Human Mutation - 2007

204 articles | Last updated: 2025-12-03 14:12:57
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Parental origin and somatic mosaicism of PHOX2B mutations in Congenital Central Hypoventilation Syndrome
Recommendations for locus-specific databases and their curation
An unusual case of hemochromatosis due to a new compound heterozygosity in HFE (p.[Gly43Asp;His63Asp]+[Cys282Tyr]): structural implications with respect to binding with transferrin receptor 1
A new cover and new challenges forHuman Mutation
Novel clinico-molecular insights in pseudoxanthoma elasticum provide an efficient molecular screening method and a comprehensive diagnostic flowchart
Molecular analysis ofSUMF1 mutations: stability and residual activity of mutant formylglycine-generating enzyme determine disease severity in multiple sulfatase deficiency
Pathogenic validation of unique germline intronic variants ofRB1 in retinoblastoma patients using minigenes
Improving sequence variant descriptions in mutation databases and literature using the Mutalyzer sequence variation nomenclature checker
Mutations in LCA5 are an uncommon cause of Leber congenital amaurosis (LCA) type II
Germline and somaticNF1 gene mutation spectrum in NF1-associated malignant peripheral nerve sheath tumors (MPNSTs)
Type 2 diabetes-associated fatty acid binding protein 2 promoter haplotypes are differentially regulated by GATA factors
Missense mutations in theTGM2 gene encoding transglutaminase 2 are found in patients with early-onset type 2 diabetes
AVPR1A andOXTR polymorphisms are associated with sexual and reproductive behavioral phenotypes in humans
Use of estimated evolutionary strength at the codon level improves the prediction of disease-related protein mutations in humans
An integrated genetic and functional analysis of the role of type II transmembrane serine proteases (TMPRSSs) in hearing loss
Genetic subtyping of Fanconi anemia by comprehensive mutation screening
GPCR NaVa database: natural variants in human G protein-coupled receptors
A variant of the Cockayne syndrome B geneERCC6 confers risk of lung cancer
Functional analysis of promoter variants in the microsomal triglyceride transfer protein (MTTP) gene
Association of IL4R gene polymorphisms with asthma in Chinese populations
Contribution ofABCC6 genomic rearrangements to the diagnosis of pseudoxanthoma elasticum in French patients
Recurrent inversion with concomitant deletion and insertion events in the coagulation factor VIII gene suggests a new mechanism for X-chromosomal rearrangements causing hemophilia A
Atypical methylmalonic aciduria: frequency of mutations in the methylmalonyl CoA epimerase gene (MCEE)
Lysinuric protein intolerance: update and extended mutation analysis of theSLC7A7 gene
Characterization of the interactions of human ZIC3 mutants with GLI3
The novel humanSHOX allelic variant database
A structured simple form for ordering genetic tests is needed to ensure coupling of clinical detail (phenotype) with DNA variants (genotype) to ensure utility in publication and databases
Future challenges for human mutation research
Representational oligonucleotide microarray analysis (ROMA) and comparison of binning and change-point methods of analysis: application to detection of del22q11.2 (DiGeorge) Syndrome
Five novel mutations in steroidogenic factor 1 (SF1,NR5A1) in 46,XY patients with severe underandrogenization but without adrenal insufficiency
MLPA screening reveals novel subtelomeric rearrangements in holoprosencephaly
Double complex mutations involving<i>F8</i>and<i>FUNDC2</i>caused by distinct break-induced replication
Contribution of<i>BRCA1</i>and<i>BRCA2</i>mutations to inherited ovarian cancer
Sequence variation database for the Wilson disease copper transporter,ATP7B
A cluster of translocation breakpoints in 2q37 is associated with overexpression of NPPC in patients with a similar overgrowth phenotype
Rapid identification of disease-causing mutations using copy number analysis within linkage intervals
The UK National Cancer Research Institute (NCRI) Informatics Initiative: promoting partnership in cancer research
Closely linked<i>cis</i>‐acting modifier of expansion of the CGG repeat in high risk<i>FMR1</i>haplotypes
Visualization of uniparental inheritance, Mendelian inconsistencies, deletions, and parent of origin effects in single nucleotide polymorphism trio data with SNPtrio
Ribosomal protein S17 gene (RPS17) is mutated in Diamond-Blackfan anemia
Multiple sulfatase deficiency is due to hypomorphic mutations of theSUMF1 gene
The importance of mutation detection in Marfan syndrome and Marfan-related disorders: report of 193FBN1 mutations
Chasing genes for mood disorders and schizophrenia in genetically isolated populations
Detection of pathogenic gene copy number variations in patients with mental retardation by genomewide oligonucleotide array comparative genomic hybridization
Lack ofSSH1 mutations in Dutch patients with disseminated superficial actinic porokeratosis: is there really an association?
