Human Mutation - 2008

226 articles | Last updated: 2025-12-03 14:12:57
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Genomic convergence to identify candidate genes for Alzheimer Disease on chromosome 10
Fertility defects revealing germline biallelic nonsense<i>NBN</i>mutations
How much mutant protein is needed to cause a protein aggregate myopathy in vivo? Lessons from an exceptional desminopathy
Classic and atypical fibrodysplasia ossificans progressiva (FOP) phenotypes are caused by mutations in the bone morphogenetic protein (BMP) type I receptor ACVR1
<i>ATP13A2</i>variability in Parkinson disease
Genotype-phenotype relationships in trichothiodystrophy patients with novel splicing mutations in the<i>XPD</i>gene
Mechanistic insights into the link between a polymorphism of the 3′UTR of the<i>SLC7A1</i>gene and hypertension
Functional characterization of three<i>CYP21A2</i>sequence variants (p.A265V, p.W302S, p.D322G) employing a yeast co-expression system
Simultaneous mutation and copy number variation (CNV) detection by multiplex PCR-based GS-FLX sequencing
Diagnostic yield, interpretation, and clinical utility of mutation screening of sarcomere encoding genes in Danish hypertrophic cardiomyopathy patients and relatives
A<sub>1</sub>ATVar: a relational database of human<i>SERPINA1</i>gene variants leading to α<sub>1</sub>-antitrypsin deficiency and application of the VariVis software
Genomic deletions of<i>OFD1</i>account for 23% of oral-facial-digital type 1 syndrome after negative DNA sequencing
Somatic mutation databases as tools for molecular epidemiology and molecular pathology of cancer: Proposed guidelines for improving data collection, distribution, and integration
Mutations and polymorphisms in the human argininosuccinate synthetase (ASS1) gene
Comprehensive clinical and molecular assessment of 32 probands with congenital contractural arachnodactyly: Report of 14 novel mutations and review of the literature
Expanded mutational spectrum in Cohen syndrome, tissue expression, and transcript variants of<i>COH1</i>
COL5A1 signal peptide mutations interfere with protein secretion and cause classic Ehlers-Danlos syndrome
Localization studies of rare missense mutations in cystic fibrosis transmembrane conductance regulator (CFTR) facilitate interpretation of genotype-phenotype relationships
Sequence variant classification and reporting: recommendations for improving the interpretation of cancer susceptibility genetic test results
Locus-specific databases and recommendations to strengthen their contribution to the classification of variants in cancer susceptibility genes
In silico analysis of missense substitutions using sequence-alignment based methods
Tumor characteristics as an analytic tool for classifying genetic variants of uncertain clinical significance
Classification of rare missense substitutions, using risk surfaces, with genetic- and molecular-epidemiology applications
Genetic evidence and integration of various data sources for classifying uncertain variants into a single model
Prediction and assessment of splicing alterations: implications for clinical testing
Mechanisms of pathogenicity in human<i>MSH2</i>missense mutants
Assessment of functional effects of unclassified genetic variants
Assessing pathogenicity: overview of results from the IARC Unclassified Genetic Variants Working Group
A database to support the interpretation of human mismatch repair gene variants
Analysis of the<i>DYSF</i>mutational spectrum in a large cohort of patients
Analysis of mutant DNA polymerase γ in patients with mitochondrial DNA depletion
Exaggerated status of “novel” and “pathogenic” mtDNA sequence variants due to inadequate database searches
Pleiotropic functional properties of androgen receptor mutants in prostate cancer
In memoriam: Victor A. McKusick (1921-2008)
A new database for ribosomal protein genes which are mutated in Diamond-Blackfan Anemia
Mutations in the small heterodimer partner gene increase morbidity risk in Japanese type 2 diabetes patients
Identification of 23<i>TGFBR2</i>and 6<i>TGFBR1</i>gene mutations and genotype-phenotype investigations in 457 patients with Marfan syndrome type I and II, Loeys-Dietz syndrome and related disorders
