Human Mutation - 2003

247 articles | Last updated: 2025-12-03 14:12:57
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Large deletions in the polycystic kidney disease 1 (PKD1) gene
VSD: A database for schizophrenia candidate genes focusing on variations
Pyrosequencing?-based SNP allele frequency estimation in DNA pools
A genetic polymorphism in the coding region of the gastric intrinsic factor gene (GIF) is associated with congenital intrinsic factor deficiency
Haplotypes ofCYP3A4 and their close linkage withCYP3A5 haplotypes in a Japanese population Japanese population
First report of a genetic polymorphism of the cytochrome P450 3A43 (CYP3A43) gene: Identification of a loss-of-function variant
Nonclassical splicing mutations in the coding and noncoding regions of the ATM Gene: Maximum entropy estimates of splice junction strengths
Subtelomeric deletions detected in patients with idiopathic mental retardation using multiplex ligation-dependent probe amplification (MLPA)
Novel mutations in theAtlastin gene (SPG3A) in families with autosomal dominant hereditary spastic paraplegia and evidence for late onset forms of HSP linked to theSPG3A locus
DGGE-based whole-gene mutation scanning of the dystrophin gene in Duchenne and Becker muscular dystrophy patients
Twenty-two novel mutations in the lysosomal ?-glucosidase gene (GAA) underscore the genotype-phenotype correlation in glycogen storage disease type II
Allelic genes of blood group antigens: A source of human mutations and cSNPs documented in the Blood Group Antigen Gene Mutation Database
Solid renal tumor severity in von Hippel Lindau disease is related to germline deletion length and location
Phenotype-genotype characterization of 10 families with severe a subunit factor XIII deficiency
Detection of thirty novel<i>FBN1</i>mutations in patients with Marfan syndrome or a related fibrillinopathy
Variants inTNFRSF5 locus and association analysis with Hepatitis B virus (HBV) infection
Cystathionine?-synthase deficiency in Georgia (USA): Correlation of clinical and biochemical phenotype with genotype
POLG mutations in sporadic mitochondrial disorders with multiple mtDNA deletions
Auditory neuropathy in patients carrying mutations in the otoferlin gene (<i>OTOF</i>)
CYP2D6 genotyping strategy based on gene copy number determination by TaqMan real-rime PCR
Screening of 383 unrelated patients affected with Menkes disease and finding of 57 gross deletions in<i>ATP7A</i>
Genomic deletions inMSH2 orMLH1 are a frequent cause of hereditary non-polyposis colorectal cancer: Identification of novel and recurrent deletions by MLPA
A pathogenic glutamate-to-aspartate substitution (D296E) in the pyruvate dehydrogenase E1 subunit genePDHA1
Bridging structural biology and genetics by computational methods: An investigation into how the R774C mutation in the AR gene can result in complete androgen insensitivity syndrome
Erratum: mutational screening of the RB1 gene in Indian patients with retinoblastoma reveals eight novel and several recurrent mutations
Mutational spectrum of the succinate semialdehyde dehydrogenase (ALDH5A1) gene and functional analysis of 27 novel disease-causing mutations in patients with SSADH deficiency
NDP gene mutations in 14 French families with Norrie disease
Mutations ofZFPM2/FOG2 gene in sporadic cases of tetralogy of Fallot
Detailed analysis of five mutations in dihydropyrimidine dehydrogenase detected in cancer patients with 5-fluorouracil-related side effects
SomaticNF1 mutation spectra in a family with neurofibromatosis type 1: Toward a theory of genetic modifiers
DHPLC analysis of potassium ion channel genes in congenital long QT syndrome
Fabry disease: Characterization of ?-galactosidase A double mutations and the D313Y plasma enzyme pseudodeficiency allele
Novel cytochrome P450 1B1 (CYP1B1) mutations in patients with primary congenital glaucoma in France
Diagnostics in patients with glutathione synthetase deficiency but without mutations in the exons of the GSS gene
Molecular analysis of the glyoxylate reductase (GRHPR) gene and description of mutations underlying primary hyperoxaluria type 2
