Human Mutation - 2017

229 articles | Last updated: 2025-12-03 14:12:57
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Substrate interaction defects in histidyl-tRNA synthetase linked to dominant axonal peripheral neuropathy
Major improvement in the detection of microsatellite instability in colorectal cancer using HSP110 T17 E-<i>ice</i> -COLD-PCR
An evaluation of the challenges to developing tumor BRCA1 and BRCA2 testing methodologies for clinical practice
Whole exome sequencing identifies a germline <i>MET</i> mutation in two siblings with hereditary wild-type <i>RET</i> medullary thyroid cancer
Issue Information
Cover Image, Volume 39, Issue 1
Properties of human genes guided by their enrichment in rare and common variants
Mutations in COQ8B (ADCK4) found in patients with steroid-resistant nephrotic syndrome alter COQ8B function
Inactivation of <i>AMMECR1</i> is associated with growth, bone, and heart alterations
Evaluation of exome filtering techniques for the analysis of clinically relevant genes
<i>EYS</i>mutation update: In silico assessment of 271 reported and 26 novel variants in patients with retinitis pigmentosa
A dystroglycan mutation (p.Cys667Phe) associated to muscle-eye-brain disease with multicystic leucodystrophy results in ER-retention of the mutant protein
A loss-of-function homozygous mutation in <i>DDX59</i> implicates a conserved DEAD-box RNA helicase in nervous system development and function
A nonstop variant in <i>REEP1</i> causes peripheral neuropathy by unmasking a 3′UTR-encoded, aggregation-inducing motif
<i>ERCC4</i> variants identified in a cohort of patients with segmental progeroid syndromes
Urine-derived podocytes-lineage cells: A promising tool for precision medicine in Alport Syndrome
Structural and functional differences in<i>PHOX2B</i>frameshift mutations underlie isolated or syndromic congenital central hypoventilation syndrome
A comprehensive approach to identification of pathogenic FANCA variants in Fanconi anemia patients and their families
Issue Information
Cover Image, Volume 38, Issue 12
Is Li-Fraumeni syndrome really much more common?
Partial and complete SPINK1 deficiency cause distinct pancreatic phenotypes
Gain-of-function<i>HCN2</i>variants in genetic epilepsy
<i>DCC</i>mutation update: Congenital mirror movements, isolated agenesis of the corpus callosum, and developmental split brain syndrome
Expanding the genetic architecture and phenotypic spectrum in the skeletal ciliopathies
Whole exome and whole genome sequencing with dried blood spot DNA without whole genome amplification
Enzyme replacement therapy prevents loss of bone and fat mass in murine homocystinuria
Quantitative mapping of genetic similarity in human heritable diseases by shared mutations
A novel inborn error of the coenzyme Q10 biosynthesis pathway: cerebellar ataxia and static encephalomyopathy due to COQ5 C‐methyltransferase deficiency
<i>CYP21A2</i>mutation update: Comprehensive analysis of databases and published genetic variants
CYP2U1 activity is altered by missense mutations in hereditary spastic paraplegia 56
Functional characterization of five<i>NR5A1</i>gene mutations found in patients with 46,XY disorders of sex development
Functional characterization of novel <i>NR5A1</i> variants reveals multiple complex roles in disorders of sex development
Cover Image, Volume 38, Issue 11
Issue Information
Large differences in proportions of harmful and benign amino acid substitutions between proteins and diseases
Differential regulation of two<i>FLNA</i>transcripts explains some of the phenotypic heterogeneity in the loss-of-function filaminopathies
HUMA: A platform for the analysis of genetic variation in humans
