Human Mutation - 1994

136 articles | Last updated: 2025-12-03 14:12:57
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Cystic fibrosis patients from the black sea region: The 1677delTA mutation
A microtiter plate assay for determining apolipoprotein E genotype and discovery of a rare allele
A Gly859Ser substitution in the triple helical domain of the α2 chain of type I collagen resulting in osteogenesis imperfecta type III in two unrelated individuals
A missense mutation (F87L) in exon 3 of the cystic fibrosis transmembrane conductance regulator gene
A new frameshift mutation, insertion of ATCT, at codon 48 in the β-globin gene causes β-thalassemia in an Indian proband
Transthyretin VAL107, a new variant associated with familial cardiac and neuropathic amyloidosis
The Ashkenazi Jewish Fanconi anemia mutation: Incidence among patients and carrier frequency in the at-risk population Ashkenazi Jewish
APC andp53 mutations in de novo colorectal adenocarcinomas
Further evidence that the failure to cleave the aminopropeptide of type I procollagen is the cause of Ehlers-Danlos syndrome type VII
Mutations in Steroid 21-Hydroxylase (CYP21)
Four newly identified ornithine transcarbamylase (OTC) mutations (D126G, R129H, I172M and W332X) in Japanese male patients with early-onset OTC deficiency
Announcement
Two NF1 mutations: Frameshift in the GAP-related domain, and loss of two codons toward the 3′ end of the gene
New alleles at microsatellite loci in CEPH families mainly arise from somatic mutations in the lymphoblastoid cell lines
A novel frameshift deletion in type IV collagen α5 gene in a juvenile-type Alport syndrome patient: An adenine deletion (2940/2943 del A) in exon 34 of COL4A5
A single-base deletion mutation in a Turkish patient with fucosidosis
Two new rhodopsin transversion mutations (L40R; M216K) in families with autosomal dominant retinitis pigmentosa
Use of denaturing gradient gel electrophoresis to identify mutant sequences in the β-glucocerebrosidase gene
A rapid restriction site screening method for the Pro207→Leu mutation in the lipoprotein lipase gene
Masthead
Masthead
Enzymatic amplification of synthetic oligodeoxyribonucleotides: Implications for triplet repeat expansions in the human genome
A new mtDNA mutation in the tRNALeu(UUR) gene associated with maternally inherited cardiomyopathy
Recurrent missense mutations at the first and second base of codon Arg243 in human lipoprotein lipase in patients of different ancestries different ancestries
Reverse dot blot probes for the screening of β-thalassernia mutationsin Asians and American blacks Asians and American blacks
Identification of a novel missense mutation (G314E) in exon 7 of the cystic fibrosis transmembrane conductance regulator gene identified in a CF patient with pancreatic sufficiency
Insertion of a T next to the donor splice site of intron 1 causes aberrantly spliced mRNA in a case of infantile GM1-gangliosidosis
Sequence of the —530 region of the β-globin gene of sickle cell anemia patients with the Arabian haplotype
Infrequent mutation of theWT1 gene in 77 Wilms' tumors
A single base mutation in the type II procollagen gene (COL2A1) that converts glycine α1-247 to serine in a family with late-onset spondyloepiphyseal dysplasia
A rapid and simple DNA fingerprinting method using RLFP and SSCP analysis of the hypervariable noncoding region of human mitochondrial DNA
Nonsense mutation in the homeobox region of the aniridia gene
Occurrence of a missense mutation in one allele and a seven basepair deletion in the other allele in a patient with adenine phosphoribosyltransferase deficiency
A mutant stop codon (TAG) in the IDUA gene is used as an acceptor splice site in a patient with Hurler syndrome (MPS IH)
Searching for the 1 in 2,400,000: A review of dystrophin gene point mutations
(ATT) trinucleotide repeats in the antithrombin gene and their use in determining the origin of repeated mutations
A new mutation (TTR Ala-47) in the transthyretin gene associated with hereditary amyloidosis
Characterization of the deletion breakpoints in a patient with steroid sulfatase deficiency
Molecular basis of neurofibromatosis type 1 (NF1): Mutation analysis and polymorphisms in the NF1 gene
Mutations of the iduronate-2-sulfatase (IDS) gene in patients with hunter syndrome (mucopolysaccharidosis II)
A tandem CC → TT transition in the p53 gene of a breast cancer
A novel mutation (G1249R) in exon 20 of the CFTR gene
Molecular genetics of metachromatic leukodystrophy
Mutations causing gaucher disease
Exhaustive analysis of the P53 gene coding sequence by denaturing gradient gel electrophoresis: Application to the detection of point mutations in acute leukemias
Identification of a point mutation and germinal mosaicism in a duchenne muscular dystrophy family
Pyruvate dehydrogenase deficiency in a male caused by a point mutation (F205L) in the E1α subunit