<i>TCF4</i>Deletions in Pitt-Hopkins Syndrome
Novel mutations of the suppressor gene<i>PTEN</i>in colorectal carcinomas stratified by microsatellite instability- and<i>TP53</i>mutation- status
Novel heterozygous<i>OTX2</i>mutations and whole gene deletions in anophthalmia, microphthalmia and coloboma
<i>MSH2</i>missense mutations and HNPCC syndrome: pathogenicity assessment in a human expression system
Update of mutations in the genes encoding the pancreatic beta-cell K<sub>ATP</sub>channel subunits Kir6.2 (<i>KCNJ11</i>) and sulfonylurea receptor 1 (<i>ABCC8</i>) in diabetes mellitus and hyperinsul
The<i>FBN2</i>gene: new mutations, locus-specific database (Universal Mutation Database<i>FBN2</i>), and genotype-phenotype correlations
Ethnically diverse causes of Walker-Warburg syndrome (WWS):<i>FCMD</i>mutations are a more common cause of WWS outside of the Middle East
Moving Microarrays Into Clinical Care
Molecular diagnosis of Duchenne/Becker muscular dystrophy: enhanced detection of dystrophin gene rearrangements by oligonucleotide array-comparative genomic hybridization
Molecular analysis of<i>ARSA</i>and<i>PSAP</i>genes in twenty-one Italian patients with metachromatic leukodystrophy: identification and functional characterization of 11 novel<i>ARSA</i>alleles
Variants of the<i>MATP</i>/<i>SLC45A2</i>gene are protective for melanoma in the French population
Detection of exonic copy-number changes using a highly efficient oligonucleotide-based comparative genomic hybridization-array method
Microarray-based mutation detection in the<i>dystrophin</i>gene
A novel 154-bp deletion in the human mitochondrial DNA control region in healthy individuals
Identification of 34 novel and 56 known<i>FOXL2</i>mutations in patients with blepharophimosis syndrome
Automated splicing mutation analysis by information theory
Genetic variants and haplotypes of the<i>caspase-8</i>and<i>caspase-10</i>genes contribute to susceptibility to cutaneous melanoma
<i>SLC45A2</i>: a novel malignant melanoma-associated gene
Enhanced ectodysplasin-A receptor (EDAR) signaling alters multiple fiber characteristics to produce the East Asian hair form East Asian
A large fraction of unclassified variants of the mismatch repair genes<i>MLH1</i>and<i>MSH2</i>is associated with splicing defects
<i>CRTAP</i>and<i>LEPRE1</i>mutations in recessive osteogenesis imperfecta
Functional analysis of an ADAMTS10 signal peptide mutation in Weill-Marchesani syndrome demonstrates a long-range effect on secretion of the full-length enzyme
Somatic mosaicism for copy number variation in differentiated human tissues
Molecular genetics of tetrahydrobiopterin-responsive phenylalanine hydroxylase deficiency
Molecular and clinical genetics of mitochondrial diseases due to<i>POLG</i>mutations
Nature and mRNA effect of 282 different<i>NF1</i>point mutations: focus on splicing alterations
Mutations in<i>TREM2</i>lead to pure early-onset dementia without bone cysts
Granulin mutations associated with frontotemporal lobar degeneration and related disorders: An update
Mutational spectrum of the oral-facial-digital type I syndrome: a study on a large collection of patients
Molecular epidemiology of chronic granulomatous disease in a series of 80 kindreds: identification of 31 novel mutations
Successful amplification of degraded DNA for use with high-throughput SNP genotyping platforms
Mutations in CNGA3 impair trafficking or function of cone cyclic nucleotide-gated channels, resulting in achromatopsia
A comprehensive analysis of normal variation and disease-causing mutations in the human<i>DSPP</i>gene
Faster online publication and indexing of “Mutation in Brief” articles via Early View
Differential functional effects of novel mutations of the transcription factor FOXL2 in BPES patients
Thailand mutation and variation database (ThaiMUT)
UMD-USHbases: a comprehensive set of databases to record and analyse pathogenic mutations and unclassified variants in seven Usher syndrome causing genes
Does proximal myotonic myopathy show anticipation?