Association ofSSH1 mutations with disseminated superficial actinic porokeratosis in Chinese pedigrees (response to Frank et al.) Chinese
Multigene deletions on chromosome 20q13.13-q13.2 includingSALL4 result in an expanded phenotype of Okihiro syndrome plus developmental delay
Genetic variant in theHSPB1 promoter region impairs the HSP27 stress response
Sample selection algorithm to improve quality of genotyping from plasma-derived DNA: to separate the wheat from the chaff
Functional polymorphisms and haplotypes in the promoter of the<i>MMP2</i>gene are associated with risk of nasopharyngeal carcinoma
Spectrum of<i>ALMS1</i>variants and evaluation of genotype-phenotype correlations in Alström syndrome
Functional analysis helps to clarify the clinical importance of unclassified variants in DNA mismatch repair genes
p.Gln200Glu, a putative constitutively active mutant of rod α-transducin (GNAT1) in autosomal dominant congenital stationary night blindness
Large spectrum of lissencephaly and pachygyria phenotypes resulting from de novo missense mutations in tubulin alpha 1A (<i>TUBA1A</i>)
Identification of novel<i>ATP7B</i>gene mutations and their functional roles in Korean patients with Wilson disease
Human Mutation bids farewell to co-editor Haig H. Kazazian, Jr.
Haig H. Kazazian, Jr.: a fifteen-year commitment to excellence
Women heterozygous forNALP7/NLRP7 mutations are at risk for reproductive wastage: report of two novel mutations
A single mutation in the GALC gene is responsible for the majority of late onset Krabbe disease patients in the Catania (Sicily, Italy) region
A novel deletion in theFTL gene causes hereditary hyperferritinemia cataract syndrome (HHCS) by alteration of the transcription start site
Effect of heterogeneity on the chromosome 10 risk in late-onset Alzheimer disease
Streamlined assessment of gene variants by high resolution melt profiling utilizing the ornithine transcarbamylase gene as a model system
Heterogeneity of<i>NSD1</i>alterations in 116 patients with Sotos syndrome
Molecular heterogeneity in fetal forms of type II lissencephaly
Polyalanine expansions might not result from unequal crossing-over
Novel biallelic mutations inMSH6 andPMS2 genes: gene conversion as a likely cause ofPMS2 gene inactivation
Centrosomal-ciliary geneCEP290/NPHP6 mutations result in blindness with unexpected sparing of photoreceptors and visual brain: implications for therapy of Leber congenital amaurosis
Polymorphic background of methionine synthase reductase modulates the phenotype of a disease-causing mutation
SEPT9 sequence alternations causing hereditary neuralgic amyotrophy are associated with altered interactions with SEPT4/SEPT11 and resistance to Rho/Rhotekin-signaling
Detection of genomic copy number changes in patients with idiopathic mental retardation by high-resolution X-array-CGH: important role for increased gene dosage of<i>XLMR</i>genes
Mutational spectrum of<i>MYO15A</i>: the large N-terminal extension of myosin XVA is required for hearing
Documentation of inherited disorders and mutation frequencies in the different religious communities in Israel in the Israeli National Genetic Database
Cancer-associated missplicing of exon 4 influences the subnuclear distribution of the DNA replication factor CIZ1
A human-specific mutation leads to the origin of a novel splice form of neuropsin (KLK8), a gene involved in learning and memory
Characterization of a familial t(16;22) balanced translocation associated with congenital cataract leads to identification of a novel gene,TMEM114, expressed in the lens and disrupted by the transloca
Subtelomeric imbalances in phenotypically normal individuals
Mutation database for the galactose-1-phosphate uridyltransferase (GALT) gene
Somatic<i>APC</i>mosaicism: a frequent cause of familial adenomatous polyposis (FAP)
Variants of the<i>ST6GALNAC2</i>promoter influence transcriptional activity and contribute to genetic susceptibility to IgA nephropathy
Array CGH identifies reciprocal 16p13.1 duplications and deletions that predispose to autism and/or mental retardation
Increased progerin expression associated with unusual<i>LMNA</i>mutations causes severe progeroid syndromes
Novel Plexor™ SNP genotyping technology: comparisons with TaqMan® and homogenous MassEXTEND™ MALDI-TOF mass spectrometry
Clinical, biochemical, and mutational spectrum of peroxisomal acyl–coenzyme A oxidase deficiency
Mutational analysis of 105 mucopolysaccharidosis type VI patients
Functional characterization of missense variants in the creatine transporter gene (SLC6A8): improved diagnostic application
Type 1 Gaucher disease: null and hypomorphic novel chitotriosidase mutations-implications for diagnosis and therapeutic monitoring
