Human Mutation - 2005

232 articles | Last updated: 2025-12-03 14:12:57
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HEY2 mutations in malformed hearts
Novel mutations in the human sucrase-isomaltase gene (SI) that cause congenital carbohydrate malabsorption
ALys amyloidosis caused by compound heterozygosity in Exon 2 (Thr70Asn) and Exon 4 (Trp112Arg) of the lysozyme gene
Low frequency of PDCD10 mutations in a panel of CCM3 probands: potential for a fourth CCM locus
Characterization of two novel GBA mutations causing Gaucher disease that lead to aberrant RNA species by using functional splicing assays
Characterization of simple sequence repeat variants linked to candidate genes for behavioral phenotypes
Functional polymorphisms in dopamine and serotonin pathway genes
Four novelTMC1 (DFNB7/DFNB11) mutations in Turkish patients with congenital autosomal recessive nonsyndromic hearing loss Turkish patients
Erratum: Novel mutations of thePCSK9 gene cause variable phenotype of autosomal dominant hypercholesterolemia
Identification of genetic aberrations on chromosome 22 outside the<i>NF2</i>locus in schwannomatosis and neurofibromatosis type 2
Identification and functional analysis ofCITED2 mutations in patients with congenital heart defects
Multiple epiphyseal dysplasia mutations inMATN3 cause misfolding of the A-domain and prevent secretion of mutant matrilin-3
Germline mutations of theMSR1 gene in prostate cancer families from Germany
The frequency of mucolipidosis type IV in the Ashkenazi Jewish population and the identification of 3 novel MCOLN1 mutations Ashkenazi Jewish population
Erratum: Dysferlin mutations in LGMD2B, Miyoshi myopathy, and atypical dysferlinopathies
High proportion of large genomic STK11 deletions in Peutz-Jeghers syndrome
Preferential occurrence of 1-2 microindels
Younger birth cohort correlates with higher breast and ovarian cancer risk in EuropeanBRCA1 mutation carriers EuropeanBRCA1 mutation carriers
Splice-site contribution in alternative splicing ofPLP1 andDM20: molecular studies in oligodendrocytes
Genetic polymorphisms in cell cycle regulatory genesMDM2 andTP53 are associated with susceptibility to lung cancer
Erratum: A double missense variation of theBUB1 gene and a defective mitotic spindle checkpoint in the pancreatic cancer cell line Hs766T
Mutation and polymorphism spectrum of the GALNS gene in mucopolysaccharidosis IVA (Morquio A)
Mutation spectrum in children with primary hemophagocytic lymphohistiocytosis: molecular and functional analyses ofPRF1, UNC13D, STX11, andRAB27A
Update of the molecular basis of familial hypercholesterolemia in The Netherlands
cblE type of homocystinuria due to methionine synthase reductase deficiency: functional correction by minigene expression
Toward a Human Variome Project
Thymopoietin (lamina-associated polypeptide 2) gene mutation associated with dilated cardiomyopathy
Identification of 29 novel and nine recurrent fibrillin-1 (FBN1) mutations and genotype-phenotype correlations in 76 patients with Marfan syndrome
Mutation rates at Y chromosome specific microsatellites
Identification of sixty-two novel and twelve known FBN1 mutations in eighty-one unrelated probands with Marfan syndrome and other fibrillinopathies
Erratum: the mutational spectrum ofENPP1 as arising after the analysis of 23 unrelated patients with generalized arterial calcification of infancy (GACI)
The first missense alteration in the MCPH1 gene causes autosomal recessive microcephaly with an extremely mild cellular and clinical phenotype
Genetic variant characterization in intron 4 of the surfactant protein B gene
Nine mutations including three novel mutations among Russian patients with acute intermittent porphyria
Molecular characterization and cancer risk associated withBRCA1 andBRCA2 splice site variants identified in multiple-case breast cancer families
Novel mutations of thePCSK9 gene cause variable phenotype of autosomal dominant hypercholesterolemia
Expression of<i>NF1</i>pseudogenes
Statistical geometry approach to the study of functional effects of human nonsynonymous SNPs