A new pathologic mitochondrial DNA mutation in the cytochrome oxidase subunit I (MT-CO1)
Mutational spectrum of<i>FAM83H</i>: the C-terminal portion is required for tooth enamel calcification
Red hair is the null phenotype of MC1R
Germline and somatic<i>NF1</i>gene mutations in plexiform neurofibromas
Further evidence for allelic heterogeneity in Hartnup disorder
Genotype-phenotype correlations in MYCN-related Feingold syndrome
High-resolution mapping of the 8p23.1 beta-defensin cluster reveals strictly concordant copy number variation of all genes
Dynamic nature of the proximal<i>AZFc</i>region of the human Y chromosome: multiple independent deletion and duplication events revealed by microsatellite analysis
SALL1 truncated protein expression in Townes-Brocks syndrome leads to ectopic expression of downstream genes
Persistence of repair proteins at unrepaired DNA damage distinguishes diseases with<i>ERCC2</i>(<i>XPD</i>) mutations: cancer-prone xeroderma pigmentosum vs. non-cancer-prone trichothiodystrophy
Dry-reagent disposable biosensor for visual genotyping of single nucleotide polymorphisms by oligonucleotide ligation reaction: application to pharmacogenetic analysis
Mutations in CYP1B1 cause primary congenital glaucoma by reduction of either activity or abundance of the enzyme
The RAS/MAPK syndromes: novel roles of the RAS pathway in human genetic disorders
Genome-wide copy number analysis using copy number inferring tool (CNIT) and DNA pooling
Genetic variation in human aquaporins and effects on phenotypes of water homeostasis
Evaluation and use of a synthetic quality control material, included in the European external quality assessment scheme for cystic fibrosis
<i>PLP1</i>splicing abnormalities identified in Pelizaeus-Merzbacher disease and SPG2 fibroblasts are associated with different types of mutations
An unusual haplotype structure on human chromosome 8p23 derived from the inversion polymorphism
Complex signatures of locus-specific selective pressures and gene conversion on Human Growth Hormone/Chorionic Somatomammotropin genes
Rapid detection of methylation change at H19 in human imprinting disorders using methylation-sensitive high-resolution melting
Investigation of citrullinemia type I variants by in vitro expression studies
Mutations and polymorphisms of the gene of the major human blood protein, serum albumin
Clinical features of maternal uniparental disomy 14 in patients with an epimutation and a deletion of the imprinted<i>DLK1/GTL2</i>gene cluster
A meta-analysis of nonsense mutations causing human genetic disease
Long interspersed nuclear element-1 (LINE1)-mediated deletion ofEVC,EVC2,C4orf6, andSTK32B in Ellis–van Creveld syndrome with borderline intelligence
Cystathionine β-synthase p.S466L mutation causes hyperhomocysteinemia in mice
Parkes Weber syndrome, vein of Galen aneurysmal malformation, and other fast-flow vascular anomalies are caused byRASA1 mutations
Spectrum of<i>HLXB9</i>gene mutations in Currarino syndrome and genotype-phenotype correlation
Evaluation of in silico splice tools for decision-making in molecular diagnosis
Mutations in the glucose-6-phosphatase-α (G6PC) gene that cause type Ia glycogen storage disease
Mutations in human monoamine-related neurotransmitter pathway genes
MLPA screening in the<i>BRCA1</i>gene from 1,506 German hereditary breast cancer cases: novel deletions, frequent involvement of exon 17, and occurrence in single early-onset cases
Mutation profile of theCDH23 gene in 56 probands with Usher syndrome type I
Reduced half-life of holocarboxylase synthetase from patients with severe Multiple Carboxylase Deficiency
Haplotype analysis suggests a single Balkan origin for the Gaucher disease [D409H;H255Q] double mutant allele
Molecular and functional characterization of eight novel GAA mutations in Italian infants with Pompe disease
<i>PROS1</i>analysis in 87 pedigrees with hereditary protein S deficiency demonstrates striking genotype-phenotype associations
Clinical, cellular, and neuropathological consequences of<i>AP1S2</i>mutations: further delineation of a recognizable X-linked mental retardation syndrome
RPS19 mutations in patients with Diamond-Blackfan anemia
Mutations in GDF6 are associated with vertebral segmentation defects in Klippel-Feil syndrome
