Journal of Medical Genetics - 2011

133 articles | Last updated: 2025-12-03 14:12:57
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T A T A T A T A
<i>NOTCH2</i>mutations in Alagille syndrome
Mutations in<i>PRRT2</i>result in paroxysmal dyskinesias with marked variability in clinical expression
Mutation screening of 75 candidate genes in 152 complex I deficiency cases identifies pathogenic variants in 16 genes including<i>NDUFB9</i>
A bias-reducing pathway enrichment analysis of genome-wide association data confirmed association of the MHC region with schizophrenia
Replication of association of<i>DENND1A</i>and<i>THADA</i>variants with polycystic ovary syndrome in European cohorts European cohorts
Further clinical and molecular delineation of the 15q24 microdeletion syndrome
A novel microdeletion syndrome at 3q13.31 characterised by developmental delay, postnatal overgrowth, hypoplastic male genitals, and characteristic facial features
Complete loss of expression of the<i>ANT1</i>gene causing cardiomyopathy and myopathy
<i>CHRNG</i>genotype–phenotype correlations in the multiple pterygium syndromes
Phenotypic spectrum of the SMAD3-related aneurysms–osteoarthritis syndrome
Duplications of<i>BHLHA9</i>are associated with ectrodactyly and tibia hemimelia inherited in non-Mendelian fashion
A genome-wide association study of men with symptoms of testicular dysgenesis syndrome and its network biology interpretation
Comprehensive sequence analysis of nine Usher syndrome genes in the UK National Collaborative Usher Study
Targeted genomic sequencing identifies<i>PRRT2</i>mutations as a cause of paroxysmal kinesigenic choreoathetosis
Identification of a novel<i>DLX5</i>mutation in a family with autosomal recessive split hand and foot malformation
Neonatal onset autosomal dominant polycystic kidney disease (ADPKD) in a patient homozygous for a<i>PKD2</i>missense mutation due to uniparental disomy
Defective NDUFA9 as a novel cause of neonatally fatal complex I disease
CHEK2*1100delC homozygosity is associated with a high breast cancer risk in women
Genetic basis of pain variability: recent advances
Linkage and association analyses of glaucoma related traits in a large pedigree from a Dutch genetically isolated population
Sex differences in reproductive fitness contribute to preferential maternal transmission of 22q11.2 deletions
Disruption of a long distance regulatory region upstream of SOX9 in isolated disorders of sex development
Pathogenic aberrations revealed exclusively by single nucleotide polymorphism (SNP) genotyping data in 5000 samples tested by molecular karyotyping
Chromosome 1p21.3 microdeletions comprising DPYD and MIR137 are associated with intellectual disability
Int22h-1/int22h-2-mediated Xq28 rearrangements: intellectual disability associated with duplications and in utero male lethality with deletions
Association between deletion size and important phenotypes expands the genomic region of interest in Phelan-McDermid syndrome (22q13 deletion syndrome)
Histone acetylation deficits in lymphoblastoid cell lines from patients with Rubinstein–Taybi syndrome
Patients with a phenotype consistent with facioscapulohumeral muscular dystrophy display genetic and epigenetic heterogeneity
Fetal akinesia: review of the genetics of the neuromuscular causes
Clinical validity of karyotyping for the diagnosis of chromosomal imbalance following array comparative genomic hybridisation
Clinical impact of unclassified variants of the BRCA1 and BRCA2 genes
De novo copy number variants associated with intellectual disability have a paternal origin and age bias
Genetic diagnosis of Duchenne and Becker muscular dystrophy using next-generation sequencing technology: comprehensive mutational search in a single platform
Phenotypic spectrum associated with CASK loss-of-function mutations
Germline BAP1 mutation predisposes to uveal melanoma, lung adenocarcinoma, meningioma, and other cancers
New findings for phenotype-genotype correlations in a large European series of holoprosencephaly cases
Allelic hierarchy of CDH23 mutations causing non-syndromic deafness DFNB12 or Usher syndrome USH1D in compound heterozygotes
New clinical genetics--2nd edition
Two distinct thyroid tumours in a patient with Cowden syndrome carrying both a 10q23 and a mitochondrial DNA germline deletion
Setleis syndrome in Mexican-Nahua sibs due to a homozygous TWIST2 frameshift mutation and partial expression in heterozygotes: review of the focal facial dermal dysplasias and subtype reclassification Mexican-Nahua
Mutations in the mitochondrial complex I assembly factor NDUFAF1 cause fatal infantile hypertrophic cardiomyopathy
Reversible infantile respiratory chain deficiency is a unique, genetically heterogenous mitochondrial disease
Maternally transmitted late-onset non-syndromic deafness is associated with the novel heteroplasmic T12201C mutation in the mitochondrial tRNAHis gene
Lessons from predictive testing for Huntington disease: 25 years on
The rs12975333 variant in the miR-125a and breast cancer risk in Germany, Italy, Australia and Spain
7q21-rs6964587 and breast cancer risk: an extended case-control study by the Breast Cancer Association Consortium
Mitochondrial DNA polymerase   mutations: an ever expanding molecular and clinical spectrum
Autosomal recessive cerebellar ataxias: the current state of affairs
A new ocular phenotype associated with an unexpected but known systemic disorder and mutation: novel use of genomic diagnostics and exome sequencing
Identification of ADAMTS18 as a gene mutated in Knobloch syndrome
Exome diagnostics: already a reality?
