Human Mutation - 2006

220 articles | Last updated: 2025-12-03 14:12:57
Caucasian
2
White
0
European
1
Other
10
Article Title Cauc. White Euro. Other Phrases Used
T A T A T A T A
NovelFGFR1 sequence variants in Kallmann syndrome, and genetic evidence that the FGFR1c isoform is required in olfactory bulb and palate morphogenesis
Whole gene deletion and splicing mutations expand thePINK1 genotypic spectrum
Sub-populations within the major European and African derived haplogroups R1b3 and E3a are differentiated by previously phylogenetically undefined Y-SNPs European derived; African derived
Microsatellite in the 3′ untranslated region of human fibroblast growth factor 9 (FGF9) gene exhibits pleiotropic effect on modulating FGF9 protein expression
Biochemical and molecular characterization of 18 patients with pyridoxine-dependent epilepsy and mutations of the antiquitin (ALDH7A1) gene
Long-range PCR facilitates the identification of<i>PMS2</i>-specific mutations
A new genetic isolate with a unique phenotype of syndromic oculocutaneous albinism: clinical, molecular, and cellular characteristics
High efficiency of mutation detection in type 1 Stickler syndrome using a two-stage approach: vitreoretinal assessment coupled with exon sequencing for screening COL2A1
Identification of novel mutations in<i>PEX2</i>,<i>PEX6</i>,<i>PEX10</i>,<i>PEX12</i>, and<i>PEX13</i>in Zellweger spectrum patients
Deficient membrane integration of the novel p.N14D<i>-GJB2</i>mutant associated with non-syndromic hearing impairment
Determination of the mutation spectrum of the<i>EXT1/EXT2</i>genes in British Caucasian patients with multiple osteochondromas, and exclusion of six candidate genes in<i>EXT</i>negative cases
Erratum: Molecular bases of antithrombin deficiency: twenty-two novel mutations in the antithrombin gene
Recessive arrhythmogenic right ventricular dysplasia due to novel cryptic splice mutation in<i>PKP2</i>
Identification of novel mutations in the human ornithine transcarbamylase (<i>OTC</i>) gene of Korean patients with OTC deficiency and transient expression of the mutant proteins in vitro Korean
Red cell glucose phosphate isomerase (GPI): a molecular study of three novel mutations associated with hereditary nonspherocytic hemolytic anemia
Immunodeficiency mutation databases (IDbases)
Bioinformatic analysis of protein structure–function relationships: case study of leukocyte elastase (ELA2) missense mutations
Chronic granulomatous disease caused by mutations other than the common GT deletion in<i>NCF1</i>, the gene encoding the p47<sup>phox</sup>component of the phagocyte NADPH oxidase
Comprehensive survey of mutations in RP2 and RPGR in patients affected with distinct retinal dystrophies: genotype–phenotype correlations and impact on genetic counseling
BTKbase: the mutation database for X-linked agammaglobulinemia
Focus on immunogenetics: diagnosis, molecular genetics, mutations, and diseases
Long contiguous stretches of homozygosity in the human genome
<i>RAG</i>-dependent primary immunodeficiencies
Molecular approaches in the diagnosis of primary immunodeficiency diseases
Population-based estimates of breast cancer risks associated with<i>ATM</i>gene variants c.7271T&gt;G and c.1066-6T&gt;G (IVS10-6T&gt;G) from the Breast Cancer Family Registry
Activation-induced cytidine deaminase: structure–function relationship as based on the study of mutants
Testing and improving experimental parameters for the use of low molecular weight targets in array-CGH experiments
The IMGT/HLA and IPD databases
The 185delAG mutation (c.68_69delAG) in theBRCA1 gene triggers translation reinitiation at a downstream AUG codon
Phenotypic heterogeneity in the XPB DNA helicase gene (<i>ERCC3</i>): xeroderma pigmentosum without and with Cockayne syndrome
