Human Mutation - 2012

235 articles | Last updated: 2025-12-03 14:12:57
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T A T A T A T A
The CDC Hemophilia A Mutation Project (CHAMP) Mutation List: A New Online Resource
Jagged1 (<i>JAG1</i>) Mutations in Alagille Syndrome: Increasing the Mutation Detection Rate
General Olfactory Sensitivity Data-base: A Unique Knowledgebase for the least Explored of our Major Senses
Making Sense of Unclassified Missense Substitutions in MMR Genes
Whole Exome Sequencing as a Tool for Autozygosity Mapping
Comprehensive Functional Assessment of<i>MLH1</i>Variants of Unknown Significance
Mutations in<i>SYNGAP1</i>Cause Intellectual Disability, Autism, and a Specific Form of Epilepsy by Inducing Haploinsufficiency
Functional Characterization of Novel Mutations Affecting Survivin (BIRC5)-Mediated Therapy Resistance in Head and Neck Cancer Patients
The UBIAD1 Prenyltransferase Links Menaquione-4 Synthesis to Cholesterol Metabolic Enzymes
High-Specificity Single-Tube Multiplex Genotyping Using Ribo-PAP PCR, Tag Primers, Alkali Cleavage of RNA/DNA Chimeras and MALDI-TOF MS
Molecular Analysis of the Rare In(Lu) Blood Type: Toward Decoding the Phenotypic Outcome of Haploinsufficiency for the Transcription Factor KLF1
Mutations in<i>NLRP</i>7 and<i>KHDC</i>3<i>L</i>Confer a Complete Hydatidiform Mole Phenotype on Digynic Triploid Conceptions
Primary Microcephaly, Impaired DNA Replication, and Genomic Instability Caused by Compound Heterozygous<i>ATR</i>Mutations
Autozygosity Mapping with Exome Sequence Data
A Novel Regulatory Defect in the Branched-Chain α-Keto Acid Dehydrogenase Complex Due to a Mutation in the<i>PPM</i><i>1</i><i>K</i>Gene Causes a Mild Variant Phenotype of Maple Syrup Urine Disease
Mechanisms of Formation of Structural Variation in a Fully Sequenced Human Genome
Kohlschütter-Tönz Syndrome: Mutations in<i>ROGDI</i>and Evidence of Genetic Heterogeneity
Statistical Analysis of Missense Mutation Classifiers
<i>FLT</i><i>4</i>/<i>VEGFR</i><i>3</i>and Milroy Disease: Novel Mutations, a Review of Published Variants and Database Update
KDM6A Point Mutations Cause Kabuki Syndrome
Unilateral Cryptorchidism in Mice Mutant for<i>Ptgds</i>
Destabilization and Mislocalization of POU3F4 by C-Terminal Frameshift Truncation and Extension Mutation
Functional Analysis of Missense Mutations of<i>OAT</i>, Causing Gyrate Atrophy of Choroid and Retina
Alternative Splicing of In-Frame Exon Associated with Premature Termination Codons: Implications for Readthrough Therapies
Genome-Wide Analysis of Human SNPs at Long Intergenic Noncoding RNAs
A homozygous missense mutation in<i>HERC2</i>associated with global developmental delay and autism spectrum disorder
The first human mitotic nonallelic homologous recombination hotspot associated with genetic disease
Identification and Biochemical Characterization of a Novel Mutation in<i>DDX11</i>Causing Warsaw Breakage Syndrome
Novel Mutations Including Deletions of the Entire<i>OFD1</i>Gene in 30 Families with Type 1 Orofaciodigital Syndrome: A Study of the Extensive Clinical Variability
Whole Exome Sequencing Reveals Uncommon Mutations in the Recently Identified Fanconi Anemia Gene<i>SLX</i><i>4</i>/<i>FANCP</i>
Predicting the Functional, Molecular, and Phenotypic Consequences of Amino Acid Substitutions using Hidden Markov Models
Identification of Novel Mutations Confirms<i>Pde4d</i>as a Major Gene Causing Acrodysostosis
The Facial Evolution: Looking Backward and Moving Forward
A Novel<i>RAB33B</i>Mutation in Smith-McCort Dysplasia
Classic EDS: Simply a type v collagen deficiency story?