Update of the Pompe disease mutation database with 107 sequence variants and a format for severity rating
Sunlight ultraviolet irradiation and<i>BRAF</i>V600 mutagenesis in human melanoma
Genetic polymorphisms for the study of multifactorial stroke
Bottlenecks in molecular testing for rare genetic diseases
Transcriptional expression of<i>cis</i>-acting and<i>trans</i>-acting splicing mutations cause autosomal dominant retinitis pigmentosa
A novel mutation in NFKBIA/IKBA results in a degradation-resistant N-truncated protein and is associated with ectodermal dysplasia with immunodeficiency
SORL1 is genetically associated with increased risk for late-onset Alzheimer disease in the Belgian population
Homozygous mutation of the PHOX2B gene in congenital central hypoventilation syndrome (Ondine's Curse)
A novel mutation in AlphaA-crystallin (CRYAA) caused autosomal dominant congenital cataract in a large Chinese family
The Mutation p.Ser298Pro in the sulphamidase gene (SGSH) is associated with a slowly progressive clinical phenotype in mucopolysaccharidosis type IIIA (Sanfilippo A Syndrome)
Genetic variability in the mitochondrial serine protease<i>HTRA2</i>contributes to risk for Parkinson disease
The infevers autoinflammatory mutation online registry: update with new genes and functions
Mutations in the chromatin-associated protein ATRX
A DNA resequencing array for pathogenic mutation detection in hypertrophic cardiomyopathy
Mutations, structural variations, and genome-wide resequencing: where to from here in our understanding of disease and evolution?
Frequent BRG1/SMARCA4-inactivating mutations in human lung cancer cell lines
A multicenter study on the prevalence and spectrum of mutations in the otoferlin gene (<i>OTOF</i>) in subjects with nonsyndromic hearing impairment and auditory neuropathy
Accurate classification of<i>MLH1/MSH2</i>missense variants with multivariate analysis of protein polymorphisms-mismatch repair (MAPP-MMR)
Cells expressing partially unfolded R789C/p.R989C type II procollagen mutant associated with spondyloepiphyseal dysplasia undergo apoptosis
The human retinitis pigmentosa GTPase regulator gene variant database
Novel and established CYP2A6 alleles impair in vivo nicotine metabolism in a population of Black African descent
MID1 mutations in patients with X-linked Opitz G/BBB syndrome
Exon skipping mutations in collagen VI are common and are predictive for severity and inheritance
K45R variant of squalene synthase increases total cholesterol levels in two study samples from a French Canadian population
Alterations of the IKBKG locus and diseases: an update and a report of 13 novel mutations
High-throughput amplicon scanning of the TP53 gene in breast cancer using high-resolution fluorescent melting curve analyses and automatic mutation calling
Large scale DNA sequencing: new challenges emerge-the 2007 Human Genome Variation Society scientific meeting
Integrated DNA, cDNA, and protein studies in Becker muscular dystrophy show high exception to the reading frame rule
Gaucher disease: mutation and polymorphism spectrum in the glucocerebrosidase gene (GBA)
<i>SIX1</i>mutation screening in 247 branchio-oto-renal syndrome families: a recurrent missense mutation associated with BOR
<i>PINK1</i>heterozygous rare variants: prevalence, significance and phenotypic spectrum
PharmGKB and the International Warfarin Pharmacogenetics Consortium: the changing role for pharmacogenomic databases and single-drug pharmacogenetics
Human genome variation and pharmacogenetics
Detection and precise mapping of germline rearrangements in<i>BRCA1, BRCA2, MSH2</i>, and<i>MLH1</i>using zoom-in array comparative genomic hybridization (aCGH)
Discovery of genetic profiles impacting response to chemotherapy: application to gemcitabine
Integration of hepatitis B virus DNA into the myeloid/lymphoid or mixed-lineage leukemia (MLL4) gene and rearrangements of MLL4 in human hepatocellular carcinoma
Validation of the performance of a comprehensive genotyping assay panel of single nucleotide polymorphisms in drug metabolism enzyme genes
Thermal instability of compound variants of carnitine palmitoyltransferase II and impaired mitochondrial fuel utilization in influenza-associated encephalopathy