Mild phenotypes and proper supercomplex assembly in human cells carrying the homoplasmic m.15557G &gt; A mutation in cytochrome<i>b</i>gene
Missense variants in the X-linked gene<i>PRPS1</i>cause retinal degeneration in females
VariantValidator: Accurate validation, mapping, and formatting of sequence variation descriptions
The evolution of a Web resource: The Galactosemia Proteins Database 2.0
Severe infantile isolated exocrine pancreatic insufficiency caused by the complete functional loss of the<i>SPINK1</i>gene
Molecular and clinical spectra of FBXL4 deficiency
Loss of interaction between plectin and type XVII collagen results in epidermolysis bullosa simplex
Functional analysis of novel<i>DEAF1</i>variants identified through clinical exome sequencing expands<i>DEAF1</i>-associated neurodevelopmental disorder (DAND) phenotype
Issue Information
Biallelic variants in<i>WARS2</i>encoding mitochondrial tryptophanyl-tRNA synthase in six individuals with mitochondrial encephalopathy
Cover Image, Volume 38, Issue 10
Mutations in<i>KARS</i>cause early-onset hearing loss and leukoencephalopathy: Potential pathogenic mechanism
Identification and characterization of three novel mutations in the<i>CASQ1</i>gene in four patients with tubular aggregate myopathy
A disease-associated mutation in the adhesion GPCR BAI2 (<i>ADGRB2</i>) increases receptor signaling activity
Fibroblasts derived from patients with opsismodysplasia display SHIP2-specific cell migration and adhesion defects
Identification and functional analysis of<i>CORIN</i>variants in hypertensive patients
Consideration of the haplotype diversity at nonallelic homologous recombination hotspots improves the precision of rearrangement breakpoint identification
Higher-than-expected population prevalence of potentially pathogenic germline<i>TP53</i>variants in individuals unselected for cancer history
Molecular genetics of syndromic and non-syndromic forms of parathyroid carcinoma
Functional and molecular studies in primary carnitine deficiency
Issue Information
Cover Image, Volume 38, Issue 9
Assessing predictions of fitness effects of missense mutations in SUMO‐conjugating enzyme UBE2I
Novel ID gene <i>CSNK2B</i> : The crossover from molecular diagnosis to research continues
Aggregation of population‐based genetic variation over protein domain homologues and its potential use in genetic diagnostics
Validation and application of a novel integrated genetic screening method to a cohort of 1,112 men with idiopathic azoospermia or severe oligozoospermia
<i>CNGB3</i> mutation spectrum including copy number variations in 552 achromatopsia patients
<i>PAX7</i>mutation in a syndrome of failure to thrive, hypotonia, and global neurodevelopmental delay
Identification and functional characterization of two missense mutations in <i>NDRG1</i> associated with Charcot‐Marie‐Tooth disease type 4D
Enzymatic characterization of novel arylsulfatase A variants using human arylsulfatase A‐deficient immortalized mesenchymal stromal cells
Truncating mutation in <i>CSNK2B</i> and myoclonic epilepsy
Detection of homozygous deletions in tumor‐suppressor genes ranging from dozen to hundreds nucleotides in cancer models
<i>IDUA</i> mutational profile and genotype–phenotype relationships in UK patients with Mucopolysaccharidosis Type I
Implementing pharmacogenomics in modern health care: The 2017 scientific meeting of the Human Genome Variation Society
Molecular combing reveals complex 4q35 rearrangements in Facioscapulohumeral dystrophy
IFN-γR1 defects: Mutation update and description of the<i>IFNGR1</i>variation database
Equivalent missense variant in the <i>FOXP2</i> and <i>FOXP1</i> transcription factors causes distinct neurodevelopmental disorders
FGF9 mutation causes craniosynostosis along with multiple synostoses