Not All Floating-Harbor Syndrome Cases are Due to Mutations in Exon 34 of<i>SRCAP</i>
Inverted Low-Copy Repeats and Genome Instability-A Genome-Wide Analysis
A Multifactorial Likelihood Model for MMR Gene Variant Classification Incorporating Probabilities Based on Sequence Bioinformatics and Tumor Characteristics: A Report from the Colon Cancer Family Regi
Description of a Large Family with Autosomal Dominant Hypercholesterolemia Associated with the<i>APOE</i>p.Leu167del Mutation
Calibration of Multiple In Silico Tools for Predicting Pathogenicity of Mismatch Repair Gene Missense Substitutions
General Olfactory Sensitivity Database (GOSdb): Candidate Genes and their Genomic Variations
Analysis of the<i>C</i><i>9orf72</i>Gene in Patients with Amyotrophic Lateral Sclerosis in Spain and Different Populations Worldwide
A Loss-of-Function Variant in the Human Histidyl-tRNA Synthetase (<i>HARS</i>) Gene is Neurotoxic In Vivo
Genetic Variant on<i>PDGFRL</i>Associated with Behçet Disease in Chinese Han Populations
JP-45/<i>JSRP</i><i>1</i>Variants Affect Skeletal Muscle Excitation-Contraction Coupling by Decreasing the Sensitivity of the Dihydropyridine Receptor
Mutation Detection by Clonal Sequencing of PCR Amplicons and Grouped Read Typing is Applicable to Clinical Diagnostics
Functional Assessment of<i>TSC</i>2 Variants Identified in Individuals with Tuberous Sclerosis Complex
VariBench: A Benchmark Database for Variations
Mutation Analysis of the<i>IL</i><i>36</i><i>RN</i>Gene in 14 Japanese Patients with Generalized Pustular Psoriasis Japanese
Analysis of the Novel Fanconi Anemia Gene<i>SLX4</i>/<i>FANCP</i>in Familial Breast Cancer Cases
A Novel Deletion in<i>SMPX</i>Causes a Rare form of X-Linked Progressive Hearing Loss in Two Families Due to a Founder Effect
Databases for neurogenetics: Introduction, overview, and challenges
Investigation of the Relationship Between Prostate Cancer and<i>MSMB</i>and<i>NCOA4</i>Genetic Variants and Protein Expression
Somatic Expansion in Mouse and Human Carriers of Fragile X Premutation Alleles
Mutations of<i>ANK3</i>identified by exome sequencing are associated with autism susceptibility
Loss of function mutation in LARP7, chaperone of 7SK ncRNA, causes a syndrome of facial dysmorphism, intellectual disability, and primordial dwarfism
Correlating Multiallelic Copy Number Polymorphisms with Disease Susceptibility
Multiple Functional Effects of RET Kinase Domain Sequence Variants in Hirschsprung Disease
Joint Analysis of SNPs and Proteins Identifies Regulatory<i>IL18</i>Gene Variations Decreasing the Chance of Spastic Cerebral Palsy
Identification of recurrent type-2<i>NF1</i>microdeletions reveals a mitotic nonallelic homologous recombination hotspot underlying a human genomic disorder
Pathological assessment of mismatch repair gene variants in Lynch syndrome: Past, present, and future
Naturally Occurring Genetic Variants of Human Caspase-1 Differ Considerably in Structure and the Ability to Activate Interleukin-1β
Comprehensive Clinical and Molecular Analysis of 12 Families with Type 1 Recessive Cutis Laxa
ALX4 gain-of-function mutations in nonsyndromic craniosynostosis
Exploratory data from complete genomes of familial alzheimer disease age-at-onset outliers
CNVD: Text mining-based copy number variation in disease database
Assessment of the potential pathogenicity of missense mutations identified in the GTPase-activating protein (GAP)-related domain of the neurofibromatosis type-1 (<i>NF1</i>) gene
Integrated evaluation of CHD7 missense substitutions for CHARGE syndrome clinical genetics
Clinical and genetic findings in a large cohort of patients with ryanodine receptor 1 gene associated myopathies
Tandem repeat polymorphisms as modulators of biological function and dysfunction
In memory of David L. Rimoin, MD, PhD (1936-2012)
Systematic curation and analysis of genomic variations and their potential functional consequences in snoRNA loci
Functional analysis of<i>TCF4</i>missense mutations that cause Pitt-Hopkins syndrome
Use of support vector machines for disease risk prediction in genome-wide association studies: Concerns and opportunities