Role of the 2 adenine (g.11293_11294insAA) insertion polymorphism in the 3′ untranslated region of the factor VII (FVII) gene: molecular characterization of a patient with severe FVII deficiency
Predicting the oncogenicity of missense mutations reported in the International Agency for Cancer Research (IARC) mutation database on p53
Locus-specific databases: from ethical principles to practice
Detection of cryptic subtelomeric chromosome abnormalities and identification of anonymous chromatin using a quantitative multiplex ligation-dependent probe amplification (MLPA) assay
Correlations between genotype and pharmacological, histological, functional, and clinical phenotypes in malignant hyperthermia susceptibility
Estimation of the frequency of occult mutations for an autosomal recessive disease in the presence of genetic heterogeneity: application to genetic hearing loss disorders
Molecular characterization of a t(2;6) balanced translocation that is associated with a complex phenotype and leads to truncation of the<i>TCBA1</i>gene
Molecular diagnosis of inherited disorders: lessons from hemoglobinopathies
<i>GATA1</i>mutations in myeloproliferative disorders: nomenclature standardization and review of the literature
The human TBX5 gene mutation database
Mutations in the holocarboxylase synthetase geneHLCS
Increased risk of idiopathic chronic pancreatitis in cystic fibrosis carriers
Linear allele-specific long-range amplification: a novel method of long-range molecular haplotyping
Mutations ofAPC andMYH in unrelated Italian patients with adenomatous polyposis coli Italian
Thromboxane synthase (TBXAS1) polymorphisms in African-American and Caucasian populations: evidence for selective pressure African-American
Novel L2HGDH mutations in 21 patients with L-2-hydroxyglutaric aciduria of Portuguese origin
Novel sequence variants in theTMC1 gene in Pakistani families with autosomal recessive hearing impairment
Lafora progressive myoclonus epilepsy mutation database-EPM2A and NHLRC1 (EMP2B) genes
A novel functionally deficientMYH variant in individuals with colorectal adenomatous polyposis
Persistent mild hypothyroidism associated with novel sequence variants of theDUOX2 gene in two siblings
Spectrum of gross deletions and insertions in theRB1 gene in patients with retinoblastoma and association with phenotypic expression
Identification of mutations in the GNPTA (MGC4170) gene coding for GlcNAc-phosphotransferase α/β subunits in Korean patients with mucolipidosis type II or type IIIA
Mutation spectrum and functional analysis of epidermis-type lipoxygenases in patients with autosomal recessive congenital ichthyosis
Palindromic AT-rich repeat in the<i>NF1</i>gene is hypervariable in humans and evolutionarily conserved in primates
Whole genome SNP arrays using DNA derived from formalin-fixed, paraffin-embedded ovarian tumor tissue
Mutations in the human<i>LKB1/STK11</i>gene
Rare missense variants inATP1A2 in families with clustering of common forms of migraine
Intrachromosomal serial replication slippage in<i>trans</i>gives rise to diverse genomic rearrangements involving inversions
Genetic variation, nucleotide diversity, and linkage disequilibrium in seven telomere stability genes suggest that these genes may be under constraint
A protein-truncating mutation inCYP17A1 in three sisters with early-onset breast cancer
Functional analysis of SAND mutations in AIRE supports dominant inheritance of the G228W mutation
Analysis of 65 tuberous sclerosis complex (TSC) patients by<i>TSC2</i>DGGE,<i>TSC1</i>/<i>TSC2</i>MLPA, and<i>TSC1</i>long-range PCR sequencing, and report of 28 novel mutations
Mutation finding in patients with dysferlin deficiency and role of the dysferlin interacting proteins annexin A1 and A2 in muscular dystrophies
NovelPEX1 coding mutations and 5′ UTR regulatory polymorphisms
Homozygous microdeletion of chromosome 4q11-q12 causes severe limb-girdle muscular dystrophy type 2E with joint hyperlaxity and contractures