Identification of a novel nonsense mutation in the fibrillin gene (FBN1) using nonisotopic techniques
A double-variant transthyretin allele (SER 6, ILE 33) in the Israeli patient “SKO” with familial amyloidotic polyneuropathy
The ornithine transcarbamylase gene: New “Private” mutations in four patients and study of a polymorphism
Scanning method to establish the molecular basis of protein C deficiencies
High throughput and economical mutation detection and RFLP analysis using a minimethod for DNA preparation from whole blood and acrylamide gel electrophoresis
North Eurasian origin of the myotonic dystrophy mutation
Identification of mutations (D128G, H141L) in the liver arginase gene of patients with hyperargininemia
Molecular characterization of a DDEI melting polymorphism at the angiotensin I-converting enzyme (ACE) locus
Time-resolved fluorometry in the diagnosis of Leber hereditary optic neuroretinopathy
Genetic basis of inherited peripheral neuropathies
Detection of point mutations by solid-phase methods
Transcriptional regulation of gene expression: Mechanisms and pathophysiology
Description and functional implications of a novel mutation in the sex-determining gene SRY
A novel mitochondrial point mutation in a maternal pedigree with sensorineural deafness
Mutation analysis of 19 North American mucopolysaccharidosis type I patients: Identification of two additional frequent mutations
A novel β-thalassemia mutation: Frameshift at codon 59 detected in an Italian carrier Italian
Mutations in the human peripherin/RDS gene associated with autosomal dominant retinitis pigmentosa
Two coding change mutations in theHIS22 allele characterize the salivary histatin 3-2 protein variant
Seven new mutations in the human ornithine transcarbamylase gene
An A-to-C substitution involving the translation initiation codon in a patient with myophosphorylase deficiency (McArdle's disease)
Population variation of common cystic fibrosis mutations
Metachromatic leukodystrophy in the Navajo Indian population: A splice site mutation in intron 4 of the arylsulfatase a gene Navajo Indian population
Erroneous assignment of Gaucher disease genotype as a consequence of a complete gene deletion
Autosomal dominant spondylarthropathy due to a type II procollagen gene(COL2A1) point mutation
Exon eight APC mutations account for a disproportionate number of familial adenomatous polyposis families
Linkage disequilibrium across the fibrinogen locus as shown by five genetic polymorphisms, G/A−455 (HaeIII), C/T−148 (HindIII/AluI), T/G+1689 (AvaII), andBclI (β-fibrinogen) andTaqI (α-fibrinogen), an
Molecular basis of fabry disease: Mutations and polymorphisms in the human α-galactosidase A gene
Does cancer kill the individual and save the species?
Analysis of the CFTR gene in the Spanish population: SSCP-screening for 60 known mutations and identification of four new mutations (Q30X, A120T, 1812-1 G→A, and 3667de14)
A novel deletion in exon 15 of the adenomatous polyposis coli gene in an Italian kindred Italian kindred
A novel mutation at the splice junction of exon 9 of the APC gene in familial adenomatous polyposis
Phenylketonuria in China: Identification and characterization of three novel nucleotide substitutions in the human phenylalanine hydroxylase gene
A cystic fibrosis patient with ΔF508, G542X and a deletion at the D7S8 locus
Erratum
Masthead
Detecting prion protein gene mutations by denaturing gradient gel electrophoresis
A 32-bp deletion (2991del32) in the cystic fibrosis gene associated with CFTR mRNA reduction
Three novel β-hexosaminidase a mutations in obligate carriers of Tay-Sachs disease
Development of RNA-SSCP protocols for the identification and screening of CFTR mutations: Identification of two new mutations
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Screening for mutations in the exon 26 of the apolipoprotein B gene in hypercholesterolemic finnish families by the single-strand conformation polymorphism method finnish families
A single base pair substitution within the paired box of PAX3 in an individual with Waardenburg syndrome type 1 (WS1)
Five novel missense mutations of the phenylalanine hydroxylase gene in phenylketonuria
Erratum
Molecular basis of acute intermittent porphyria: Mutations and polymorphisms in the human hydroxymethylbilane synthase gene
Characterization of two novel adenomatous polyposis coli (APC) gene mutations in patients with familial adenomatous polyposis (FAP)
FH-Sydney 1 and 2: Two novel frameshift mutations in exon 10 of the low-density lipoprotein receptor gene detected by heteroduplex formation
A robotics-assisted procedure for large scale cystic fibrosis mutation analysis
Mutation at the catalytic site (M519V) in glycogen storage disease type II (Pompe disease)
DNA mutational analysis of type 1 and type 3 gaucher patients: How well do mutations predict phenotype?