Mutation screening of the C1 inhibitor gene among Hungarian patients with hereditary angioedema
Loss of a single amino acid from dystrophin resulting in Duchenne muscular dystrophy with retention of dystrophin protein
Alleles of polymorphic sites that correspond to hyperactive variants of CYP1B1 protein are significantly less frequent in Japanese as compared to American and German populations
Identification of variants inNFKBIA and association analysis with hepatocellular carcinoma risk among chronic HBV patients
Single nucleotide polymorphisms and haplotype frequencies ofCYP3A5 in a Japanese population
Characterisation of a 161 kb deletion extending from the NBR1 to the BRCA1 genes in a French breast-ovarian cancer family
Sodium channel gene (SCN5A) mutations in 44 index patients with Brugada syndrome: Different incidences in familial and sporadic disease
Fourteen novel OPA1 mutations in autosomal dominant optic atrophy including two de novo mutations in sporadic optic atrophy
A Novel PTPN11 mutation in LEOPARD syndrome
The mutation spectrum of the APC gene in FAP patients from southern Italy: Detection of known and four novel mutations
Identification of four novel melanocortin 1 receptor (MC1R) gene variants in a Mediterranean population
Mutations ofZFPM2/FOG2 gene in sporadic cases of tetralogy of Fallot
Genotyping microarray (gene chip) for the<i>ABCR</i>(<i>ABCA4</i>) gene
Molecular pathology of NEU1 gene in sialidosis
Tau (MAPT) mutation Arg406Trp presenting clinically with Alzheimer disease does not share a common founder in Western Europe
Fifty microdeletions among 112 cases of Sotos syndrome: Low copy repeats possibly mediate the common deletion
High throughput detection of microsatellite instability by denaturing high-performance liquid chromatography
The<i>RET</i>C620S mutation causes multiple endocrine neoplasia type 2A (MEN2A) but not Hirschsprung disease (HSCR) in a family cosegregating both phenotypes
DNA deamination enables direct PCR amplification of the cystatin B (CSTB) gene-associated dodecamer repeat expansion in myoclonus epilepsy type Unverricht-Lundborg
Glucokinase (<i>GCK</i>) mutations in hyper- and hypoglycemia: Maturity-onset diabetes of the young, permanent neonatal diabetes, and hyperinsulinemia of infancy
The molecular basis of glutamate formiminotransferase deficiency
Genetic variation in a haplotype block spanningIDE influences Alzheimer disease
Identification of 21 novel glucokinase (GCK) mutations in UK and European Caucasians with maturity-onset diabetes of the young (MODY) European Caucasians
Identification of twelve novel mutations in patients with classic and variant forms of maple syrup urine disease
Mutational analysis of<i>BRCA1</i>and<i>BRCA2</i>in Mediterranean Spanish women with early-onset breast cancer: Identification of three novel pathogenic mutations Mediterranean Spanish women
Ten novel mutations in<i>VMD2</i>associated with Best macular dystrophy (BMD)
Identification of nine novel arylsulfatase a (ARSA) gene mutations in patients with metachromatic leukodystrophy (MLD)
Deletion mapping in Alport syndrome and Alport syndrome-diffuse leiomyomatosis reveals potential mechanisms of visceral smooth muscle overgrowth
Novel MLH1 mutations and a novel MSH2 polymorphism identified by SSCP and DHPLC in Portuguese HNPCC families
ConstitutionalNF1 mutations in neurofibromatosis 1 patients with malignant peripheral nerve sheath tumors
<i>BUB1</i>infrequently mutated in human breast carcinomas
Identification of a frequent variant inALG6, the cause of Congenital Disorder of Glycosylation-Ic
Identification of 58 novel mutations in Niemann-Pick disease type C: Correlation with biochemical phenotype and importance of<i>PTC1</i>-like domains in<i>NPC1</i>
Mutational spectrum of theWFS1 gene in Wolfram syndrome, nonsyndromic hearing impairment, diabetes mellitus, and psychiatric disease
Approaches to identify genes for complex human diseases: Lessons from Mendelian disorders
Analysis of<i>BRCA1</i>and<i>BRCA2</i>genes in Spanish breast/ovarian cancer patients: A high proportion of mutations unique to Spain and evidence of founder effects Spanish
MLYCD mutation analysis: Evidence for protein mistargeting as a cause of MLYCD deficiency