Evidence for adaptive selection acting on the tRNA and rRNA genes of human mitochondrial DNA
Interactive visual analysis of SNP data for rapid autozygosity mapping in consanguineous families
Combined use of MLPA and nonfluorescent multiplex PCR analysis by high performance liquid chromatography for the detection of genomic rearrangements
Twenty-one novel mutations in the GLB1 gene identified in a large group of GM1-gangliosidosis and Morquio B patients: possible common origin for the prevalent p.R59H mutation among gypsies gypsies
Low frequency ofAXIN2 mutations and high frequency ofMUTYH mutations in patients with multiple polyposis
MUT-TP53: a versatile matrix for TP53 mutation verification and publication
Unique substitution ofCHEK2 andTP53 mutations implicated in primary prostate tumors and cancer cell lines
The cystathionine beta-synthase variant c.844_845ins68 protects against CNS demyelination in X-linked adrenoleukodystrophy
Deletion hotspot in the argininosuccinate lyase gene: association with topoisomerase II and DNA polymerase α sites
Somatic mutations ofGUCY2F,EPHA3, andNTRK3 in human cancers
A two-tier approach to mutation detection in theCOL4A5 gene for Alport syndrome
ATM mutations in Italian families with ataxia telangiectasia include two distinct large genomic deletions
Identification of the microdeletion breakpoint in aGLRA1null allele of Turkish hyperekplexia patients
Biological effects of four PSEN1 gene mutations causing Alzheimer disease with spastic paraparesis and cotton wool plaques
ComprehensiveNF1 screening on cultured Schwann cells from neurofibromas
A recessive Mendelian model to predict carrier probabilities of DFNB1 for nonsyndromic deafness
PAR1 deletions downstream ofSHOX are the most frequent defect in a Spanish cohort of Léri-Weill dyschondrosteosis (LWD) probands Spanish cohort
Spectrum of<i>HSPG2</i>(Perlecan) mutations in patients with Schwartz-Jampel syndrome
SNPSplicer: systematic analysis of SNP-dependent splicing in genotyped cDNAs
<i>NF1</i>mutation rather than individual genetic variability is the main determinant of the<i>NF1</i>-transcriptional profile of mutations affecting splicing
Common variations in the<i>IL4R</i>gene affect splicing and influence natural expression of the soluble isoform
Structural assessment of single amino acid mutations: application to TP53 function
Expanding the spectrum ofTBX5 mutations in Holt-Oram syndrome: detection of two intragenic deletions by quantitative real time PCR, and report of eight novel point mutations
Three novel thiopurine S-methyltransferase allelic variants (TPMT*20, *21, *22) – association with decreased enzyme function
Mutations in<i>RYR1</i>in malignant hyperthermia and central core disease
Mutation profile of theGAA gene in 40 Italian patients with late onset glycogen storage disease type II Italian
<i>KCNQ4</i>: a gene for age-related hearing impairment?
Genetic evidence for ubiquitin-specific proteases<i>USP24</i>and<i>USP40</i>as candidate genes for late-onset Parkinson disease
Mutations in the BH<sub>4</sub>-metabolizing genes GTP cyclohydrolase I, 6-pyruvoyl-tetrahydropterin synthase, sepiapterin reductase, carbinolamine-4a-dehydratase, and dihydropteridine reductase
Identification of 45 novel mutations in the nebulin gene associated with autosomal recessive nemaline myopathy
CpG methylation accounts for a recurrent mutation (c.1222C&gt;T) in the humanPAH gene
Mutations in the genes encoding the transcription factors hepatocyte nuclear factor 1 alpha (<i>HNF1A</i>) and 4 alpha (<i>HNF4A</i>) in maturity-onset diabetes of the young
Duplications in theDMD gene
Major affective disorders and schizophrenia: a common molecular signature?