Review and update of<i>SPRED1</i>mutations causing legius syndrome
Developing the “next generation” of genetic association databases for complex diseases
A guide for functional analysis of<i>BRCA1</i>variants of uncertain significance
A modular approach to disease registry design: Successful adoption of an internet-based rare disease registry
Associations among genotype, clinical phenotype, and intracellular localization of trafficking proteins in ARC syndrome
Spinocerebellar ataxias: An example of the challenges associated with genetic databases for dynamic mutations
ALSoD: A user-friendly online bioinformatics tool for amyotrophic lateral sclerosis genetics
Identification of fifteen novel germline variants in the<i>BRCA1</i>3′UTR reveals a variant in a breast cancer case that introduces a functional<i>miR-103</i>target site
Functional characterization of<i>MLH1</i>missense variants identified in lynch syndrome patients
Oncogenic potential is related to activating effect of cancer single and double somatic mutations in receptor tyrosine kinases
Validation of a quantitative PCR-high-resolution melting protocol for simultaneous screening of<i>COL1A1</i>and<i>COL1A2</i>point mutations and large rearrangements: Application for diagnosis of osteo
Revisiting genotype-phenotype overlap in neurogenetics: Triplet-repeat expansions mimicking spastic paraplegias
Human variome project country nodes: Documenting genetic information within a country
Exome sequencing is an efficient tool for genetic screening of Charcot-Marie-Tooth Disease
Absence of<i>FKBP10</i>in recessive type XI osteogenesis imperfecta leads to diminished collagen cross-linking and reduced collagen deposition in extracellular matrix
The Genome Generation by Elizabeth Finkel
Comprehensive functional assessment of<i>MLH1</i>variants of unknown significance
Microattribution and nanopublication as means to incentivize the placement of human genome variation data into the public domain
Improving the rigor of mutation reports: Biologic parentage and de novo mutations
The<i>KAT6B</i>-related disorders genitopatellar syndrome and Ohdo/SBBYS syndrome have distinct clinical features reflecting distinct molecular mechanisms
Comprehensive molecular analysis demonstrates type V collagen mutations in over 90% of patients with classic EDS and allows to refine diagnostic criteria
The inherited ataxias: Genetic heterogeneity, mutation databases, and future directions in research and clinical diagnostics
Mutations in PLOD2 cause autosomal-recessive connective tissue disorders within the Bruck syndrome-Osteogenesis imperfecta phenotypic spectrum
ABCMdb: A database for the comparative analysis of protein mutations in ABC transporters, and a potential framework for a general application
Molecular characterization of Joubert syndrome in Saudi Arabia
<i>CFTR</i>mutation combinations producing frequent complex alleles with different clinical and functional outcomes
Santorini mutation detection meeting 2011: Rapid advance in sequencing technology poses challenges for interpretation of genetic variations
Congenital myasthenic syndromes: Achievements and limitations of phenotype-guided gene-after-gene sequencing in diagnostic practice: A study of 680 patients
Influence of genetic variation on alternate<i>MUTYH</i>transcript isoforms
How real are our data? Copy number variation in lymphoblastoid and other cell lines
The mechanism of BH4-responsive hyperphenylalaninemia-As it occurs in the ENU1/2 genetic mouse model
Update of the pompe disease mutation database with 60 novel GAA sequence variants and additional studies on the functional effect of 34 previously reported variants
Analysis of the regulatory and catalytic domains of PTEN-induced kinase-1 (PINK1)
Conserved and quickly evolving immunome genes have different evolutionary paths
PRRT2 Mutations are the major cause of benign familial infantile seizures
Solving bottlenecks in data sharing in the life sciences
Gaucher disease paradigm: From ERAD to comorbidity
Ribosome readthrough accounts for secreted full-length factor IX in hemophilia B patients with nonsense mutations