N-terminal CFTR missense variants severely affect the behavior of the CFTR chloride channel
Lafora disease in the Indian population:EPM2A andNHLRC1 gene mutations and their impact on subcellular localization of laforin and malin Indian population
Novel CCM1, CCM2, and CCM3 mutations in patients with cerebral cavernous malformations: in-frame deletion in CCM2 prevents formation of a CCM1/CCM2/CCM3 protein complex
6-mercaptopurine and 9-(2-phosphonyl-methoxyethyl) adenine (PMEA) transport altered by two missense mutations in the drug transporter gene ABCC4
A panel of ancestry informative markers for estimating individual biogeographical ancestry and admixture from four continents: utility and applications
Frequent mutations in the neurotrophic tyrosine receptor kinase gene family in large cell neuroendocrine carcinoma of the lung
CYBB, an NADPH-oxidase gene: restricted diversity in humans and evidence for differential long-term purifying selection on transmembrane and cytosolic domains
Activating NOTCH3 mutation in a patient with small-vessel-disease of the Brain
A putative exonic splicing enhancer in exon 7 of thePDHA1 gene affects splicing of adjacent exons
RET Gly691Ser mutation is associated with primary vesicoureteral reflux in the French-Canadian population from Quebec French-Canadian population from Quebec
Transcriptional deregulation and a missense mutation define ANKRD1 as a candidate gene for total anomalous pulmonary venous return
Spectrum of USH2A mutations in Scandinavian patients with Usher syndrome type II
Usher syndrome type 1 due to missense mutations on bothCDH23 alleles: investigation of mRNA splicing
Null mutations causing depletion of the type 1 ryanodine receptor (RYR1) are commonly associated with recessive structural congenital myopathies with cores
In vitro functional studies of naturally occurring pathogenic PRKAR1A mutations that are not subject to nonsense mRNA decay
A human phospholamban promoter polymorphism in dilated cardiomyopathy alters transcriptional regulation by glucocorticoids
Missense mutations in<i>POU4F3</i>cause autosomal dominant hearing impairment DFNA15 and affect subcellular localization and DNA binding
Branchio-oto-renal syndrome (BOR): novel mutations in the<i>EYA1</i>gene, and a review of the mutational genetics of BOR
Novel mutations in exon 2 of<i>MATN3</i>affect residues within the α-helices of the A-domain and can result in the intracellular retention of mutant matrilin-3
Clinical and molecular features of mitochondrial DNA depletion due to mutations in deoxyguanosine kinase
Novel mutations of the GLA gene in Japanese patients with Fabry disease and their functional characterization by active site specific chaperone
Sequence variation in the ATP-binding domain of the Wilson disease transporter, ATP7B, affects copper transport in a yeast model system
Myoclonus-dystonia: significance of large<i>SGCE</i>deletions
A 12-bp deletion in the 5'-flanking region of the<i>SERPINH1</i>gene affects promoter activity and protects against preterm premature rupture of membranes in African Americans African Americans
The<i>MLH1</i>variants p.Arg265Cys and p.Lys618Ala affect protein stability while p.Leu749Gln affects heterodimer formation
Comprehensive analysis of<i>LRRK2</i>in publicly available Parkinson's disease cases and neurologically normal controls
A clinical, genetic, and biochemical characterization of<i>SPG7</i>mutations in a large cohort of patients with hereditary spastic paraplegia
Sedoheptulokinase deficiency due to a 57-kb deletion in cystinosis patients causes urinary accumulation of sedoheptulose: elucidation of the<i>CARKL</i>gene
Rapid identification of somatic mutations in colorectal and breast cancer tissues using mismatch repair detection (MRD)
Molecular characterization of novel progranulin (<i>GRN</i>) mutations in frontotemporal dementia
Mutation spectrum of<i>MYO7A</i>and evaluation of a novel nonsyndromic deafness<i>DFNB2</i>allele with residual function
Patterns of missplicing caused by<i>RB1</i>gene mutations in patients with retinoblastoma and association with phenotypic expression
Construction of a multiplex allele-specific PCR-based universal array (ASPUA) and its application to hearing loss screening
Somatic<i>FGF9</i>mutations in colorectal and endometrial carcinomas associated with membranous<b>β</b>-catenin