Alzheimer dementia caused by a novel mutation located in the APP C-terminal intracytosolic fragment
Population distribution of the functional caspase-12 allele
Visualization ofMAPT inversion on stretched chromosomes of tau-negative frontotemporal dementia patients
An automated procedure to identify biomedical articles that contain cancer-associated gene variants
Acute myelogenous leukemia–derivedSMAD4 mutations target the protein to ubiquitin-proteasome degradation
Microdeletions involving theSCN1A gene may be common inSCN1A-mutation-negative SMEI patients
Human mtDNA site-specific variability values can act as haplogroup markers
Variable pathogenic potentials of mutations located in the desmin alpha-helical domain
Ethnic variation in AMD-associated complement factor H polymorphism p.Tyr402His
National and ethnic mutation databases: recording populations' genography
Phenotypic consequences of branch point substitutions
Paternal bias in parental origin of<i>HRAS</i>mutations in Costello syndrome
Spectrum of factor XI (F11) mutations in the UK population – 116 index cases and 140 mutations
Missense mutation in theN-acetylglucosamine-1-phosphotransferase gene (GNPTA) in a patient with mucolipidosis II induces changes in the size and cellular distribution of GNPTG
Mutational analysis of theABCC6 gene and the proximalABCC6 gene promoter in German patients with pseudoxanthoma elasticum (PXE) German patients
Identification of forty-five novel and twenty-three knownNF1 mutations in Chinese patients with neurofibromatosis type 1 Chinese
Direct or indirect association in a complex disease: the role ofSLC22A4 andSLC22A5 functional variants in Crohn disease
Mutational spectrum and genotype–phenotype correlations in mevalonate kinase deficiency
V(D)J recombinase mediated inter-chromosomalHPRT alterations at cryptic recombination signal sequences in peripheral human T cells
Extensive mutational analysis ofPRKCSH andSEC63 broadens the spectrum of polycystic liver disease
Characterization ofCHEK2 mutations in prostate cancer
Newborn screening for 3-methylcrotonyl-CoA carboxylase deficiency: population heterogeneity ofMCCA andMCCB mutations and impact on risk assessment
Frequency and localization of mutations in the 106 exons of theRYR1 gene in 50 individuals with malignant hyperthermia
Molecular dissection of the Y chromosome haplogroup E-M78 (E3b1a): a posteriori evaluation of a microsatellite-network-based approach through six new biallelic markers
The contribution of genes involved in potassium-recycling in the inner ear to noise-induced hearing loss
Father-to-daughter transmission of Cornelia de Lange syndrome caused by a mutation in the 5′ untranslated region of theNIPBL Gene
<i>TGFBR1</i>and<i>TGFBR2</i>mutations in patients with features of Marfan syndrome and Loeys-Dietz syndrome
Mutations of human cationic trypsinogen (PRSS1) and chronic pancreatitis
Identification and in silico analyses of novelTGFBR1 andTGFBR2 mutations in Marfan syndrome-related disorders
Semiquantitative multiplex PCR: a useful tool for large rearrangement screening and characterization
MLPA: a rapid, reliable, and sensitive method for detection and analysis of abnormalities of 22q
McArdle disease: the mutation spectrum of<i>PYGM</i>in a large Italian cohort
Sub-Saharan African coding sequence variation and haplotype diversity at the<i>NAT2</i>gene
Detection of 95 novel mutations in coagulation factor VIII gene<i>F8</i>responsible for hemophilia A: results from a single institution
A quality assessment survey of SNP genotyping laboratories
Novel and recurrent mutations in keratin<i>KRT5</i>and<i>KRT14</i>genes in epidermolysis bullosa simplex: implications for disease phenotype and keratin filament assembly
In vitro analysis of genomic instability triggered by BRCA1 missense mutations
A new large<i>CFTR</i>rearrangement illustrates the importance of searching for complex alleles
Werner syndrome and mutations of the<i>WRN</i>and<i>LMNA</i>genes in France
Mutational spectrum of maple syrup urine disease in Spain
The heterogeneous nature of germline mutations in NF1 patients with malignant peripheral serve sheath tumours (MPNSTs)