Identification of novel rare mutations of DACT1 in human neural tube defects
Autosomal recessive lethal congenital contractural syndrome type 4 (LCCS4) caused by a mutation in MYBPC1
Inactive matriptase-2 mutants found in IRIDA patients still repress hepcidin in a transfection assay despite having lost their serine protease activity
Paralogous annotation of disease-causing variants in long QT syndrome genes
Locus‐specific mutation databases for neurodegenerative brain diseases
Mismatch repair analysis of inherited MSH2 and/or MSH6 variation pairs found in cancer patients
Toward a mtDNA locus-specific mutation database using the LOVD platform
Ontological phenotype standards for neurogenetics
Phenotype-optimized sequence ensembles substantially improve prediction of disease-causing mutation in cystic fibrosis
Dominant-negative STAT1 SH2 domain mutations in unrelated patients with mendelian susceptibility to mycobacterial disease
Tandem repeat sequence variation as causative Cis-eQTLs for protein-coding gene expression variation: The case of CSTB
The use of arrays to detect copy-number variations in clinical practice
Retinitis pigmentosa: More genes, more variants, more work
Reading through nonsense as therapy for propionic acidemia?
Mitochondrial tRNA mutations are associated with maternally inherited hypertension in two Han Chinese pedigrees
Prostaglandin transporter mutations cause pachydermoperiostosis with myelofibrosis
Functional analysis of nonsynonymous single nucleotide polymorphisms in human SLC26A9
Further delineation of CANT1 phenotypic spectrum and demonstration of its role in proteoglycan synthesis
A novel classification system to predict the pathogenic effects of CHD7 missense variants in CHARGE syndrome
LTBP2 mutations cause Weill-Marchesani and Weill-Marchesani-like syndrome and affect disruptions in the extracellular matrix
Molecular and biochemical characterization of a unique mutation in CCS, the human copper chaperone to superoxide dismutase
SAMHD1 is a nucleic-acid binding protein that is mislocalized due to aicardi-goutières syndrome-associated mutations
Autosomal dominant polycystic kidney disease: Comprehensive mutation analysis of PKD1 and PKD2 in 700 unrelated patients
NGS catalog: A database of next generation sequencing studies in humans
PON-P: Integrated predictor for pathogenicity of missense variants
Differential MicroRNA expression tracks neoplastic progression in inflammatory bowel disease-associated colorectal cancer
Differential effects of AKT1(p.E17K) expression on human mammary luminal epithelial and myoepithelial cells
A review of a multifactorial probability based model for classification of BRCA1 and BRCA2 variants of uncertain significance (VUS)
Guidelines for splicing analysis in molecular diagnosis derived from a set of 327 combined in silico/in vitro studies on BRCA1 and BRCA2 variants
Deep phenotyping for precision medicine
A novel mutation in YARS2 causes myopathy with lactic acidosis and sideroblastic anemia
A novel 13 base pair insertion in the sonic hedgehog ZRS limb enhancer (ZRS/<i>LMBR1</i>) causes preaxial polydactyly with triphalangeal thumb
Splice site, frameshift, and chimeric<i>GFAP</i>mutations in Alexander disease
SURF1-associated leigh syndrome: A case series and novel mutations
Systematic analysis and functional annotation of variations in the genome of an Indian individual
Mutation update on the CHD7 gene involved in CHARGE syndrome
Inherited deleterious variants in<i>GALNT12</i>are associated with CRC susceptibility
11q13 is a susceptibility locus for hormone receptor positive breast cancer
More single-nucleotide mutations surround small insertions than small deletions in primates
Targeted exome sequencing in clear cell renal cell carcinoma tumors suggests aberrant chromatin regulation as a crucial step in ccRCC development
Insertion of an SVA element, a nonautonomous retrotransposon, in<i>PMS2</i>intron 7 as a novel cause of lynch syndrome
Correlation assessment among clinical phenotypes, expression analysis and molecular modeling of 14 novel variations in the human galactose-1-phosphate uridylyltransferase gene