ATP1A2 mutations in 11 families with familial hemiplegic migraine
SALL1 mutation analysis in Townes-Brocks syndrome: twelve novel mutations and expansion of the phenotype
Mutation spectrum of the APC gene in 83 Korean FAP families
Molecular analysis of the<i>HEXA</i>gene in Italian patients with infantile and late Onset Tay-Sachs disease: detection of fourteen novel alleles
DHPLC Analysis of patients with Nevoid Basal Cell Carcinoma Syndrome reveals novel<i>PTCH</i>missense mutations in the sterol-sensing domain
De novo Alu element insertions targeted to a sequence common to the BRCA1 and BRCA2 genes
Marked decrease in specific activity contributes to disease phenotype in two human glucose 6-phosphate dehydrogenase mutants, G6PD<sub>Union</sub>and G6PD<sub>Andalus</sub>
Twenty-four novel mutations in Wilson disease patients of predominantly European ancestry predominantly European ancestry
Novel EXT1 and EXT2 mutations identified by DHPLC in Italian patients with multiple osteochondromas
Worldwide haplotype diversity and coding sequence variation at human bitter taste receptor loci
Scanning of genetic effects of alcohol metabolism gene (<i>ADH1B</i>and<i>ADH1C</i>) polymorphisms on the risk of alcoholism
UMD (Universal Mutation Database): 2005 update
Comparison of yield and genotyping performance of multiple displacement amplification and OmniPlex™ whole genome amplified DNA generated from multiple DNA sources
LDL-receptor mutations in Europe
Molecular genetics of pseudoxanthoma elasticum: type and frequency of mutations in<i>ABCC6</i>
Spectrum of molecular defects and mutation detection rate in patients with severe hemophilia A
<i>PEX1</i>mutations in the Zellweger spectrum of the peroxisome biogenesis disorders
Microdeletions and microinsertions causing human genetic disease: common mechanisms of mutagenesis and the role of local DNA sequence complexity
Factor XI deficiency database: an interactive web database of mutations, phenotypes, and structural analysis tools
Gaucher mutation N188S is associated with myoclonic epilepsy
A response to Kowarz et al.: Gaucher mutation c.680A&gt;G (p.N227S) is associated with myoclonic epilepsy
Functional analysis of 13 mutant alleles identified in Gaucher disease patients: pathogenic changes and “modifier” polymorphisms
<i>SALL4</i>mutations in Okihiro syndrome (Duane-radial ray syndrome), acro-renal-ocular syndrome, and related disorders
Strong bias in the location of functional promoter polymorphisms
The 5′ region of the<i>MSH2</i>gene involved in hereditary non-polyposis colorectal cancer contains a high density of recombinogenic sequences
3-Methylcrotonyl-CoA carboxylase deficiency: Mutation analysis in 28 probands, 9 symptomatic and 19 detected by newborn screening
Rapid recognition of aberrant dHPLC elution profiles using the Transgenomic NavigatorTM software
Dysferlin mutations in LGMD2B, Miyoshi myopathy, and atypical dysferlinopathies
Functionalin vitro characterization of 14SMPD1 mutations identified in Italian patients affected by Niemann Pick Type B disease Italian patients
Complexity of the genotype-phenotype correlation in familial exudative vitreoretinopathy with mutations in the<i>LRP5</i>and/or<i>FZD4</i>genes
Identification and analysis of 21 novel disease-causing amino acid substitutions in the conserved part of ATP7A
Evaluation of the molecular mechanisms involved in the gain of function of a Li-FraumeniTP53 Mutation
dbQSNP: A database of SNPs in human promoter regions with allele frequency information determined by single-strand conformation polymorphism-based methods
LOVD: Easy creation of a locus-specific sequence variation database using an “LSDB-in-a-box” approach
Complex gene rearrangements caused by serial replication slippage
The 2004 Human Genome Variation Society scientific meeting
Hereditary angioedema: The mutation spectrum of<i>SERPING1/C1NH</i>in a large Spanish cohort
A rapid microarray based whole genome analysis for detection of uniparental disomy
Novel rearrangement of chromosome band 22q11.2 causing 22q11 microdeletion syndrome-like phenotype and rhabdoid tumor of the kidney