ThePAH gene, phenylketonuria, and a paradigm shift
Determination of haplotypes from single DNA molecules: a method for single-molecule barcoding
Missense and silent mutations in COL2A1 result in Stickler syndrome but via different molecular mechanisms
Novel mutations in theZEB1 gene identified in Czech and British patients with posterior polymorphous corneal dystrophy
Aberrant splicing is a common mutational mechanism inMKS1, a key player in Meckel-Gruber syndrome
Clinical and mutational profile in spinal muscular atrophy with respiratory distress (SMARD): defining novel phenotypes through hierarchical cluster analysis
Functional profiling of uncommon<i>VCAM1</i>promoter polymorphisms prevalent in African American populations African American populations
Database of somatic mutations in EGFR with analyses revealing indel hotspots but no smoking-associated signature
Mutations and polymorphisms in the human N-acetylglutamate synthase (<i>NAGS</i>) gene
Large-scale population-based metabolic phenotyping of thirteen genetic polymorphisms related to one-carbon metabolism
Progranulin null mutations in both sporadic and familial frontotemporal dementia
Double de novo mutations ofELA2 in cyclic and severe congenital neutropenia
Night blindness-associated mutations in the ligand-binding, cysteine-rich, and intracellular domains of the metabotropic glutamate receptor 6 abolish protein trafficking
Molecular and in silico analyses of the full-length isoform of usherin identify new pathogenic alleles in Usher type II patients
Identification and characterization of a novel RPGR isoform in human retina
Novel mutations in FRMD7 in X-linked congenital nystagmus
Epistatic interactions with a common hypomorphicRET allele in syndromic Hirschsprung disease
The novel p.L1649Q mutation in the<i>SCN1A</i>epilepsy gene is associated with familial hemiplegic migraine: genetic and functional studies
Mutational analysis of the<i>HGSNAT</i>gene in Italian patients with mucopolysaccharidosis IIIC (Sanfilippo C syndrome)
Molecular analysis and characterization of nine novel CTSK mutations in twelve patients affected by pycnodysostosis
Spectrum of<i>MKS1</i>and<i>MKS3</i>mutations in Meckel syndrome: a genotype-phenotype correlation
Allelic heterogeneity of molecular events in human coagulation factor IX in Asian Indians Asian Indians
Novel<i>SLC4A11</i>mutations in patients with recessive congenital hereditary endothelial dystrophy (CHED2)
Mutational analysis of<i>CACNA1G</i>in idiopathic generalized epilepsy
Screening for the<i>BRCA1-ins6kbEx13</i>mutation: potential for misdiagnosis
Effective detection of corrected dystrophin loci in<i>mdx</i>mouse myogenic precursors
Syndrome-causing mutations of the<i>BLM</i>gene in persons in the Bloom's Syndrome Registry
Overexpression of the C-type natriuretic peptide (CNP) is associated with overgrowth and bone anomalies in an individual with balanced t(2;7) translocation
Involvement ofDFNB59 mutations in autosomal recessive nonsyndromic hearing impairment
Heterozygous nonsense mutationSATB2 associated with cleft palate, osteoporosis, and cognitive defects
Pathogenic mutations in Parkinson disease
PKDB: Polycystic Kidney Disease Mutation Database-a gene variant database for autosomal dominant polycystic kidney disease
Interpreting missense variants: comparing computational methods in human disease genes<i>CDKN2A</i>,<i>MLH1</i>,<i>MSH2</i>,<i>MECP2</i>, and tyrosinase (<i>TYR</i>)
Promoter polymorphisms in the<i>MATP</i>(<i>SLC45A2</i>) gene are associated with normal human skin color variation
The 2006 Human Genome Variation Society scientific meeting
Autosomal dominant anhidrotic ectodermal dysplasias at the EDARADD locus
A new variant database for mismatch repair genes associated with Lynch syndrome
Linkage to a known gene but no mutation identified: comprehensive reanalysis ofSPG4 HSP pedigrees reveals large deletions as the sole cause
Mutations other than null mutations producing a pathogenic loss of progranulin in frontotemporal dementia
Spectrum of NPHP6/CEP290 mutations in Leber congenital amaurosis and delineation of the associated phenotype
Retinopathy mutations in the bZIP protein NRL alter phosphorylation and transcriptional activity
PhenCode: connecting ENCODE data with mutations and phenotype
Argininosuccinate lyase deficiency: mutational spectrum in Italian patients and identification of a novel<i>ASL</i>pseudogene
RILM: a web-based resource to aid comparative and functional analysis of the insulin and IGF-1 receptor family
Impact of mutant p53 functional properties on<i>TP53</i>mutation patterns and tumor phenotype: lessons from recent developments in the IARC TP53 database