Rapid detection of ?-globin gene (HBB) mutations coupling heteroduplex and primer-extension analysis by DHPLC
RNA analysis of eight<i>BRCA1</i>and<i>BRCA2</i>unclassified variants identified in breast/ovarian cancer families from Spain
Mutational analysis of theAGL gene: Five novel mutations in GSD III patients
Mutation spectrum of human<i>SLC39A4</i>in a panel of patients with acrodermatitis enteropathica
Identification of eight novel glucokinase mutations in Italian children with maturity-onset diabetes of the young
Spectrum ofFANCA mutations in Italian Fanconi anemia patients: Identification of six novel alleles and phenotypic characterization of the S858R variant
Mutational screening of the RB1 gene in Indian patients with retinoblastoma reveals eight novel and several recurrent mutations
The E148QMEFV allele is not implicated in the development of familial Mediterranean fever
CFTR genotypes in patients with normal or borderline sweat chloride levels
Structural organization of the human carbamyl phosphate synthetase I gene (CPS1) and identification of two novel genetic lesions
Erratum: Neurofibromatosis type 1 (NF1): Identification of eight unreported mutations in NF1 gene in Italian patients
Update of the UMD-<i>FBN1</i>mutation database and creation of an<i>FBN1</i>polymorphism database
Genotyping African haplotypes in ATM using a co-spotted single-base extension assay African
Comparative analysis of the FOXL2 gene and characterization of mutations in BPES patients
Trimethylaminuria and a humanFMO3 mutation database
Translocation and gross deletion breakpoints in human inherited disease and cancer II: Potential involvement of repetitive sequence elements in secondary structure formation between DNA ends
Translocation and gross deletion breakpoints in human inherited disease and cancer I: Nucleotide composition and recombination-associated motifs
Negligible validation rate for public domain stop-codon SNPs
PTEN: One Gene, Many Syndromes
Standardizing mutation nomenclature: Why bother?
Spectrum of sequence variation in the FANCG gene: An International Fanconi Anemia Registry (IFAR) study
ATM gene alterations in childhood acute lymphoblastic leukemias
Association analysis of novelTBX21 variants with asthma phenotypes
Identification of novel variants in transforming growth factor-beta 1 (TGFB1) gene and association analysis with bone mineral density
Identification and characterization of genomic rearrangements ofMSH2 andMLH1 in Lynch syndrome (HNPCC) by novel techniques
Molecular analysis in Fabry disease in Spain: Fifteen novel GLA mutations and identification of a homozygous female
CFTR mutations in patients from Colombia: Implications for local and regional molecular diagnosis programs
Twenty-three novelBRCA1 andBRCA2 sequence alterations in breast and/or ovarian cancer families in Southern Germany
Five novel inactivating mutations in the thyroid peroxidase gene responsible for congenital goiter and iodide organification defect
Mutational analysis of the<i>BRCA1</i>-interacting genes<i>ZNF350/ZBRK1</i>and<i>BRIP1</i>/<i>BACH1</i>among<i>BRCA1</i>and<i>BRCA2</i>-negative probands from breast-ovarian cancer families and among
Deficiency of UDP-GlcNAc:Dolichol Phosphate N-Acetylglucosamine-1 Phosphate Transferase (DPAGT1) Causes a Novel Congenital Disorder of Glycosylation Type Ij
Mutation screening of the N-myc downstream-regulated gene 1 (NDRG1) in patients with Charcot-Marie-Tooth Disease
Evaluation of multiplex capillary heteroduplex analysis: A rapid and sensitive mutation screening technique
Gross rearrangements in theMECP2 gene in three patients with rett syndrome: Implications for routine diagnosis of Rett syndrome
Mutations and polymorphisms in the human methyl CpG-binding protein MECP2
Mitochondrial DNA variant 16189T&gt;C is associated with susceptibility to endometrial cancer
A genetic and phenotypic analysis in Spanish spinal muscular atrophy patients with c.399_402del AGAG, the most frequently found subtle mutation in the SMN1 gene
Digenic progressive external ophthalmoplegia in a sporadic patient: Recessive mutations in<i>POLG</i>and<i>C10orf2/Twinkle</i>