Assessment of multiple displacement amplification for polymorphism discovery and haplotype determination at a highly polymorphic locus,MC1R
Twenty-six novelEFNB1 mutations in familial and sporadic craniofrontonasal syndrome (CFNS)
Identification of nine novelDHCR7 missense mutations in patients with Smith-Lemli-Opitz syndrome (SLOS)
Sarcoglycanopathies and the risk of undetected deletion alleles in diagnosis
Novel COL4A5, COL4A4, and COL4A3 mutations in Alport syndrome
Erratum: Sodium channel gene (SCN5A) mutations in 44 index patients with brugada syndrome: Different incidences in familial and sporadic disease
Novel mutations in type 2 Gaucher disease in Chinese and their functional characterization by heterologous expression
Mutation analysis of the GJB2 (Connexin 26) gene in Egypt
BMPR2 mutations have short lifetime expectancy in primary pulmonary hypertension
Niemann-Pick type C disease: Subcellular location and functional characterization of NPC2 proteins with naturally occurring missense mutations
Sjögren-Larsson syndrome: Diversity of mutations and polymorphisms in the fatty aldehyde dehydrogenase gene (ALDH3A2)
Alteration of DNA binding, dimerization, and nuclear translocation of SHOX homeodomain mutations identified in idiopathic short stature and Leri-Weill dyschondrosteosis
InSNP: A tool for automated detection and visualization of SNPs and InDels
Determination of variants in the 3′-region of the Tyrosinase gene requires locus specific amplification
The phenotypic spectrum of<i>COL2A1</i>mutations
TNF polymorphisms in Alzheimer disease and functional implications on CSF beta-amyloid levels
Sensitive multistep clinical molecular screening of 180 unrelated individuals with retinoblastoma detects 36 novel mutations in theRB1 gene
Surveyor™ Nuclease: A new strategy for a rapid identification of heteroplasmic mitochondrial DNA mutations in patients with respiratory chain defects
Subtelomeric rearrangements in the mentally retarded: A comparison of detection methods
Diagnostic DHPLC Quality Assurance (DDQA): A collaborative approach to the generation of validated and standardized methods for DHPLC-based mutation screening in clinical genetics laboratories
ATM mutations, haplotype analysis, and immunological status of Russian patients with ataxia telangiectasia
Application of genomewide SNP arrays for detection of simulated susceptibility loci
TwoATM variants and breast cancer risk
High frequency of ENG and ALK1/ACVRL1 mutations in German HHT patients
Different mechanisms preclude mutant CLDN14 proteins from forming tight junctions in vitro
Functional analysis of natural mutations in two TWIST protein motifs
<i>SCN1A</i>mutations and epilepsy
Mutations ofCOL10A1 in Schmid metaphyseal chondrodysplasia
The spectrum of aldolase B (ALDOB) mutations and the prevalence of hereditary fructose intolerance in Central Europe
On ubiquitin ligases and cancer
Novel and recurrent mutations in the C-terminal domain ofCOMP cluster in two distinct regions and result in a spectrum of phenotypes within the pseudoachondroplasia - multiple epiphyseal dysplasia dis
A new insight into PMM2 mutations in the French population
Conservation of the RBI gene in human and primates
Germline mutations of<i>AXIN2</i>are not associated with nonsyndromic colorectal cancer
Detection of heterozygous deletions and duplications in the MECP2 gene in Rett syndrome by Robust Dosage PCR (RD-PCR)
Autosomal dominant hereditary spastic paraplegia: DHPLC-based mutation analysis ofSPG4 reveals eleven novel mutations
Identification of novel GCK and HNF1A/TCF1 mutations and polymorphisms in German families with maturity-onset diabetes of the young (MODY)
Nephrogenic diabetes insipidus caused by mutation of Tyr205: A key residue of V2 vasopressin receptor function
Characterization of a new disease-causing mutation of<i>SH2D1A</i>in a family with X-linked lymphoproliferative disease
Identification of novel mutations in classical galactosemia