Multiple displacement amplification to create a long-lasting source of DNA for genetic studies
Identification of a novel mutation of<i>SH3BP2</i>in cherubism and demonstration that<i>SH3BP2</i>mutations lead to increased NFAT activation
Mutations and polymorphisms in the human ornithine transcarbamylase (<i>OTC</i>) gene
Mutation analysis of<i>COL1A1</i>and<i>COL1A2</i>in patients diagnosed with osteogenesis imperfecta type I-IV
Genetic variability, haplotypes, and htSNPs for exons 1 at the human<i>UGT1A</i>locus
A common mutation in the<i>CBS</i>gene explains a high incidence of homocystinuria in the Qatari population
Novel mutations in<i>ENG</i>and<i>ACVRL1</i>identified in a series of 200 individuals undergoing clinical genetic testing for hereditary hemorrhagic telangiectasia (HHT): correlation of genotype with
High efficiency of mutation detection in type 1 stickler syndrome using a two-stage approach: vitreoretinal assessment coupled with exon sequencing for screening COL2A1
Mutations in two regions of<i>FLNB</i>result in atelosteogenesis I and III
Mutations in the<i>lipoma HMGIC fusion partner-like 5 (LHFPL5)</i>gene cause autosomal recessive nonsyndromic hearing loss
A homozygous nonsense mutation in the methylmalonyl-CoA epimerase gene (<i>MCEE</i>) results in mild methylmalonic aciduria
Mean age-of-onset of familial alzheimer disease caused by presenilin mutations correlates with both increased Aβ42 and decreased Aβ40
Variation in retinitis pigmentosa-11 (<i>PRPF31</i>or<i>RP11</i>) gene expression between symptomatic and asymptomatic patients with dominant<i>RP11</i>mutations
The Cypriot and Iranian National Mutation Frequency Databases
Hepatic and neuromuscular forms of glycogenosis type III: nine mutations inAGL
Distribution ofENG andACVRL1 (ALK1) mutations in French HHT patients
Mutational spectrum of type I collagen genes in Korean patients with osteogenesis imperfecta Korean
Molecular bases of antithrombin deficiency: twenty-two novel mutations in the antithrombin gene
Determination of genomic copy number with quantitative microsphere hybridization
Novel sequence variants in dysferlin-deficient muscular dystrophy leading to mRNA decay and possible C2-domain misfolding
TGFBI gene mutations in corneal dystrophies
A DGGE system for comprehensive mutation screening of<i>BRCA1</i>and<i>BRCA2</i>: application in a Dutch cancer clinic setting
m.6267G&gt;A: a recurrent mutation in the human mitochondrial DNA that reduces cytochrome c oxidase activity and is associated with tumors
A homozygous<i>ZMPSTE24</i>null mutation in combination with a heterozygous mutation in the<i>LMNA</i>gene causes Hutchinson-Gilford progeria syndrome (HGPS): insights into the pathophysiology of HGPS
Genome-wide identification of pseudogenes capable of disease-causing gene conversion
The spectrum of<i>WRN</i>mutations in Werner syndrome patients
Reduced secretion of fibulin 5 in age-related macular degeneration and cutis laxa
GENOMIZER: an integrated analysis system for genome-wide association data
The<i>ATM</i>missense mutation p.Ser49Cys (c.146C&gt;G) and the risk of breast cancer
Comparisons of PCR-based genome amplification systems using CpG island microarrays
Megalencephalic leukoencephalopathy with subcortical cysts: an update and extended mutation analysis ofMLC1
Pharmacological rescue of carnitine transport in primary carnitine deficiency
The Cathepsin D rs17571 polymorphism: effects on CSF tau concentrations in Alzheimer disease
A subset of patients with epithelial basement membrane corneal dystrophy have mutations inTGFBI/BIGH3
Rapid detection of submicroscopic chromosomal rearrangements in children with multiple congenital anomalies using high density oligonucleotide arrays
Long-range PCR facilitates the identification ofPMS2-specific mutations
Double-strand DNA break repair with replication slippage on two strands: a novel mechanism of deletion formation
Contrasting behaviors of mutant cystathionine beta-synthase enzymes associated with pyridoxine response
The c.43_44insCTG variation inPCSK9 is associated with low plasma LDL-cholesterol in a Caucasian population