Loss of the actin regulator HSPC300 results in clear cell renal cell carcinoma protection in Von Hippel-Lindau patients
Extensive in silico analysis of NF1 splicing defects uncovers determinants for splicing outcome upon 5′ splice-site disruption
Highly sensitive DNA detection and point mutation identification: an electrochemical approach based on the combined use of ligase and reverse molecular beacon
Progress in understanding the biology of the human mutagen LINE-1
Elucidation of penetrance variability of a<i>ZIC3</i>mutation in a family with complex heart defects and functional analysis of<i>ZIC3</i>mutations in the first zinc finger domain
Ultraviolet radiation and melanoma: a systematic review and analysis of reported sequence variants
Mutations in the fibrinogen gene cluster accounting for congenital afibrinogenemia: an update and report of 10 novel mutations
Truncating mutation of the<i>DFNB59</i>gene causes cochlear hearing impairment and central vestibular dysfunction
Genetic variants in<i>MGMT</i>and risk of lung cancer in Southeastern Chinese: a haplotype-based analysis Southeastern Chinese
Partial duplication at<i>AZFc</i>on the Y chromosome is a risk factor for impaired spermatogenesis in Han Chinese in Taiwan Han Chinese
Impact of the<i>Alcohol-Dehydrogenase</i>(<i>ADH</i>)<i>1C</i>and<i>ADH1B</i>polymorphisms on drinking behavior in nonalcoholic Japanese Japanese
Novel high-throughput SNP genotyping cosegregation analysis for genetic diagnosis of autosomal recessive retinitis pigmentosa and Leber congenital amaurosis
Classification of missense variants of unknown significance in<i>BRCA1</i>based on clinical and tumor information
Comprehensive evaluation of allele frequency differences of<i>MC1R</i>variants across populations
Extensive gene conversion at the<i>PMS2</i>DNA mismatch repair locus
Patterns of variation in DNA segments upstream of transcription start sites
Signal peptide variants that impair secretion of pancreatic secretory trypsin inhibitor (SPINK1) cause autosomal dominant hereditary pancreatitis
Two classes of low-copy repeats comediate a new recurrent rearrangement consisting of duplication at 8p23.1 and triplication at 8p23.2
Mutations of the<i>RDX</i>gene cause nonsyndromic hearing loss at the<i>DFNB24</i>locus
Deletion of exon 16 of the dystrophin gene is not associated with disease
CAG and CTG repeat polymorphism in exons of human genes shows distinct features at the expandable loci
Low proportion of whole exon deletions causing phenylketonuria in Denmark and Germany
GM1 gangliosidosis: molecular analysis of nine patients and development of an RT-PCR assay for GLB1 gene expression profiling
A proposed molecular diagnostic flowchart for myophosphorylase deficiency (McArdle disease) in blood samples from Spanish patients
Incidence and clinical features of X-linked Cornelia de Lange syndrome due toSMC1L1 mutations
HbVar database of human hemoglobin variants and thalassemia mutations: 2007 update
MID1 mutation screening in a large cohort of Opitz G/BBB syndrome patients: twenty-nine novel mutations identified
Mutation frequencies of X-linked mental retardation genes in families from the EuroMRX consortium
Genotype-phenotype correlations ofKCNJ2 mutations in Japanese patients with Andersen-Tawil syndrome
Conspicuous involvement of desmin tail mutations in diverse cardiac and skeletal myopathies
Identification of seven novel germline mutations in the human E-cadherin (CDH1) Gene
Townes-Brocks syndrome: twenty novelSALL1 mutations in sporadic and familial cases and refinement of theSALL1 hot spot region
Three arginine to cysteine substitutions in the pro-alpha (I)-collagen chain cause Ehlers-Danlos syndrome with a propensity to arterial rupture in early adulthood
Quantitated transcript haplotypes (QTH) of<i>AGTR1</i>, reduced abundance of mRNA haplotypes containing 1166C (rs5186:A&gt;C), and relevance to metabolic syndrome traits
Case-control genetic association study of fibulin-6 (<i>FBLN6</i>or<i>HMCN1</i>) variants in age-related macular degeneration (AMD)
Y-position cysteine substitution in type I collagen (α1(I) R888C/p.R1066C) is associated with osteogenesis imperfecta/Ehlers-Danlos syndrome phenotype
<i>RPGR</i>mutation analysis and disease: an update
Truncation ofNHEJ1 in a patient with polymicrogyria
ZFHX1B mutations in patients with Mowat-Wilson syndrome
Italian Rett database and biobank
Identification and computationally-based structural interpretation of naturally occurring variants of human protein C
A large genomic deletion in thePDHX gene caused by the retrotranspositional insertion of a full-length LINE-1 element
Response to: getting rid of thePMS2 pseudogenes: mission impossible?