Alu-element insertion in the homeodomain of<i>HESX1</i>and aplasia of the anterior pituitary
Identification and characterization of three large deletions and a deletion/polymorphism in the CFTR gene
<i>eOPA1</i>: An online database for<i>OPA1</i>mutations
A separation-free assay for the detection of mutations: Combination of homogeneous time-resolved fluorescence and minisequencing
Thirteen novel mutations in the<i>NR0B1</i>(<i>DAX1</i>) gene as cause of adrenal hypoplasia congenita
Identification of a novel frameshift mutation in the DFNB31/WHRN gene in a Tunisian consanguineous family with hereditary non-syndromic recessive hearing loss
Comparison of PCR-based mutation detection methods and application for identification of mouseSult1a1 mutant embryonic stem cell clones using pooled templates
BAC-based PCR fragment microarray: High-resolution detection of chromosomal deletion and duplication breakpoints
Genomic rearrangements in theBRCA1 andBRCA2 genes
TCOF1 mutation database: Novel mutation in the alternatively spliced exon 6A and update in mutation nomenclature
Quantitative analysis of<i>SMN1</i>and<i>SMN2</i>genes based on DHPLC: A highly efficient and reliable carrier-screening test
Characterization of iduronate-2-sulfatase gene-pseudogene recombinations in eight patients with Mucopolysaccharidosis type II revealed by a rapid PCR-based method
Population spectrum of<i>ACADM</i>genotypes correlated to biochemical phenotypes in newborn screening for medium-chain acyl-CoA dehydrogenase deficiency
KinMutBase: A registry of disease-causing mutations in protein kinase domains
Ancient origin of the CAG expansion causing Huntington disease in a Spanish population
Lysinuric protein intolerance: identification and functional analysis of mutations of the SLC7A7 gene
Identification of ten novel mutations in patients with eIF2B-related disorders
Identification of 14 novel mutations inDHCR7 causing the Smith-Lemli-Opitz syndrome and delineation of theDHCR7 mutational spectra in Spain and Italy
Thirty-four novel mutations of the GLA gene in 121 patients with Fabry disease
Biotinidase deficiency: Novel mutations and their biochemical and clinical correlates
Automated splicing mutation analysis by information theory
Bovine model of Marfan syndrome results from an amino acid change (c.3598G&gt;A, p.E1200K) in a calcium-binding epidermal growth factor-like domain of fibrillin-1
Identification of novel mutations in patients with Shwachman-Diamond syndrome
Conservation of the<i>RB1</i>gene in human and primates
Mutation analysis of theHOX paralogous 4-13 genes in children with acute lymphoid malignancies: Identification of a novel germline mutation ofHOXD4 leading to a partial loss-of-function
Mutational analysis of theNPHP4 gene in 250 patients with nephronophthisis
Misprocessing of the<i>CFTR</i>protein leads to mild cystic fibrosis phenotype
Gene conversion cetween functional trypsinogen genes<i>PRSS1</i>and<i>PRSS2</i>associated with chronic pancreatitis in a six-year-old girl
<i>SIMPLE</i>mutations in Charcot-Marie-Tooth disease and the potential role of its protein product in protein degradation
Hellenic National Mutation Database: a prototype database for mutations leading to inherited disorders in the Hellenic population Hellenic population
Polymorphism in the nuclear excision repair geneERCC2/XPD: association between an exon 6-exon 10 haplotype and susceptibility to cutaneous basal cell carcinoma
A response to Suzuki et al. ?How pathogenic is the p.D104N/endostatin polymorphic allele ofCOL18A1 in Knobloch syndrome??
Late onset N-acetylglutamate synthase deficiency caused by hypomorphic alleles
<i>cblE</i>Type of homocystinuria due to methionine synthase reductase deficiency: Functional correction by minigene expression
Validation of dye-binding/high-resolution thermal denaturation for the identification of mutations in theSLC22A5 gene
Genetic association of the APP binding protein 2 gene (APBB2) with late onset Alzheimer disease
How pathogenic is the p.D104N/endostatin polymorphic allele of<i>COL18A1</i>in Knobloch syndrome?