Detection of single nucleotide substitution by competitive allele-specific short oligonucleotide hybridization (CASSOH) with immunochromatographic strip
Detection and assignment ofTP53 mutations in tumor DNA using peptide mass signature genotyping
Detection of mitochondrial DNA mutations in gestational trophoblastic disease
Novel intronic polymorphisms in theRET proto-oncogene and their association with Hirschsprung disease
Founder mutation in the BRCA1 gene in Malay breast cancer patients from Singapore
Four novel mutations inAPOB causing heterozygous and homozygous familial hypobetalipoproteinemia
BRCA1 and BRCA2 mutations in breast/ovarian cancer patients from central Italy
Myocilin analysis by DHPLC in French POAG patients: Increased prevalence of Q368X mutation
Neurofibromatosis eurofibromatosis type 1 (NF1): Identification of eight unreported mutations in NF1 gene in Italian patients Italian
The 342-kb deletion in<i>GJB6</i>is not present in patients with non-syndromic hearing loss from Austria patients from Austria
Polymorphisms in fatty acid-binding protein-3 (FABP3) - putative association with type 2 diabetes mellitus
Determination of SMN1 and SMN2 copy number using TaqMan™ technology
A specific GFP expression assay, penetrance estimate, and histological assessment for a putative splice site mutation inBRCA1
Clear relationship betweenETF/ETFDH genotype and phenotype in patients with multiple acyl-CoA dehydrogenation deficiency
Spectrum of<i>CLN6</i>mutations in variant late infantile neuronal ceroid lipofuscinosis
Enhanced allele-specific PCR discrimination in SNP genotyping using 3? locked nucleic acid (LNA) primers
Identification of 16 novel mutations in the argininosuccinate synthetase gene and genotype-phenotype correlation in 38 classical citrullinemia patients
Genetic diversity of the alpha-1-antitrypsin gene in Africans identified using a novel genotyping assay
Independent mutational events are rare in the ATM gene: Haplotype prescreening enhances mutation detection rate
One gene, two phenotypes:ROR2 mutations in autosomal recessive Robinow syndrome and autosomal dominant brachydactyly type B
Denaturing HPLC analysis of DNA deletions and insertions
A phylogenetic approach to assessing the significance of missense mutations in disease genes
The molecular basis of glutamate formiminotransferase deficiency
Epigenetic detection of human chromosome 14 uniparental disomy
Spectrum of CBS mutations in 16 homocystinuric patients from the Iberian Peninsula: High prevalence of T191M and absence of I278T or G307S
Screening for large rearrangements of the<i>BRCA1</i>gene in German breast or ovarian cancer families using semi-quantitative multiplex PCR method
Six novel mutations of theRUNX2 gene in Italian patients with cleidocranial dysplasia
The analysis ofBRCA1 mutations in eastern Chinese patients with early onset breast cancer and affected relatives
Prion susceptibility and protective alleles exhibit marked geographic differences
Comparison of the CFTR mutation spectrum in three cohorts of patients of Celtic origin from Brittany (France) and Ireland Celtic origin
Severe hypophosphatasia: Characterization of fifteen novel mutations in the ALPL gene
Single base substitutions at the initiator codon in the mitochondrial acetoacetyl-CoA thiolase (ACAT1/T2) gene result in production of varying amounts of wild-type T2 polypeptide
Cultured fibroblasts as a tool for improvement of molecular analysis in ornithine transcarbamylase (OTC) deficiency
Molecular and phenotypic heterogeneity in mitochondrial trifunctional protein deficiency due to ?-subunit mutations
Human Gene Mutation Database (HGMD<sup>®</sup>): 2003 update
Variations of the human glucocorticoid receptor gene (NR3C1): Pathological and in vitro mutations and polymorphisms
Pseudoexon activation in theDMD gene as a novel mechanism for Becker muscular dystrophy
Molecular detection of novel WFS1 mutations in patients with Wolfram syndrome by a DHPLC-based assay
Mutation analysis in patients with N-acetylglutamate synthase deficiency
De novo<i>SCN1A</i>mutations are a major cause of severe myoclonic epilepsy of infancy
AnABCA4 genomic deletion in patients with Stargardt disease
