthrogryposis associated with an autosomal recessive <i>TNNT3</i> splice variant
<i>Cis</i>variants identified in F508del complex alleles modulate CFTR channel rescue by small molecules
<i>MYO5B</i>,<i>STX3</i>, and<i>STXBP2</i>mutations reveal a common disease mechanism that unifies a subset of congenital diarrheal disorders: A mutation update
Structural and sequence variants in patients with Silver-Russell syndrome or similar features-Curation of a disease database
Autosomal recessive primary microcephaly due to <i>ASPM</i> mutations: An update
A noncoding variant in <i>GANAB</i> explains isolated polycystic liver disease (PCLD) in a large family