Survival among children with “Lethal” congenital contracture syndrome 11 caused by novel mutations in the gliomedin gene ( <i>GLDN</i> )
HDR-del: A tool based on Hamming distance for prioritizing pathogenic chromosomal deletions in exome sequencing
Identification and functional analysis of an<i>ADAMTSL1</i>variant associated with a complex phenotype including congenital glaucoma, craniofacial, and other systemic features in a three‐generation hu
Leveraging splice‐affecting variant predictors and a minigene validation system to identify Mendelian disease‐causing variants among exon‐captured variants of uncertain significance
Rare coding variants in <i>MAPK7</i> predispose to adolescent idiopathic scoliosis
The role of de novo mutations in the development of amyotrophic lateral sclerosis
Reports from CAGI: The Critical Assessment of Genome Interpretation
Using whole‐exome sequencing to investigate the genetic bases of lysosomal storage diseases of unknown etiology
Issue Information
Pathophysiologic effects of <i>CHCHD2</i> variants associated with late‐onset Parkinson disease
Postzygotic single nucleotide mosaicisms and autism risk
When more really means more: WGS standards and quality control
Cover Image, Volume 38, Issue 8
Analysis of large-scale sequencing cohorts does not support the role of variants in<i>UCP2</i>as a cause of hyperinsulinaemic hypoglycaemia
New role of LRP5, associated with nonsyndromic autosomal-recessive hereditary hearing loss
Characterization of cryptic splicing in germline <i>PTEN</i> intronic variants in Cowden syndrome
<i>BOC</i> is a modifier gene in holoprosencephaly
Compound heterozygosity for loss-of-function<i>GARS</i>variants results in a multisystem developmental syndrome that includes severe growth retardation
Investigating DNA-, RNA-, and protein-based features as a means to discriminate pathogenic synonymous variants
Heterozygous variants in<i>ACTL6A</i>, encoding a component of the BAF complex, are associated with intellectual disability
Quantification of transmission risk in a male patient with a <i>FLNB</i> mosaic mutation causing Larsen syndrome: Implications for genetic counseling in postzygotic mosaicism cases
Working toward precision medicine: Predicting phenotypes from exomes in the Critical Assessment of Genome Interpretation (CAGI) challenges
“Matching” consent to purpose: The example of the Matchmaker Exchange
Issue Information
Assessment of variant pathogenicity in a highly admixed population
Cover Image, Volume 38, Issue 7
MiSynPat: An integrated knowledge base linking clinical, genetic, and structural data for disease-causing mutations in human mitochondrial aminoacyl-tRNA synthetases
Prenatal diagnosis: Down syndrome or more?
DeepBipolar: Identifying genomic mutations for bipolar disorder via deep learning
Semi-automated cancer genome analysis using high-performance computing
<i>CFTR</i>-France, a national relational patient database for sharing genetic and phenotypic data associated with rare<i>CFTR</i>variants
Reduced cell surface levels of GPI-linked markers in a new case with PIGG loss of function
Hermansky-Pudlak syndrome type 2: Aberrant pre-mRNA splicing and mislocalization of granule proteins in neutrophils
A <i>RAB27A</i> duplication in several cases of Griscelli syndrome type 2: An explanation for cases lacking a genetic diagnosis
<i>CSNK2B</i> splice site mutations in patients cause intellectual disability with or without myoclonic epilepsy
Identification of a functional enhancer variant within the chronic pancreatitis‐associated<i>SPINK1</i>c.101A&gt;G (p.Asn34Ser)‐containing haplotype
Recessive mutations in <i>MSTO1</i> cause mitochondrial dynamics impairment, leading to myopathy and ataxia