Mutations in the retinoblastoma gene and their expression in somatic and tumor cells of patients with hereditary retinoblastoma
Three novel mutations (I506S, S466X, 1651A→T) in exon 10 of the cystic fibrosis transmembrane conductance regulator (CFTR) detected in patients of Southern German Descent Southern German Descent
Identification of a new frameshift mutation (3724 delG) in exon 19 of the CFTR gene
A novel δº-Thalassemia mutation: TGG→TAG (TRP→STOP) at codon 37
C to T mutation causing premature termination of CD40 ligand at amino acid 221 in a patient affected by Hyper IgM syndrome
A novel insertional mutation of a single base in exon 34 of the neurofibromatosis-1 gene
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Mutation detection by denaturing gradient gel electrophoresis (DGGE)
Familial adenomatous polyposis (FAP): Frequency, penetrance, and mutation rate
Identification of rare and novel mutations in the CFTR genes of CF patients in Southern England
A novel mutation (Val-373 to Glu) in the catalytic domain of factor IX, resulting in moderately/severe hemophilia B in a Southern French patient
Editorial
Mutations in PAX3 associated with waardenburg syndrome type I
Screening for CF mutations in adult cystic fibrosis patients with a directed and optimized SSCP strategy
Hb FM-Fort Ripley: Confirmation of autosormal dominant inheritance and diagnosis by PCR and direct nucleotide sequencing
Allele frequency data for VNTR locus D17S79: Identification of an internalHaeIII polymorphism in the black population black population
Single-strand conformation polymorphism (SSCP) analysis of the COL3A1 gene detects a mutation that results in the substitution of glycine 1009 to valine and causes severe Ehlers-Danlos syndrome type I
Loss of heterozygosity on chromosome 5 in sporadic ovarian carcinoma is a late event and is not associated with mutations in APC at 5q21-22
Identification of four new mutations in the cystic fibrosis transmembrane conductance regulator gene: I148T, L1077P, Y1092X, 2183AA→G
Nonisotopic detection of mutations using a modified single-strand conformation polymorphism analysis
Masthead
Characterization of a disease-causing Glu119-Lys mutation in the low-density lipoprotein receptor gene in two Danish families with heterozygous familial hypercholesterolemia
Non-phenylketonuria hyperphenylalaninaemia in Northern Ireland: Frequent mutation allows screening and early diagnosis
Complex arylsulfatase A alleles causing metachromatic leukodystrophy
Rapid and noninvasive screening of patients with mitochondrial myopathy
Genome scanning detects genetic alterations in human ovarian carcinoma
Identification of mutations in four hemophilia B patients of Turkish origin, including a novel deletion of base 6411 of Turkish origin
Discussion on mutation nomenclature
Deletion spanning the 5′ ends of both the COL4A5 and COL4A6 genes in a patient with Alport's syndrome and leiomyomatosis
Homologous loci DXYS156X and DXYS156Y contain a polymorphic pentanucleotide repeat (TAAAA)n and map to human X and Y chromosomes
Identification of two novel mutations in the CFTR gene: 3007 del G and 3271 +1 G→A
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Mutation analysis of Jewish hunter patients in Israel
Marked zinc activation of ester hydrolysis by a mutation, 67-His (CAT) to Arg (CGT), in the active site of human carbonic anhydrase I
Phenylketonuria in Southern Poland: A new splice mutation in intron 9 at the PAH locus
Masthead
Substitution of glycine-172 by arginine in the α1 chain of type I collagen in a patient with osteogenesis imperfecta, type III
Genetic heterogeneity of apolipoprotein E and its influence on plasma lipid and lipoprotein levels
Mutation analysis for cystic fibrosis to determine carrier status in 167 sperm donors from the Nebraska genetic semen bank