Frameshift mutation in p53 regulator<i>RPL26</i>is associated with multiple physical abnormalities and a specific pre-ribosomal RNA processing defect in diamond-blackfan anemia
Deep intronic<i>APC</i>mutations explain a substantial proportion of patients with familial or early-onset adenomatous polyposis
Regulatory variations in the era of next-generation sequencing: Implications for clinical molecular diagnostics
Back to the future: From genome to metabolome
Genome-wide arrays: Quality criteria and platforms to be used in routine diagnostics
Observ-OM and Observ-TAB: Universal syntax solutions for the integration, search, and exchange of phenotype and genotype information
Identification of a truncation mutation of acylglycerol kinase (AGK) gene in a novel autosomal recessive cataract locus
Rett networked database: An integrated clinical and genetic network of rett syndrome databases
Using PhenX measures to identify opportunities for cross-study analysis
Deep sequencing of the<i>LRRK2</i>gene in 14,002 individuals reveals evidence of purifying selection and independent origin of the p.Arg1628Pro mutation in Europe
Integration of global resources for human genetic variation and disease
Representation of rare diseases in health information systems: The orphanet approach to serve a wide range of end users
Mouse genetic and phenotypic resources for human genetics
Actin out with filamin: Two sides of the story
KMD: A country-specific genetic variation resource for Korea
Exome and whole-genome sequencing for gene discovery: The future is now!
Knowledge engineering for health: A new discipline required to bridge the “ICT gap” between research and healthcare
Arrays in postnatal and prenatal diagnosis: An exploration of the ethics of consent
Large-scale objective association of mouse phenotypes with human symptoms through structural variation identified in patients with developmental disorders
ApiNATOMY: A novel toolkit for visualizing multiscale anatomy schematics with phenotype-related information
Mutation spectrum in the large GTPase dynamin 2, and genotype-phenotype correlation in autosomal dominant centronuclear myopathy
Chromosomal variation in lymphoblastoid cell lines
Clinical and genetic findings in a large cohort of patients with ryanodine receptor 1 gene-associated myopathies
Ribo-polymerase chain reaction-A facile method for the preparation of chimeric RNA/DNA applied to DNA sequencing
Genome-wide arrays in routine diagnostics of hematological malignancies
Novel mutations in the KCND3-encoded Kv4.3 K+ channel associated with autopsy-negative sudden unexplained death
<i>MUTYH</i>gene expression and alternative splicing in controls and polyposis patients
A diagnostic genetic test for the physical mapping of germline rearrangements in the susceptibility breast cancer genes BRCA1 and BRCA2
Mutations in the prostaglandin transporter encoding gene<i>SLCO2A1</i>Cause primary hypertrophic osteoarthropathy and isolated digital clubbing
The introduction of arrays in prenatal diagnosis: A special challenge
Standard terminology for phenotypic variations: The Elements of Morphology project, its current progress, and future directions
An AT-rich region in the APC gene may cause misinterpretation of familial adenomatous polyposis molecular screening
Microarray-based copy number analysis of neurofibromatosis type-1 (NF1)-associated malignant peripheral nerve sheath tumors reveals a role for Rho-GTPase pathway genes in NF1 tumorigenesis
Next-generation sequencing demands next-generation phenotyping
MouseFinder: Candidate disease genes from mouse phenotype data
Phenotypic information in genomic variant databases enhances clinical care and research: The international standards for cytogenomic arrays consortium experience
Large-scale objective phenotyping of 3D facial morphology
Looks normal, but may not function properly: A “new” mouse model for primary ciliary dyskinesia
Olfactory cells aid in the understanding of neurodegenerative disorders
Feasibility of nonsense mutation readthrough as a novel therapeutical approach in propionic acidemia
Diagnostic interpretation of array data using public databases and internet sources