Objective assessment of the evolutionary action equation for the fitness effect of missense mutations across CAGI‐blinded contests
Mutation of <i>serine/threonine protein kinase 36</i> ( <i>STK36</i> ) causes primary ciliary dyskinesia with a central pair defect
RettBASE: Rett syndrome database update
Matching phenotypes to whole genomes: Lessons learned from four iterations of the personal genome project community challenges
Ensemble variant interpretation methods to predict enzyme activity and assign pathogenicity in the CAGI4 <i>NAGLU</i> (Human N‐acetyl‐glucosaminidase) and <i>UBE2I<
CAGI4 SickKids clinical genomes challenge: A pipeline for identifying pathogenic variants
CAGI4 Crohn's exome challenge: Marker SNP versus exome variant models for assigning risk of Crohn disease
Reporting practices for unsolicited and secondary findings from next‐generation sequencing technologies: Perspectives of laboratory personnel
Missense variant pathogenicity predictors generalize well across a range of function‐specific prediction challenges
Postzygotic single‐nucleotide mosaicisms contribute to the etiology of autism spectrum disorder and autistic traits and the origin of mutations
Determination of disease phenotypes and pathogenic variants from exome sequence data in the CAGI 4 gene panel challenge
No significant enrichment of rare functionally defective <i>CPA1</i> variants in a large Chinese idiopathic chronic pancreatitis cohort
Protein destabilization and loss of protein‐protein interaction are fundamental mechanisms in <i>cblA</i> ‐type methylmalonic aciduria
A recurrent de novo mutation in<i>ACTG1</i>causes isolated ocular coloboma
The novel αB‐crystallin ( <i>CRYAB</i> ) mutation p.D109G causes restrictive cardiomyopathy
Haplotype reference consortium panel: Practical implications of imputations with large reference panels
Deficient activity of alanyl-tRNA synthetase underlies an autosomal recessive syndrome of progressive microcephaly, hypomyelination, and epileptic encephalopathy
The c.797 G&gt;A (p.R266K) cystathionine β-synthase mutation causes homocystinuria by affecting protein stability
Cover Image, Volume 38, Issue 6
Issue Information
Mapping genotype-phenotype associations of nsSNPs in coiled-coil oligomerization domains of the human proteome
De novo<i>IGF2</i>mutation on the paternal allele in a patient with Silver–Russell syndrome and ectrodactyly
<i>In vitro</i>recapitulation of the site-specific editing (to wild-type) of mutant<i>IDS</i>mRNA transcripts, and the characterization of IDS protein translated from the edited mRNAs
Critical points for an accurate human genome analysis
Exploring the limits of the usefulness of mutagenesis in studies of allosteric mechanisms
Performance of in silico tools for the evaluation of p16INK4a (CDKN2A) variants in CAGI
panelcn.MOPS: Copy-number detection in targeted NGS panel data for clinical diagnostics
Large differences in proportions of harmful and benign amino acid substitutions between proteins and diseases
Monogenic diabetes syndromes: Locus-specific databases for Alström, Wolfram, and Thiamine-responsive megaloblastic anemia
Varied pathological and therapeutic response effects associated with<i>CHCHD2</i>mutant and risk variants
HLA‐HD: An accurate HLA typing algorithm for next‐generation sequencing data
Stratified polygenic risk prediction model with application to CAGI bipolar disorder sequencing data
The influence of SNP-based chromosomal microarray and NIPT on the diagnostic yield in 10,000 fetuses with and without fetal ultrasound anomalies
Whole‐protein alanine‐scanning mutagenesis of allostery: A large percentage of a protein can contribute to mechanism
Once doesn't count: Phenotype-driven gene hunting in cohorts
Bad blood contaminating germline databases?