Polymorphisms of<i>LIG4</i>and<i>XRCC4</i>involved in the NHEJ pathway interact to modify risk of glioma
Annotating single amino acid polymorphisms in the UniProt/Swiss-Prot knowledgebase
Platelet receptor P2RY12 haplotypes predict restenosis after percutaneous coronary interventions
Progranulin locus deletion in frontotemporal dementia
Syndromic true hermaphroditism due to an R-spondin1 (<i>RSPO1</i>) homozygous mutation
NPC-db, a Niemann-Pick type C disease gene variation database
Mutation analysis in nephronophthisis using a combined approach of homozygosity mapping, CEL I endonuclease cleavage, and direct sequencing
Increased forensic efficiency of DNA fingerprints through simultaneous resolution of length and nucleotide variability by high-performance mass spectrometry
Evidence of a four-hit mechanism involving<i>SMARCB1</i>and<i>NF2</i>in schwannomatosis-associated schwannomas
Position of nonmuscle myosin heavy chain IIA (NMMHC-IIA) mutations predicts the natural history ofMYH9-related disease
CoagMDB: a database analysis of missense mutations within four conserved domains in five vitamin K-dependent coagulation serine proteases using a text-mining tool
Profiling of copy number variations (CNVs) in healthy individuals from three ethnic groups using a human genome 32 K BAC-clone-based array
Classification of ambiguous mutations in DNA mismatch repair genes identified in a population-based study of colorectal cancer
Detailed analysis of 22q11.2 with a high density MLPA probe set
The Human Intermediate Filament Database: comprehensive information on a gene family involved in many human diseases
<i>PIK3CA</i>cancer mutations display gender and tissue specificity patterns
RNA-based mutation analysis identifies an unusual MSH6 splicing defect and circumvents PMS2 pseudogene interference
A frequent functional SNP in the<i>MMP1</i>promoter is associated with higher disease severity in recessive dystrophic epidermolysis bullosa
Primary ciliary dyskinesia associated with normal axoneme ultrastructure is caused by<i>DNAH11</i>mutations
Detection of unrecognized low-level mtDNA heteroplasmy may explain the variable phenotypic expressivity of apparently homoplasmic mtDNA mutations
Expression of the muscle glycogen phosphorylase gene in patients with McArdle disease: the role of nonsense-mediated mRNA decay
Genomewide SNP assay reveals mutations underlying Parkinson disease
Mutations that impair interaction properties of TRIM32 associated with limb-girdle muscular dystrophy 2H
Clinical, molecular, and protein correlations in a large sample of genetically diagnosed Italian limb girdle muscular dystrophy patients
Allelotyping by massively parallel pyrosequencing of SNP-carrying trinucleotide threads
An XML-based interchange format for genotype-phenotype data
Mutation analysis in Costello syndrome: functional and structural characterization of the<i>HRAS</i>p.Lys117Arg mutation
<i>Myocilin</i>allele-specific glaucoma phenotype database
A new locus-specific database (LSDB) for mutations in the<i>TGFBR2</i>gene: UMD-<i>TGFBR2</i>
Molecular genetics of tetrahydrobiopterin-responsive phenylalanine hydroxylase deficiency
Arterial tortuosity syndrome: clinical and molecular findings in 12 newly identified families
Multiple endocrine neoplasia type 1 (MEN1): analysis of 1336 mutations reported in the first decade following identification of the gene
Multiplex PCR-based real-time invader assay (mPCR-RETINA): a novel SNP-based method for detecting allelic asymmetries within copy number variation regions
Array-MLPA: comprehensive detection of deletions and duplications and its application to DMD patients
Missense and nonsense mutations in the alternatively-spliced exon 2 of<i>COL2A1</i>cause the ocular variant of Stickler syndrome
Identification of the first germline mutation in the extracellular domain of the follitropin receptor responsible for spontaneous ovarian hyperstimulation syndrome
Comprehensive cDNA study and quantitative transcript analysis of mutant<i>OPA1</i>transcripts containing premature termination codons
Mutations of the<i>CEP290</i>gene encoding a centrosomal protein cause Meckel-Gruber syndrome
Does the nonsense-mediated mRNA decay mechanism prevent the synthesis of truncated BRCA1, CHK2, and p53 proteins?