Human Mutation - 2016

189 articles | Last updated: 2025-12-03 14:12:57
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T A T A T A T A
Issue Information
Cover Image, Volume 38, Issue 1
Are Functional Assays for Pathogenicity Assessment of Genetic Variants Overrated?
SLC4A11 Three-Dimensional Homology Model Rationalizes Corneal Dystrophy-Causing Mutations
Diagnostic Targeted Resequencing in 349 Patients with Drug-Resistant Pediatric Epilepsies Identifies Causative Mutations in 30 Different Genes
Whole-Genome Sequencing of Cytogenetically Balanced Chromosome Translocations Identifies Potentially Pathological Gene Disruptions and Highlights the Importance of Microhomology in the Mechanism of Fo
Human<i>RECQ</i>Helicase Pathogenic Variants, Population Variation and “Missing” Diseases
A Cell Type-Specific Expression Signature Predicts Haploinsufficient Autism-Susceptibility Genes
Issue Information
Cover Image, Volume 37, Issue 12
High-Throughput Sequencing in the Context of Human Genetic Diseases: Now and Tomorrow
How to Define Pathogenicity, Health, and Disease?
Mutations in<i>TRAPPC11</i>are associated with a congenital disorder of glycosylation
Comparison of Bioinformatics Prediction, Molecular Modeling, and Functional Analyses of<i>FOXC1</i>Mutations in Patients with Axenfeld-Rieger Syndrome
Functional Characterization and Rescue of a Deep Intronic Mutation in<i>OCRL</i>Gene Responsible for Lowe Syndrome
Pharmacological Chaperoning: A Potential Treatment for PMM2-CDG
Novel<i>BRCA1</i>and<i>BRCA2</i>Tumor Test as Basis for Treatment Decisions and Referral for Genetic Counselling of Patients with Ovarian Carcinomas
Characterization of a Rare Nonpathogenic Methylenetetrahydrofolatereductase (<i>MTHFR</i>) Gene Mutation p.Lys215del in a Southern Italian family Southern Italian family
The<i>CHRNA5/CHRNA3/CHRNB4</i>Nicotinic Receptor Regulome: Genomic Architecture, Regulatory Variants, and Clinical Associations
The Human Serotonin Type 3 Receptor Gene (<i>HTR3A-E</i>) Allelic Variant Database
The Importance of Assessment of Function in the Era of <i>In Silico</i> Analysis
Cover Image, Volume 37, Issue 11
Issue Information
11p15 ICR1 Partial Deletions Associated with<i>IGF2/H19</i>DMR Hypomethylation and Silver-Russell Syndrome
Mutation in<i>SSUH2</i>Causes Autosomal-Dominant Dentin Dysplasia Type I
mirVAFC: A Web Server for Prioritizations of Pathogenic Sequence Variants from Exome Sequencing Data via Classifications
A Comprehensive Functional Analysis of<i>NTRK1</i>Missense Mutations Causing Hereditary Sensory and Autonomic Neuropathy Type IV (HSAN IV)
Investigating the Molecular Mechanisms Behind Uncharacterized Cysteine Losses from Prediction of Their Oxidation State
Deletions Overlapping<i>VCAN</i>Exon 8 Are New Molecular Defects for Wagner Disease
<i>WRN</i>Mutation Update: Mutation Spectrum, Patient Registries, and Translational Prospects
Gene Variant Databases and Sharing: Creating a Global Genomic Variant Database for Personalized Medicine
Am I My Family's Keeper? Disclosure Dilemmas in Next-Generation Sequencing
WES/WGS Reporting of Mutations from Cardiovascular “Actionable” Genes in Clinical Practice: A Key Role for UMD Knowledgebases in the Era of Big Databases
Content Analysis of Informed Consent for Whole Genome Sequencing Offered by Direct-to-Consumer Genetic Testing Companies
An Application of NGS for Molecular Investigations in Perrault Syndrome: Study of 14 Families and Review of the Literature
Increased Population Risk of<i>AIP</i>-Related Acromegaly and Gigantism in Ireland
BRCA Share: A Collection of Clinical BRCA Gene Variants
Assessment of the InSiGHT Interpretation Criteria for the Clinical Classification of 24<i>MLH1</i>and<i>MSH2</i>Gene Variants
Cover Image, Volume 37, Issue 10
Issue Information
Cancer Genetics May Aid Diagnostics of Developmental Disorders