Intronic alterations in<i>BRCA1</i>and<i>BRCA2</i>: effect on mRNA splicing fidelity and expression
Optimization and evaluation of single-cell whole-genome multiple displacement amplification
Confirmation of the origin of NISCH syndrome
Jagged1 (<i>JAG1</i>) mutations in Alagille syndrome: increasing the mutation detection rate
Denaturing temperature selection may underestimate keratin mutation detection by DHPLC
The expanding phenotype of<i>POMT1</i>mutations: from Walker-Warburg syndrome to congenital muscular dystrophy, microcephaly, and mental retardation
Genes and loci involved in febrile seizures and related epilepsy syndromes
Interaction between<i>IL1B</i>gene promoter polymorphisms in determining susceptibility to<i>Helicobacter pylori</i>associated duodenal ulcer
Aberrant splicing in the ocular albinism type 1 gene (OA1/GPR143) is corrected in vitro by morpholino antisense oligonucleotides
Mutational data integration in gene-oriented files of the Hermansky-Pudlak Syndrome database
NovelJARID1C/SMCX mutations in patients with X-linked mental retardation
Functional polymorphism inALOX15 results in increased allele-specific transcription in macrophages through binding of the transcription factor SPI1
Trafficking-competent and trafficking-defectiveKCNJ2 mutations in Andersen syndrome
Parental mosaicism can cause recurrent transmission ofSCN1A mutations associated with severe myoclonic epilepsy of infancy
Determination of genomic copy number with quantitative microsphere hybridization
Distinct patterns of germ-line deletions inMLH1 andMSH2: the implication of Alu repetitive element in the genetic etiology of Lynch syndrome (HNPCC)
The distribution of constitutional and somatic mutations in the neurofibromatosis 2 gene
Present status of outcome prediction of invasive coronary treatment by using genetic markers
dbRIP: A highly integrated database of retrotransposon insertion polymorphisms in humans
A second-site mutation in the initiation codon ofWAS (WASP) results in expansion of subsets of lymphocytes in an Wiskott-Aldrich syndrome patient
GermlineLEMD3 mutations are rare in sporadic patients with isolated melorheostosis
Five novel androgen receptor gene mutations associated with complete androgen insensitivity syndrome
Vacuolating megalencephalic leukoencephalopathy with subcortical cysts: functional studies of novel variants inMLC1
Characterization and functional investigation of single nucleotide polymorphisms (SNPs) in the human TLR5 gene
Study of four genes belonging to the folate pathway: transcobalamin 2 is involved in the onset of non-syndromic cleft lip with or without cleft palate
Functional analysis of splicing mutations and of an exon 2 polymorphic variant ofSERPING1/C1NH
Mutation profile of the MYO7A gene in Spanish patients with Usher syndrome type I
Recessive dystrophic epidermolysis bullosa caused by COL7A1 hemizygosity and a missense mutation with complex effects on splicing
De novo gene conversion in the RCA gene cluster (1q32) causes mutations in complement factor H associated with atypical hemolytic uremic syndrome
A novel mutation in theGATA4 gene in patients with Tetralogy of Fallot
Functional splicing assay shows a pathogenic intronic mutation in neurofibromatosis type 1 (NF1) due to intronic sequence exonization
Mutation study of Spanish patients with hereditary hemorrhagic telangiectasia and expression analysis of Endoglin and ALK1
MPL mutations in 23 patients suffering from congenital amegakaryocytic thrombocytopenia: the type of mutation predicts the course of the disease
High prevalence ofCBS p.T191M mutation in homocystinuric patients from Colombia
Molecular diversity of Glanzmann thrombasthenia in southern India: new insights into mRNA splicing and structure-function correlations of<b>α</b>IIb<b>β</b>3 integrin (<i>ITGA2B, ITGB3</i>)
Mechanisms of the interaction between twoADAMTS13 gene mutations leading to severe deficiency of enzymatic activity
Apolipoprotein (APOE) gene is associated with progression of age-related macular degeneration (AMD)
DLG5 variants contribute to Crohn disease risk in a Canadian population