Next‐generation genetic testing for retinitis pigmentosa
The androgen receptor gene mutations database: 2012 update
KMD: Korean mutation database for genes related to diseases
An analysis of exome sequencing for diagnostic testing of the genes associated with muscle disease and spastic paraplegia
Analysis of DNA sequence variants detected by high-throughput sequencing
Classification of mismatch repair gene missense variants with PON-MMR
Confirmation of association of<i>FCGR3B</i>but not<i>FCGR3A</i>copy number with susceptibility to autoantibody positive rheumatoid arthritis
Haploinsufficiency of<i>SOX5</i>at 12p12.1 is associated with developmental delays with prominent language delay, behavior problems, and mild dysmorphic features
Functional characterization of protein variants of the human multidrug transporter ABCC2 by a novel targeted expression system in fibrosarcoma cells
VAR-MD: A tool to analyze whole exome-genome variants in small human pedigrees with mendelian inheritance
Association of glycosylated hemoglobin with the gene encoding CDKAL1 in the Korean Association Resource (KARE) study
Assessing the Enrichment Performance in Targeted Resequencing Experiments
Detecting false-positive signals in exome sequencing
Maps for the world of genomic medicine: The 2011 CSHL Personal Genomes meeting
Ovarian cancer susceptibility alleles and risk of ovarian cancer in<i>BRCA1</i>and<i>BRCA2</i>mutation carriers
Transcriptional hallmarks of noonan syndrome and noonan‐like syndrome with loose anagen hair
Nonsynonymous variants in the<i>SMAD6</i>gene predispose to congenital cardiovascular malformation
<i>SEPT12</i>mutations cause male infertility with defective sperm annulus
A p.D116G mutation in CREB1 leads to novel multiple malformation syndrome resembling<i>CrebA</i>knockout mouse
<i>PCDH19</i>-related infantile epileptic encephalopathy: An unusual X-linked inheritance disorder
Common <i>CFTR</i> haplotypes and susceptibility to chronic pancreatitis and congenital bilateral absence of the vas deferens
Planar cell polarity gene mutations in severe neural tube defects
Differential MicroRNA expression tracks neoplastic progression in inflammatory bowel disease-associated colorectal cancer
Rare germline mutations in<i>PALB2</i>and breast cancer risk: A population-based study
Novel mitochondrial DNA mutations responsible for maternally inherited nonsyndromic hearing loss
Presence of ATM protein and residual kinase activity correlates with the phenotype in ataxia-telangiectasia: A genotype-phenotype study
Update of PAX2 mutations in renal coloboma syndrome and establishment of a locus-specific database
Rapid, comprehensive analysis of the dystrophin transcript by a custom micro-fluidic exome array
Improved diagnostics lead to identification of three new patients with congenital disorder of glycosylation-Ip
Exome and genome analysis as a tool for disease identification and treatment: The 2011 human genome variation society scientific meeting
Genome-wide and gene-based association implicates FRMD6 in alzheimer disease
Genome-wide analysis of familial dysautonomia and kinetin target genes with patient olfactory ecto-mesenchymal stem cells
Disease-associated mutations in the actin-binding domain of filamin B cause cytoplasmic focal accumulations correlating with disease severity
Tissue-specific differences in the proportion of mosaic large NF1 deletions are suggestive of a selective growth advantage of hematopoietic del(+/−) stem cells
A frequent somatic mutation in CD274 3′-UTR leads to protein over-expression in gastric cancer by disrupting miR-570 binding
HGV2011: Personalized genomic medicine meets the incidentalome
Identification of 83 novel alpha-mannosidosis-associated sequence variants: Functional analysis of MAN2B1 missense mutations
Functional assessment of TSC1 missense variants identified in individuals with tuberous sclerosis complex
Non-USH2A mutations in USH2 patients
Identification and functional characterization of two novel mutations in the α-helical loop (residues 484-503) of CYBB/gp91phox resulting in the rare X91+ variant of chronic granulomatous disease