Issue Information
DaMold: A data-mining platform for variant annotation and visualization in molecular diagnostics research
Cover Image, Volume 38, Issue 5
Lessons from the CAGI‐4 Hopkins clinical panel challenge
Genetic variants in microRNAs and their binding sites within gene 3′UTRs associate with susceptibility to age-related macular degeneration
Aberrant<i>HRAS</i>transcript processing underlies a distinctive phenotype within the RASopathy clinical spectrum
Detecting<i>PKD1</i>variants in polycystic kidney disease patients by single-molecule long-read sequencing
Microtubule-associated defects caused by <i>EFHC1</i> mutations in juvenile myoclonic epilepsy
Benchmarking predictions of allostery in liver pyruvate kinase in CAGI4
Exomic variants of an elderly cohort of Brazilians in the ABraOM database
Phenotype-genotype correlations of PIGO deficiency with variable phenotypes from infantile lethality to mild learning difficulties
Purinergic receptors<i>P2RX4</i>and<i>P2RX7</i>in familial multiple sclerosis
A functional SNP regulated by miR-196a-3p in the 3′UTR of<i>FGF2</i>is associated with bone mineral density in the Chinese population Chinese population
Cover Image, Volume 38, Issue 4
Key insights into the protein tyrosine phosphatase <i>PTPN11</i> /SHP2 associated with noonan syndrome and cancer
Issue Information
Overstressed response to <i>EIF2S3</i> variants in MEHMO syndrome
Rare deleterious variants in <i>GRHL3</i> are associated with human spina bifida
Single-base substitutions in the<i>CHM</i>promoter as a cause of choroideremia
Maternal mutations of<i>FOXF1</i>cause alveolar capillary dysplasia despite not being imprinted
Whole-transcriptome sequencing in blood provides a diagnosis of spinal muscular atrophy with progressive myoclonic epilepsy
Characterization of chromosomal abnormalities in pregnancy losses reveals critical genes and loci for human early development
Mutation in mitochondrial complex IV subunit COX5A causes pulmonary arterial hypertension, lactic acidemia, and failure to thrive
TarSeqQC: Quality control on targeted sequencing experiments in R
Mutations of the aminoacyl-tRNA-synthetases SARS and WARS2 are implicated in the etiology of autosomal recessive intellectual disability
<i>EDNRB</i>mutations cause Waardenburg syndrome type II in the heterozygous state
Nonketotic hyperglycinemia: Functional assessment of missense variants in<i>GLDC</i>to understand phenotypes of the disease
Accurate eQTL prioritization with an ensemble‐based framework
PON‐P and PON‐P2 predictor performance in CAGI challenges: Lessons learned
Predicting phenotype from genotype: Improving accuracy through more robust experimental and computational modeling
Pathogenic <i>ASXL1</i> somatic variants in reference databases complicate germline variant interpretation for Bohring-Opitz Syndrome
A variant in a<i>cis</i>-regulatory element enhances claudin-14 expression and is associated with pediatric-onset hypercalciuria and kidney stones
Mutations in genes encoding polycomb repressive complex 2 subunits cause Weaver syndrome
A SAMHD1 mutation associated with Aicardi-Goutières syndrome uncouples the ability of SAMHD1 to restrict HIV-1 from its ability to downmodulate type I interferon in humans
Predicting gene expression in massively parallel reporter assays: A comparative study
Matchmaking facilitates the diagnosis of an autosomal-recessive mitochondrial disease caused by biallelic mutation of the tRNA isopentenyltransferase (<i>TRIT1</i> ) gene
Deficiency of the sphingosine-1-phosphate lyase SGPL1 is associated with congenital nephrotic syndrome and congenital adrenal calcifications
One Gene, Several Diseases: The Characteristics of Pleiotropic Proteins
Issue Information
Cover Image, Volume 38, Issue 3
Annotation of functional impact of voltage-gated sodium channel mutations
Loss-of-Function Mutations in <i>KIF15</i> Underlying a Braddock-Carey Genocopy
An Emerging Female Phenotype with Loss‐of‐Function Mutations in the <i>Aristaless‐</i> Related Homeodomain Transcription Factor <i>ARX</i>
Filamin B Loss-of-Function Mutation in Dimerization Domain Causes Autosomal-Recessive Spondylocarpotarsal Synostosis Syndrome with Rib Anomalies
Axonal Neuropathies due to Mutations in Small Heat Shock Proteins: Clinical, Genetic, and Functional Insights into Novel Mutations
Predicting enhancer activity and variant impact using gkm‐SVM
Mutations in the Human Argininosuccinate Synthetase (<i>ASS1</i>) Gene, Impact on Patients, Common Changes, and Structural Considerations
Classification of Genes: Standardized Clinical Validity Assessment of Gene-Disease Associations Aids Diagnostic Exome Analysis and Reclassifications
Clinically Distinct Phenotypes of Canavan Disease Correlate with Residual Aspartoacylase Enzyme Activity
Blind prediction of deleterious amino acid variations with SNPs&amp;GO
Recurrent Rearrangements of Human Amylase Genes Create Multiple Independent CNV Series
Crohn disease risk prediction—Best practices and pitfalls with exome data
<i>FGFR1</i> Analyses in Four Patients with Hypogonadotropic Hypogonadism with Split-Hand/Foot Malformation: Implications for the Promoter Region
Congenital Disorders of Glycosylation: A Pipeline to Treatment?