Studying the epigenome using next generation sequencing
Association of HFE and TMPRSS6 genetic variants with iron and erythrocyte parameters is only in part dependent on serum hepcidin concentrations
Combined malonic and methylmalonic aciduria: exome sequencing reveals mutations in the ACSF3 gene in patients with a non-classic phenotype
Novel inborn error of folate metabolism: identification by exome capture and sequencing of mutations in the MTHFD1 gene in a single proband
Association of a genetic variant of BTN2A1 with metabolic syndrome in East Asian populations
Correction
Fibroblast growth factor 10 haploinsufficiency causes chronic obstructive pulmonary disease
The FOXE1 and NKX2-1 loci are associated with susceptibility to papillary thyroid carcinoma in the Japanese population
What can exome sequencing do for you?
Acetazolamide-responsive exercise-induced episodic ataxia associated with a novel homozygous DARS2 mutation
Identification of quantitative trait loci for murine autoimmune pancreatitis
A RaDiCAL approach to gene discovery
Biallelic MLH1 SNP cDNA expression or constitutional promoter methylation can hide genomic rearrangements causing Lynch syndrome
Efficiency of translation termination in humans is highly dependent upon nucleotides in the neighbourhood of a (premature) termination codon
Linkage and association analysis of hyperthyrotropinaemia in an Alpine population reveal two novel loci on chromosomes 3q28-29 and 6q26-27
Prevalence of BRCA1 and BRCA2 mutations in triple negative breast cancer
XX males SRY negative: a confirmed cause of infertility
Detection of APC germ line mosaicism in patients with de novo familial adenomatous polyposis: a plea for the protein truncation test
Predicting PTEN mutations: an evaluation of Cowden syndrome and Bannayan-Riley-Ruvalcaba syndrome clinical features
NLRP7 in the spectrum of reproductive wastage: rare non-synonymous variants confer genetic susceptibility to recurrent reproductive wastage
Genetic analysis of inherited bone marrow failure syndromes from one prospective, comprehensive and population-based cohort and identification of novel mutations
Replacement of the myotonic dystrophy type 1 CTG repeat with 'non-CTG repeat' insertions in specific tissues
A role for XRCC2 gene polymorphisms in breast cancer risk and survival
Determining the frequency of de novo germline mutations in DNA mismatch repair genes
Microtriplication of 11q24.1: a highly recognisable phenotype with short stature, distinctive facial features, keratoconus, overweight, and intellectual disability
Respiratory chain complex I deficiency due to NDUFA12 mutations as a new cause of Leigh syndrome
Variants in or near KITLG, BAK1, DMRT1, and TERT-CLPTM1L predispose to familial testicular germ cell tumour
Array comparative genomic hybridisation on first polar bodies suggests that non-disjunction is not the predominant mechanism leading to aneuploidy in humans
Correction
MEIS1 and BTBD9: genetic association with restless leg syndrome in end stage renal disease
Accounting for genetic heterogeneity in homozygosity mapping: application to Mendelian susceptibility to mycobacterial disease
Genome-wide linkage and association mapping identify susceptibility alleles in ABCC4 for Kawasaki disease
The 57 kb deletion in cystinosis patients extends into TRPV1 causing dysregulation of transcription in peripheral blood mononuclear cells
Special issue on structural genomic alterations: ready for prime time
Genomic alterations that contribute to the development of isolated and non-isolated congenital diaphragmatic hernia
Manitoba-oculo-tricho-anal (MOTA) syndrome is caused by mutations in FREM1
FOXN1 mutation abrogates prenatal T-cell development in humans
Multiple congenital anomalies-hypotonia-seizures syndrome is caused by a mutation in PIGN
Hearing function and thresholds: a genome-wide association study in European isolated populations identifies new loci and pathways European isolated populations
The genetics of panic disorder
RET and GDNF mutations are rare in fetuses with renal agenesis or other severe kidney development defects
Revisiting genome wide association studies (GWAS) in coeliac disease: replication study in Spanish population and expression analysis of candidate genes
Correction
Association of HLA class I markers with multiple sclerosis in the Italian and UK population: evidence of two independent protective effects
The core FOXG1 syndrome phenotype consists of postnatal microcephaly, severe mental retardation, absent language, dyskinesia, and corpus callosum hypogenesis
Euchromatic 9q13-q21 duplication variants are tandem segmental amplifications of sequence reciprocal to 9q13-q21 deletions
Unexpected findings in cancer predisposition genes detected by array comparative genomic hybridisation: what are the issues?