Both Generic and Protein-Specific Tolerance Predictors Are Needed
Chondroitin Sulfate<i>N</i>-acetylgalactosaminyltransferase-1 (CSGalNAcT-1) Deficiency Results in a Mild Skeletal Dysplasia and Joint Laxity
Actionable Genes, Core Databases, and Locus-Specific Databases
How to Identify Pathogenic Mutations among All Those Variations: Variant Annotation and Filtration in the Genome Sequencing Era
From Wet‐Lab to Variations: Concordance and Speed of Bioinformatics Pipelines for Whole Genome and Whole Exome Sequencing
ExonImpact: Prioritizing Pathogenic Alternative Splicing Events
Identical<i>NR5A1</i>Missense Mutations in Two Unrelated 46,XX Individuals with Testicular Tissues
Analysis of Heteroplasmic Variants in the Cardiac Mitochondrial Genome of Individuals with Down Syndrome
Genotype-Phenotype Correlations of Malignant Hyperthermia and Central Core Disease Mutations in the Central Region of the RYR1 Channel
Functional SNP in 3′-UTR MicroRNA-Binding Site of<i>ZNF350</i>Confers Risk for Age-Related Cataract
Mini-Exome Coupled to Read-Depth Based Copy Number Variation Analysis in Patients with Inherited Ataxias
Acute Intermittent Porphyria: Predicted Pathogenicity of<i>HMBS</i>Variants Indicates Extremely Low Penetrance of the Autosomal Dominant Disease
Classification of Missense Variants in<i>XRCC2</i>by Functional Analysis: Implications for Breast Cancer Association Studies
Cover Image, Volume 37, Issue 9
Silent or Not Silent? Consequences of the Human mt-cyb Polymorphism
Issue Information
Noncoding RNA: Current Deep Sequencing Data Analysis Approaches and Challenges
Base-Biased Evolution of Disease-Associated Mutations in the Human Genome
Clinical Interpretation of Variants from Next-Generation Sequencing: The 2016 Scientific Meeting of the Human Genome Variation Society
Loss of Major DNase I Hypersensitive Sites in Duplicated<i>β-globin</i>Gene Cluster Incompletely Silences<i>HBB</i>Gene Expression
Novel Genetic, Clinical, and Pathomechanistic Insights into TFG-Associated Hereditary Spastic Paraplegia
Molecular Chaperones in the Pathogenesis of Amyotrophic Lateral Sclerosis: The Role of HSPB1
Effects of Different Variants in the<i>ENPP1</i>Gene on the Functional Properties of Ectonucleotide Pyrophosphatase/Phosphodiesterase Family Member 1
<i>TMEM231</i>Gene Conversion Associated with Joubert and Meckel-Gruber Syndromes in the Same Family
Identification of a Large<i>DNAJB2</i>Deletion in a Family with Spinal Muscular Atrophy and Parkinsonism
New Directions in Gaucher Disease
Three‐Dimensional Model of Human Nicotinamide Nucleotide Transhydrogenase (NNT) and Sequence‐Structure Analysis of its Disease‐Causing Variations
Discovery and Functional Annotation of<i>PRSS1</i>Promoter Variants in Chronic Pancreatitis
Comprehensive Mutation Analysis of<i>PMS2</i>in a Large Cohort of Probands Suspected of Lynch Syndrome or Constitutional Mismatch Repair Deficiency Syndrome
Regulatory Single-Nucleotide Variant Predictor Increases Predictive Performance of Functional Regulatory Variants
Fourth Generation of Next‐Generation Sequencing Technologies: Promise and Consequences
Deep Intronic Sequence Variants in<i>COL2A1</i>Affect the Alternative Splicing Efficiency of Exon 2, and May Confer a Risk for Rhegmatogenous Retinal Detachment
Mutations Causing Slow-Channel Myasthenia Reveal That a Valine Ring in the Channel Pore of Muscle AChR is Optimized for Stabilizing Channel Gating
Mutational Spectrum of<i>MYO15A</i>and the Molecular Mechanisms of DFNB3 Human Deafness
Variations in the Genome: The Mutation Detection 2015 Meeting on Detection, Genome Sequencing, and Interpretation
Mutational Spectrum in Holoprosencephaly Shows That FGF is a New Major Signaling Pathway
Coagulation Factor XIIIA Subunit Missense Mutations Affect Structure and Function at the Various Steps of Factor XIII Action
Deep Genetic Connection Between Cancer and Developmental Disorders