Cover Image, Volume 38, Issue 2
Issue Information
Predicting Severity of Disease-Causing Variants
Quantification of Phenotype Information Aids the Identification of Novel Disease Genes
Structural, Functional, and Clinical Characterization of a Novel<i>PTPN11</i>Mutation Cluster Underlying Noonan Syndrome
Transdifferentiation of Human Dermal Fibroblasts to Smooth Muscle-Like Cells to Study the Effect of<i>MYH11</i>and<i>ACTA2</i>Mutations in Aortic Aneurysms
ORAI1 Mutations with Distinct Channel Gating Defects in Tubular Aggregate Myopathy
<i>In Silico</i>Functional Meta-Analysis of 5,962<i>ABCA4</i>Variants in 3,928 Retinal Dystrophy Cases
Flexible and Scalable Full-Length CYP2D6 Long Amplicon PacBio Sequencing
Compound Heterozygosity for Null Mutations and a Common Hypomorphic Risk Haplotype in<i>TBX6</i>Causes Congenital Scoliosis
<i>EIF2S3</i>Mutations Associated with Severe X-Linked Intellectual Disability Syndrome MEHMO
Somatic <i>MED12</i> Nonsense Mutation Escapes mRNA Decay and Reveals a Motif Required for Nuclear Entry
Non-Coding Variation: The 2016 Annual Scientific Meeting of the Human Genome Variation Society
Heterozygous Pathogenic Variant in<i>DACT1</i>Causes an Autosomal-Dominant Syndrome with Features Overlapping Townes-Brocks Syndrome
Mendelian Disorders of Cornification Caused by Defects in Intracellular Calcium Pumps: Mutation Update and Database for Variants in ATP2A2 and ATP2C1 Associated with Darier Disease and Hailey-Hailey D
Synonymous Somatic Variants in Human Cancer Are Not Infamous: A Plea for Full Disclosure in Databases and Publications
Doublet-Mediated DNA Rearrangement-A Novel and Potentially Underestimated Mechanism for the Formation of Recurrent Pathogenic Deletions
The Clinical Next-Generation Sequencing Database: A Tool for the Unified Management of Clinical Information and Genetic Variants to Accelerate Variant Pathogenicity Classification
<i>TBK1</i> Mutation Spectrum in an Extended European Patient Cohort with Frontotemporal Dementia and Amyotrophic Lateral Sclerosis Extended European Patient Cohort
PERCH: A Unified Framework for Disease Gene Prioritization
Understanding the Genomic Structure of Copy-Number Variation of the Low-Affinity Fcγ Receptor Region Allows Confirmation of the Association of<i>FCGR3B</i>Deletion with Rheumatoid Arthritis
Assessment of<i>TP53</i>Polymorphisms and<i>MDM2</i>SNP309 in Premenopausal Breast Cancer Risk
Landscape of Pleiotropic Proteins Causing Human Disease: Structural and System Biology Insights
Rare Deleterious<i>PARD3</i>Variants in the aPKC-Binding Region are Implicated in the Pathogenesis of Human Cranial Neural Tube Defects Via Disrupting Apical Tight Junction Formation
Detecting AGG Interruptions in Male and Female FMR1 Premutation Carriers by Single-Molecule Sequencing
Clinical and Molecular Characteristics of SLC16A2 (MCT8) Mutations in Three Families with the Allan-Herndon-Dudley Syndrome