Germline PALB2 mutation analysis in breast-pancreas cancer families
Molecular screening of ADAMTSL2 gene in 33 patients reveals the genetic heterogeneity of geleophysic dysplasia
Screening patients referred to a metabolic clinic for lysosomal storage disorders
Rapid detection of a mutation causing X-linked leucoencephalopathy by exome sequencing
Decreased dyskerin levels as a mechanism of telomere shortening in X-linked dyskeratosis congenita
Identification of clinically relevant mosaicism in type I hereditary haemorrhagic telangiectasia
Novel FH mutations in families with hereditary leiomyomatosis and renal cell cancer (HLRCC) and patients with isolated type 2 papillary renal cell carcinoma
Kearns-Sayre syndrome caused by defective R1/p53R2 assembly
C14ORF179 encoding IFT43 is mutated in Sensenbrenner syndrome
CHD7 mutations and CHARGE syndrome: the clinical implications of an expanding phenotype
Mosaic ACVRL1 and ENG mutations in hereditary haemorrhagic telangiectasia patients
Spinocerebellar ataxia type 15: diagnostic assessment, frequency, and phenotypic features
Gastrointestinal polyps in McCune Albright syndrome
Molecular mechanisms of phenotypic variability in junctional epidermolysis bullosa
Sweating ability and genotype in individuals with X-linked hypohidrotic ectodermal dysplasia
A 4.6 kb genomic duplication on 20p12.2-12.3 is associated with brachydactyly type A2 in a Chinese family
Case series: 2q33.1 microdeletion syndrome--further delineation of the phenotype
The power of high-resolution non-targeted array-CGH in identifying intragenic rearrangements responsible for Cohen syndrome
Correction
Melanoma risk for CDKN2A mutation carriers who are relatives of population-based case carriers in Australia and the UK
Correction
A homozygous nonsense mutation (c.214C-&gt;A) in the biliverdin reductase alpha gene (BLVRA) results in accumulation of biliverdin during episodes of cholestasis
A molecular analysis of individuals with neurofibromatosis type 1 (NF1) and optic pathway gliomas (OPGs), and an assessment of genotype-phenotype correlations
High incidence of recurrent copy number variants in patients with isolated and syndromic Mullerian aplasia
Cranial meningiomas in 411 neurofibromatosis type 2 (NF2) patients with proven gene mutations: clear positional effect of mutations, but absence of female severity effect on age at onset
Deletions and rearrangements of the H19/IGF2 enhancer region in patients with Silver-Russell syndrome and growth retardation
LRPPRC mutations cause a phenotypically distinct form of Leigh syndrome with cytochrome c oxidase deficiency
DICER1 syndrome: clarifying the diagnosis, clinical features and management implications of a pleiotropic tumour predisposition syndrome
Clinical features distinguish childhood chordoma associated with tuberous sclerosis complex (TSC) from chordoma in the general paediatric population
Rapid identification of mutations in GJC2 in primary lymphoedema using whole exome sequencing combined with linkage analysis with delineation of the phenotype
Genotype-phenotype study of familial haemophagocytic lymphohistiocytosis type 3
What did we learn from the genome-wide association study for tuberculosis susceptibility?
Rapid detection of genetic variants in hypertrophic cardiomyopathy by custom DNA resequencing array in clinical practice
Chromosome fragility in patients with Fanconi anaemia: diagnostic implications and clinical impact
Sequence analysis of the shelterin telomere protection complex genes in dyskeratosis congenita