A novel splice site mutation in theTRIM37 gene causes mulibrey nanism in a Turkish family with phenotypic heterogeneity
A multiplex methylation PCR assay for identification of uniparental disomy of chromosome 7
Mutations in the molybdenum cofactor biosynthetic genesMOCS1, MOCS2, andGEPH
Mutant NDUFV2 subunit of mitochondrial complex I causes early onset hypertrophic cardiomyopathy and encephalopathy
Incorrect headers and abstracts inHuman Mutation, Volume 21, Issue 1, January 2003
Capillary electrophoresis-based single strand DNA conformation analysis in high-throughput mutation screening
Mutational analysis of the ATRX gene by DGGE: A powerful diagnostic approach for the ATRX syndrome
Detection of hotspot mutations and polymorphisms using an enhanced PCR-RFLP approach
Characterization of mutations in severe methylenetetrahydrofolate reductase deficiency reveals an FAD-responsive mutation
RettBASE: The IRSA MECP2 variation database—a new mutation database in evolution
Correlation between genotype, metabolic data, and clinical presentation in carnitine palmitoyltransferase 2 (CPT2) deficiency
Designing and implementing quality control for multi-center screening of mutations in the ATM gene among women with breast cancer
Novel mutations in the CLN6 gene causing a variant late infantile neuronal ceroid lipofuscinosis
Germline TP53 Mutations and Li-Fraumeni Syndrome
A Novel mutation L619F in the cardiac Na channel SCN5A associated with long-QT syndrome (LQT3): a role for the I-II linker in inactivation gating
Spectrum of GJB2 mutations in Turkey comprises both Caucasian and Oriental variants: Roles of parental consanguinity and assortative mating Oriental variants
Oculopharyngeal muscular dystrophy (OPMD) due to a small duplication in the PABPN1 gene
BRCA1 and BRCA2 mutation analysis in breast-ovarian cancer families from northeastern Poland
Molecular variation of human HSP90α and HSP90β genes in Caucasians
Functional consequences of an<i>LMNA</i>mutation associated with a new cardiac and non-cardiac phenotype
Characterization of 17 novel endoglin mutations associated with hereditary hemorrhagic telangiectasia
IVS10–6T&gt;G, an ancient ATM germline mutation linked with breast cancer
ATM gene alterations in childhood acute lymphoblastic leukemias
Two common founder mutations of the fanconi anemia group g gene FANCG/XRCC9 in the Japanese population
The molecular basis of phenylalanine hydroxylase deficiency in Croatia
Mutation analysis of the entire keratin 5 and 14 genes in patients with epidermolysis bullosa simplex and identification of novel mutations
Pathogenic mutations of the lipoprotein lipase gene in Chinese patients with hypertriglyceridemic type 2 diabetes
Human growth hormone 1 (<i>GH1</i>) gene expression: Complex haplotype-dependent influence of polymorphic variation in the proximal promoter and locus control region
Novel mutations of the growth hormone 1 (GH1) gene disclosed by modulation of the clinical selection criteria for individuals with short stature
Phenylketonuria mutations in Europe
Genetic diversity within the R408W phenylketonuria mutation lineages in Europe
Mitochondrial DNA mutation correlates with stage progression and prognosis in non-small cell lung cancer
HowPAH gene mutations cause hyper-phenylalaninemia and why mechanism matters: Insights from in vitro expression
Phenylketonuria: Genotype-phenotype correlations based on expression analysis of structural and functional mutations in<i>PAH</i>
Comparative multiplex dosage analysis detects whole exon deletions at the phenylalanine hydroxylase locus
PAHdb 2003: What a locus-specific knowledgebase can do
Mutations in theAUH gene cause 3-methylglutaconic aciduria type I
A role for overdominant selection in phenylketonuria? Evidence from molecular data
Focus on the molecular genetics of phenylketonuria
The molecular basis of phenylketonuria in Lithuania
The molecular basis of phenylketonuria in Latvia
Mutational spectrum in German patients with phenylalanine hydroxylase deficiency
Tetrahydrobiopterin sensitivity in German patients with mild phenylalanine hydroxylase deficiency