Single nucleotide polymorphisms in theMATP gene are associated with normal human pigmentation variation
Role of ?-galactosidase and elastin binding protein in lysosomal and nonlysosomal complexes of patients with GM1-gangliosidosis
Meta-analysis of gross insertions causing human genetic disease: Novel mutational mechanisms and the role of replication slippage
A novel mutation in the dihydrolipoamide dehydrogenase E3 subunit gene (DLD) resulting in an atypical form of α-ketoglutarate dehydrogenase deficiency
Detection of ?-galactosidase a mutations causing fabry disease by denaturing high performance liquid chromatography
Mutations in the<i>MMAA</i>Gene in Patients With the<i>cblA</i>Disorder of Vitamin B<sub>12</sub>Metabolism
Novel somatic mutations in the BRCA1 gene in sporadic breast tumors
Two novel mutations of the AIRE protein affecting its homodimerization properties
Identification and characterization of five novel MAN2B1 mutations in Italian patients with alpha-mannosidosis
Hepatic manifestation is associated withALK1 in hereditary hemorrhagic telangiectasia: Identification of five novelALK1 and one novelENG mutations
Novel mutations and polymorphisms in genes causing hereditary hemorrhagic telangiectasia
Twelve novel<i>JAG1</i>gene mutations in polish Alagille syndrome patients
Seventeen novel<i>PLP1</i>mutations in patients with Pelizaeus-Merzbacher disease
Genetic polymorphisms in the transforming growth factor beta-induced gene associated with BMI
PIK3CA mutations in advanced ovarian carcinomas
Molecular characterization of Italian nevoid basal cell carcinoma syndrome patients
P gene mutations associated with oculocutaneous albinism type II (OCA2)
Large genomic rearrangements in MECP2
Two independent retrotransposon insertions at the same site within the coding region of BTK
Spectrum ofUGT1A1 mutations in Crigler-Najjar (CN) syndrome patients: identification of twelve novel alleles and genotype-phenotype correlation
Molecular diversity and thrombotic risk in protein S deficiency: The PROSIT study
Cone cGMP-gated channel mutations and clinical findings in patients with achromatopsia, macular degeneration, and other hereditary cone diseases
MALDI-TOF MS and TaqMan® assisted SNP genotyping of DNA isolated from formalin-fixed and paraffin-embedded tissues (FFPET)
Algorithm for efficient<i>PKHD1</i>mutation screening in autosomal recessive polycystic kidney disease (ARPKD)
PolyMAPr: Programs for polymorphism database mining, annotation, and functional analysis
Spectrum of sequence variations in the <i>FANCA</i> gene: An International Fanconi Anemia Registry (IFAR) study
Natural haplotypes in the regulatory sequences affect human alcohol dehydrogenase 1C ( <i>ADH1C</i> ) gene expression
DNA damage detection and repair, and the involvement of epigenetic states
Mutation rates in the dystrophin gene: A hotspot of mutation at a CpG dinucleotide
Meta‐Analysis of gross insertions causing human genetic disease: Novel mutational mechanisms and the role of replication slippage
Toward the evaluation of function in genetic variability: Characterizing human SNP frequencies and establishing BAC‐transgenic mice carrying the human<i>CYP1A1_CYP1A2</i>locus
A common haplotype at the 5′ end of the<i>RET</i>proto‐oncogene, overrepresented in Hirschsprung patients, is associated with reduced gene expression
Functional significance of a deep intronic mutation in the <i>ATM</i> gene and evidence for an alternative exon 28a
<i>Retracted</i> : Identification of seven novel mutations in ABCD1 by a DHPLC‐based assay in Italian patients with X‐linked adrenoleukodystrophy Italian patients
A novel mutation in the GUCY2D gene responsible for an early onset severe RP different from the usual GUCY2D‐LCA phenotype
Erratum: Germline mutations in <i>MLH1</i> , <i>MSH2</i> and <i>MSH6</i> in Korean hereditary non‐polyposis colorectal cancer fa Korean
Large deletions of the APC gene in 15% of mutation‐negative patients with classical polyposis (FAP): A Belgian study
Distinct novel mutations affecting the same base in the <i>NIPA1</i> gene cause autosomal dominant hereditary spastic paraplegia in two Chinese families
Molecular basis of methylmalonyl‐CoA mutase apoenzyme defect in 40 European patients affected by <i>mut</i> ° and <i>mut</i> – forms of methylmalonic ac European patients
Characterization of genotype–phenotype relationships and stratification by the <i>CARD15</i> variant genotype for inflammatory bowel disease susceptibility loci using multiple
A novel constitutional mutation affecting splicing of retinoblastoma tumor suppressor gene intron 23 causes partial loss of pRB activity
Erratum: Identification of novel and rare mutations in California Hispanic and African American cystic fibrosis patients Hispanic; African American
Molecular and functional analysis of intragenic SMN1 mutations in patients with spinal muscular atrophy