MicroRNA Genetic Variation: From Population Analysis to Functional Implications of Three Allele Variants Associated with Cancer
Closing the gap in Genetic Data Analysis pipelines with ASPIREdb
Issue Information
Skewed X-inactivation and Females with Intellectual Disability
Silent Tyrosinemia Type I Without Elevated Tyrosine or Succinylacetone Associated with Liver Cirrhosis and Hepatocellular Carcinoma
The Complementarity Between Protein-Specific and General Pathogenicity Predictors for Amino Acid Substitutions
Cover Image, Volume 37, Issue 8
Inborn Error of Cobalamin Metabolism Associated with the Intracellular Accumulation of Transcobalamin-Bound Cobalamin and Mutations in<i>ZNF143</i>, Which Codes for a Transcriptional Activator
MonoSeq Variant Caller Reveals Novel Mononucleotide Run Indel Mutations in Tumors with Defective DNA Mismatch Repair
Ectrodactyly and Lethal Pulmonary Acinar Dysplasia Associated with Homozygous<i>FGFR2</i>Mutations Identified by Exome Sequencing
A Role for the Chromatin‐Remodeling Factor<i>BAZ1A</i>in Neurodevelopment
<i>TP53</i>Variations in Human Cancers: New Lessons from the IARC TP53 Database and Genomics Data
Biallelic Mutations in <i>DNM1L</i> are Associated with a Slowly Progressive Infantile Encephalopathy
<i>DCDC2</i>Mutations Cause Neonatal Sclerosing Cholangitis
Somatically Acquired LINE-1 Insertions in Normal Esophagus Undergo Clonal Expansion in Esophageal Squamous Cell Carcinoma
Mutation Update for Kabuki Syndrome Genes<i>KMT2D</i>and<i>KDM6A</i>and Further Delineation of X-Linked Kabuki Syndrome Subtype 2
Recessive <i>PIGN</i> Mutations in Fryns Syndrome: Evidence for Genetic Heterogeneity
Cover Image, Volume 37, Issue 7
Super-Transactivation<i>TP53</i>Variant in the Germline of a Family with Li-Fraumeni Syndrome
Ehlers-Danlos Syndrome Caused by Biallelic<i>TNXB</i>Variants in Patients with Congenital Adrenal Hyperplasia
Issue Information
Human Mitochondrial Cytochrome <i>b</i> Variants Studied in Yeast: Not All Are Silent Polymorphisms
Splicing Defect in Mitochondrial Seryl-tRNA Synthetase Gene Causes Progressive Spastic Paresis Instead of HUPRA Syndrome
The SSV Evaluation System: A Tool to Prioritize Short Structural Variants for Studies of Possible Regulatory and Causal Variants
Arginine-Glycine Amidinotransferase Deficiency and Functional Characterization of Missense Variants in<i>GATM</i>
Functional Analysis of Missense Variants in the Putative Breast Cancer Susceptibility Gene<i>XRCC2</i>
A Review of Whole-Exome Sequencing Efforts Toward Hereditary Breast Cancer Susceptibility Gene Discovery
Extreme Growth Failure is a Common Presentation of Ligase IV Deficiency
A Significant Regulatory Mutation Burden at a High-Affinity Position of the CTCF Motif in Gastrointestinal Cancers
Identification of 83 Novel Alpha-Mannosidosis-Associated Sequence Variants: Functional Analysis of<i>MAN2B1</i>Missense Mutations
Molecular Genetic Characterization of 151<i>Mut</i>-Type Methylmalonic Aciduria Patients and Identification of 41 Novel Mutations in<i>MUT</i>
Interactive Exploration, Analysis, and Visualization of Complex Phenome-Genome Datasets with ASPIREdb
Identification of Intellectual Disability Genes in Female Patients with a Skewed X-Inactivation Pattern
Identification of Intragenic Exon Deletions and Duplication of<i>TCF12</i>by Whole Genome or Targeted Sequencing as a Cause of<i>TCF12</i>-Related Craniosynostosis
Mutations in<i>GAS8</i>, a Gene Encoding a Nexin-Dynein Regulatory Complex Subunit, Cause Primary Ciliary Dyskinesia with Axonemal Disorganization
Enrichment of SNPs in Functional Categories Reveals Genes Affecting Complex Traits
Mitochondrial DNA Variation and Heteroplasmy in Monozygotic Twins Clinically Discordant for Multiple Sclerosis
Three Novel Heterozygous Point Mutations of<i>NR3C1</i>Causing Glucocorticoid Resistance