Comprehensive mutation analysis of<i>GLDC</i>,<i>AMT</i>, and<i>GCSH</i>in nonketotic hyperglycinemia
Mutations inEDAR account for one-quarter of non-ED1-related hypohidrotic ectodermal dysplasia
Nonsynonymous SNPs: validation characteristics, derived allele frequency patterns, and suggestive evidence for natural selection
Singapore Human Mutation/Polymorphism Database: a country-specific database for mutations and polymorphisms in inherited disorders and candidate gene association studies
DRD4 gene variant associated with body mass: The National Longitudinal Study of Adolescent Health
Paternal uniparental isodisomy of the entire chromosome 3 revealed in a person with no apparent phenotypic disorders
Detection of heterozygousSALL1 deletions by quantitative real time PCR proves the contribution of aSALL1 dosage effect in the pathogenesis of Townes-Brocks syndrome
BMPR2 gene rearrangements account for a significant proportion of mutations in familial and idiopathic pulmonary arterial hypertension
DHPLC-based mutation analysis of ENG and ALK-1 genes in HHT Italian population Italian population
Mutation spectra ofABCC8 gene in Spanish patients with hyperinsulinism of infancy (HI)
Novel inactivating mutations of FANCC in Brazilian patients with Fanconi anemia
Mutations of the TGF-β type II receptorBMPR2 in pulmonary arterial hypertension
Functional assays testing pathogenicity of 14 cystathionine-beta synthase mutations
A rare missense mutation in a type 2 diabetes patient decreases the transcriptional activity of human sterol regulatory element binding protein-1
Novel PRG4 mutations underlie CACP in Saudi families
Distribution of human SNPs and its effect on high-throughput genotyping
Mutations in the genes encoding the pancreatic beta-cell K<sub>ATP</sub>channel subunits Kir6.2 (<i>KCNJ11</i>) and SUR1 (<i>ABCC8</i>) in diabetes mellitus and hyperinsulinism
Masking selected sequence variation by incorporating mismatches into melting analysis probes
A haplotype containing the<i>p53</i>polymorphisms Ins16bp and Arg72Pro modifies cancer risk in<i>BRCA2</i>mutation carriers
2005 Human Genome Variation Society Scientific Meeting
Photoprotein aequorin as a novel reporter for SNP genotyping by primer extension–application to the variants of mannose-binding lectin gene
RNAi-based suppression and replacement of<i>rds</i>-peripherin in retinal organotypic culture
PTCH mutations: distribution and analyses
Systematic mRNA analysis for the effect ofMLH1 andMSH2 missense and silent mutations on aberrant splicing
Predicting the transactivation activity of p53 missense mutants using a four-body potential score derived from Delaunay tessellations
Effect of the codon 72 polymorphism (c.215G&gt;C, p.Arg72Pro) in combination with somatic sequence variants in theTP53 gene on survival in patients with advanced ovarian carcinoma
Validation of the use of DNA pools and primer extension in association studies of sporadic colorectal cancer for selection of candidate SNPs
Microelectronic DNA chip for hereditary hyperferritinemia cataract syndrome, a model for large-scale analysis of disorders of iron metabolism
Prioritizing regions of candidate genes for efficient mutation screening
Altered transmission of maternal angiotensin II receptor haplotypes in fetal growth restriction
Different genetic pathways in the development of periocular sebaceous gland carcinomas in presumptive Muir-Torre syndrome patients
Functional polymorphism in<i>ALOX15</i>results in increased allele-specific transcription in macrophages through binding of the transcription factor SPI1
Spectrum of mutations in<i>mut</i>methylmalonic acidemia and identification of a common Hispanic mutation and haplotype Hispanic
Mutation, selection, and evolution of the Crohn disease susceptibility gene<i>CARD15</i>
The UMD TP53 database and website: update and revisions
Fates of Cdh23/CDH23 with mutations affecting the cytoplasmic region
An interactive web database of factor H-associated hemolytic uremic syndrome mutations: insights into the structural consequences of disease-associated mutations
Cryptic haplotypes of<i>SERPINA1</i>confer susceptibility to chronic obstructive pulmonary disease