Gene structure of human carbamylphosphate synthetase 1 and novel mutations in patients with neonatal onset
Germline mutations in the<i>BRCA1</i>and<i>BRCA2</i>genes in Turkish breast/ovarian cancer patients
A double missense variation of theBUB1 gene and a defective mitotic spindle checkpoint in the pancreatic cancer cell line Hs766T
Identification of six novel<i>MSH2</i>and<i>MLH1</i>germline mutations in HNPCC
Identification of seven novel mutations in theGAN gene
Genetic analysis of familial colorectal cancer in Israeli Arabs
Genetic analysis of Variegate Porphyria (VP) in Italy: Identification of six novel mutations in the protoporphyrinogen oxidase (PPOX) gene
Mutational analysis of<i>BRCA2</i>in Spanish breast cancer patients from Castilla-Leon: Identification of four novel truncating mutations
Novel<i>MLH1</i>and<i>MSH2</i>germline mutations in the first HNPCC families identified in Slovakia
Mutational spectrum of the iduronate 2 sulfatase gene in 25 unrelated Korean Hunter syndrome patients: Identification of 13 novel mutations
Six novel ATM mutations in Italian patients with classical ataxia-telangiectasia
Identification of novel SNPs in the interleukin 6 receptor gene (IL6R)
Bruton tyrosine kinase gene mutations in Argentina
Mutations in the human homologue ofDrosophila patched in Japanese nevoid basal cell carcinoma syndrome patients
Haplotype analysis of the<i>BRCA2</i>9254delATCAT recurrent mutation in breast/ovarian cancer families from Spain
Mutation analysis in 16 patients with mtDNA depletion
TP53 mutations in familial breast cancer: Functional aspects
TP53 mutation in colorectal cancer
DisruptingTP53 in mouse models of human cancers
TP53 and head and neck neoplasms
TP53 mutations in human skin cancers
GermlineTP53 mutations and Li-Fraumeni syndrome
The UMD-p53 database: New mutations and analysis tools
TP53 in hematological cancer: Low incidence of mutations with significant clinical relevance
TP53 family members and human cancers
TP53 and liver carcinogenesis
TheTP53 gene, tobacco exposure, and lung cancer
TP53 and gastric carcinoma: A review
TP53 and ovarian cancer
TP53 mutations in workers exposed to occupational carcinogens
<i>TP53</i>and breast cancer
Significance of TP53 mutations in human cancer: A critical analysis of mutations at CpG dinucleotides
Focus on the p53 gene and cancer: Advances inTP53 mutation research
The role of TP53 in Cervical carcinogenesis
Absence of exon 15BRAF germline mutations in familial melanoma
Rapid detection of exon 1 NRAS gene mutations using universal heteroduplex generator technology
Clinical and molecular study in congenital muscular dystrophy with partial laminin ?2 (LAMA2) deficiency
Characterizing mutations in samples with low-level mosaicism by collection and analysis of DHPLC fractionated heteroduplexes
Missense mutations in the DNA-binding region and termination codon in PAX6
The Iranian Human Mutation Gene Bank: A data and sample resource for worldwide collaborative genetics research
Spectrum of sequence variation in theFANCG gene: An International Fanconi Anemia Registry (IFAR) study
Mutations in theCACNA1F andNYX genes in British CSNBX families
Phenotypic cellular characterization of an Ataxia telangiectasia patient carrying a causal homozygous missense mutation
Screening of patients with hereditary spastic paraplegia reveals seven novel mutations in theSPG4 (Spastin) gene
Identification of GUCY2D gene mutations in CORD5 families and evidence of incomplete penetrance
BRCA2 germline mutations in Cypriot patients with familial breast/ovarian cancer
NF1 gene analysis based on DHPLC
Functional analysis of LKB1/STK11 mutants and two aberrant isoforms found in Peutz-Jeghers Syndrome patients
Comprehensive scanning of theATM gene with DOVAM-S
High-throughput single strand conformation polymorphism mutation detection by automated capillary array electrophoresis: validation of the method
Novel mutations in the<i>TCIRG1</i>gene encoding the a3 subunit of the vacuolar proton pump in patients affected by infantile malignant osteopetrosis
Further evidence for role of a promoter variant in the TNFRSF6 gene in Alzheimer disease
MEFV sequence variants and amyloidosis: still an enigmatic question