Cover Image, Volume 37, Issue 6
Issue Information
Gain-of-Function Mutations in<i>RARB</i>Cause Intellectual Disability with Progressive Motor Impairment
Rare Noncoding Mutations Extend the Mutational Spectrum in the <i>PGAP3</i> Subtype of Hyperphosphatasia with Mental Retardation Syndrome
Expanding the Phenotype Associated with NAA10‐Related N‐Terminal Acetylation Deficiency
Functional and Clinical Consequences of Novel α-Galactosidase A Mutations in Fabry Disease
Cover Image, Volume 37, Issue 5
Issue Information
Strømme Syndrome Is a Ciliary Disorder Caused by Mutations in CENPF
Complex Copy Number Variation of <i>AMY1</i> does not Associate with Obesity in two East Asian Cohorts East Asian
DNA Diagnostics of Hereditary Hearing Loss: A Targeted Resequencing Approach Combined with a Mutation Classification System
High Frequency of Pathogenic Rearrangements in <i>SPG11</i> and Extensive Contribution of Mutational Hotspots and Founder Alleles
A Homozygous<i>Nme7</i>Mutation Is Associated with<i>Situs Inversus Totalis</i>
Fryns Syndrome Associated with Recessive Mutations in PIGN in two Separate Families
Understanding N-Acetyl-L-Glutamate Synthase Deficiency: Mutational Spectrum, Impact of Clinical Mutations on Enzyme Functionality, and Structural Considerations
Mucolipidosis III <i>GNPTG</i> Missense Mutations Cause Misfolding of the γ Subunit of GlcNAc-1-Phosphotransferase
Infectious Enthusiasm! Larger than Life! That Laugh! That Smile! In Loving Memory of Richard G.H. (Dick) Cotton
25 Years of<i>Human Mutation</i>
NGS-Based Assay for the Identification of Individuals Carrying Recessive Genetic Mutations in Reproductive Medicine
The Human Variome Project
Variation Interpretation Predictors: Principles, Types, Performance, and Choice
A Mechanistic Link between L1 Retrotransposition and Chromothripsis
Cover Image, Volume 37, Issue 4
Issue Information
A Distinct Class of Chromoanagenesis Events Characterized by Focal Copy Number Gains
HGVS Recommendations for the Description of Sequence Variants: 2016 Update
Gene Variant Databases and Sharing: Creating a Global Genomic Variant Database for Personalized Medicine
ALG1-CDG: Clinical and Molecular Characterization of 39 Unreported Patients
What's in a Name? A Coordinated Approach toward the Correct Use of a Uniform Nomenclature to Improve Patient Reports and Databases
MSeqDR: A Centralized Knowledge Repository and Bioinformatics Web Resource to Facilitate Genomic Investigations in Mitochondrial Disease
LSDBs and How They Have Evolved
Adding In Silico Assessment of Potential Splice Aberration to the Integrated Evaluation of <i>BRCA</i> Gene Unclassified Variants
Marfan Syndrome and Related Disorders: 25 Years of Gene Discovery
Genetics of Phenylketonuria: Then and Now
Human Variome Project Quality Assessment Criteria for Variation Databases
HGVS Nomenclature in Practice: An Example from the United Kingdom National External Quality Assessment Scheme
Prioritizing Variants in Complete Hereditary Breast and Ovarian Cancer Genes in Patients Lacking Known<i>BRCA</i>Mutations
Understanding the Pathogenicity of Noncoding Mismatch Repair Gene Promoter Variants in Lynch Syndrome
Mutation Update and Review of Severe Methylenetetrahydrofolate Reductase Deficiency
CoNVaDING: Single Exon Variation Detection in Targeted NGS Data
Microdeletions, Rearrangements, and Cancer Susceptibility
Issue Information
Cover Image, Volume 37, Issue 3
Extensive Variation in the Mutation Rate Between and Within Human Genes Associated with Mendelian Disease
Mutations of the RTEL1 Helicase in a Hoyeraal-Hreidarsson Syndrome Patient Highlight the Importance of the ARCH Domain
UMD‐Predictor: A High‐Throughput Sequencing Compliant System for Pathogenicity Prediction of any Human cDNA Substitution
ESHG Plenary Debate 2015: Should Clinical Geneticists have their Genome Sequenced?