Getting rid of thePMS2 pseudogenes: mission impossible?
Allelic loss analysis by denaturing high-performance liquid chromatography and electrospray ionization mass spectrometry
Novel mutations in the small leucine-rich repeat protein/proteoglycan (SLRP) genes in high myopia
Genotype–phenotype correlations in hereditary familial retinoblastoma
Schimke immunoosseous dysplasia: suggestions of genetic diversity
Deletion mapping in Xp21 for patients with complex glycerol kinase deficiency using SNP mapping arrays
The interactive Factor H-atypical hemolytic uremic syndrome mutation database and website: update and integration of membrane cofactor protein and Factor I mutations with structural models
Consortium for osteogenesis imperfecta mutations in the helical domain of type I collagen: regions rich in lethal mutations align with collagen binding sites for integrins and proteoglycans
Deletion of the<i>parkin</i>and<i>PACRG</i>gene promoter in early-onset parkinsonism
Diversity of cystathionine β-synthase haplotypes bearing the most common homocystinuria mutation c.833T&gt;C: a possible role for gene conversion
Prevalence and functionality of paucimorphic and private<i>MC4R</i>mutations in a large, unselected European British population, scanned by meltMADGE European British population
Diversity, parental germline origin, and phenotypic spectrum of de novo<i>HRAS</i>missense changes in Costello syndrome
Transgenic mice carrying theH258N mutation in the gene encoding the β-subunit of phosphodiesterase-6 (PDE6B) provide a model for human congenital stationary night blindness
Detection of ultrarare somatic mutation in the human<i>TP53</i>gene by bidirectional pyrophosphorolysis-activated polymerization allele-specific amplification
Protein- and mRNA-based phenotype-genotype correlations in DMD/BMD with point mutations and molecular basis for BMD with nonsense and frameshift mutations in the DMD gene
Multiexon skipping leading to an artificial DMD protein lacking amino acids from exons 45 through 55 could rescue up to 63% of patients with Duchenne muscular dystrophy
Single base-pair substitutions in exon-intron junctions of human genes: nature, distribution, and consequences for mRNA splicing
Diverse chromosome breakage mechanisms underlie subtelomeric rearrangements, a common cause of mental retardation
Attenuation of disease phenotype through alternative translation initiation in low-penetrance retinoblastoma
Mutational spectrum of the<i>NF2</i>gene: a meta-analysis of 12 years of research and diagnostic laboratory findings
The Italian XLMR bank: a clinical and molecular database
Somatic microindels: analysis in mouse soma and comparison with the human germline
Mutations in the<i>C7orf11</i>(<i>TTDN1</i>) gene in six nonphotosensitive trichothiodystrophy patients: no obvious genotype-phenotype relationships
A common variant located in the 3′UTR of theRET gene is associated with protection from Hirschsprung disease
Spectrum of molecular defects and mutation detection rate in patients with mild and moderate hemophilia A
Mineralocorticoid receptor mutations are the principal cause of renal type 1 pseudohypoaldosteronism
Understanding the recent evolution of the human genome: insights from human-chimpanzee genome comparisons
Genotype-phenotype correlations in von Hippel-Lindau disease
Weak definition of<i>IKBKAP</i>exon 20 leads to aberrant splicing in familial dysautonomia
Two central core disease (CCD) deletions in the C-terminal region of RYR1 alter muscle excitation-contraction (EC) coupling by distinct mechanisms