Paternal or Maternal Uniparental Disomy of Chromosome 16 Resulting in Homozygosity of a Mutant Allele Causes Fanconi Anemia
MYO3A Causes Human Dominant Deafness and Interacts with Protocadherin 15-CD2 Isoform
mutation3D: Cancer Gene Prediction Through Atomic Clustering of Coding Variants in the Structural Proteome
Strømme Syndrome Is a Ciliary Disorder Caused by Mutations in<i>CENPF</i>
Pathogenicity Interpretation in the Age of Precision Medicine: The 2015 Annual Scientific Meeting of the Human Genome Variation Society
Cover Image, Volume 37, Issue 2
Issue Information
<i>DGAT2</i>Mutation in a Family with Autosomal-Dominant Early-Onset Axonal Charcot-Marie-Tooth Disease
VariOtator, a Software Tool for Variation Annotation with the Variation Ontology
Systematic Analysis of<i>CCNO</i>Variants in a Defined Population: Implications for Clinical Phenotype and Differential Diagnosis
Classification and Clinical Management of Variants of Uncertain Significance in High Penetrance Cancer Predisposition Genes
Germline Chromothripsis Driven by L1-Mediated Retrotransposition and Alu/Alu Homologous Recombination
Does IARS2 Deficiency Cause an Intrinsic Disorder of Bone Development (Skeletal Dysplasia) or Are the Reported Skeletal Changes Secondary to Growth Hormone Deficiency and Neuromuscular Involvement?
De Novo Truncating Mutations in the Kinetochore-Microtubules Attachment Gene<i>CHAMP1</i>Cause Syndromic Intellectual Disability
Variants Within<i>TSC2</i>Exons 25 and 31 Are Very Unlikely to Cause Clinically Diagnosable Tuberous Sclerosis
ECGene: A Literature‐Based Knowledgebase of Endometrial Cancer Genes
Sporadic and Familial Congenital Cataracts: Mutational Spectrum and New Diagnoses Using Next‐Generation Sequencing
Functional Evaluation of Nine Missense-Type Variants of the Human DNA Glycosylase Enzyme MUTYH in the Japanese Population Japanese population
MESP1 Mutations in Patients with Congenital Heart Defects
Weaver Syndrome‐Associated EZH2 Protein Variants Show Impaired Histone Methyltransferase Function In Vitro
Enhanced SRSF5 Protein Expression Reinforces Lamin A mRNA Production in HeLa Cells and Fibroblasts of Progeria Patients
Mutations in<i>FLNC</i>are Associated with Familial Restrictive Cardiomyopathy
<i>ADIPOR1</i>Is Mutated in Syndromic Retinitis Pigmentosa
The Contribution of Whole Gene Deletions and Large Rearrangements to the Mutation Spectrum in Inherited Tumor Predisposing Syndromes
dbNSFP v3.0: A One-Stop Database of Functional Predictions and Annotations for Human Nonsynonymous and Splice-Site SNVs