Journal of Medical Genetics - 2013

127 articles | Last updated: 2025-12-03 14:12:57
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The novel mitochondrial 16S rRNA 2336T>C mutation is associated with hypertrophic cardiomyopathy
Glycosylphosphatidylinositol (GPI) anchor deficiency caused by mutations in<i>PIGW</i>is associated with West syndrome and hyperphosphatasia with mental retardation syndrome
Mutations in<i>SDHD</i>lead to autosomal recessive encephalomyopathy and isolated mitochondrial complex II deficiency
Direct-to-consumer pharmacogenomic testing is associated with increased physician utilisation
A new scoring system in cancer genetics: application to criteria for<i>BRCA1</i>and<i>BRCA2</i>mutation screening
A genome-wide copy number association study of osteoporotic fractures points to the 6p25.1 locus
<i>KIF1C</i>mutations in two families with hereditary spastic paraparesis and cerebellar dysfunction
Correlation between<i>FMR1</i>expression and clinical phenotype in discordant dichorionic–diamniotic monozygotic twin sisters with the fragile x mutation
The<i>HNF4A</i>R76W mutation causes atypical dominant Fanconi syndrome in addition to a β cell phenotype
Breast and ovarian cancer risks in a large series of clinically ascertained families with a high proportion of BRCA1 and BRCA2 Dutch founder mutations
Ovarian cancer familial relative risks by tumour subtypes and by known ovarian cancer genetic susceptibility variants
A type I interferon signature identifies bilateral striatal necrosis due to mutations in<i>ADAR1</i>
Fanconi anaemia,<i>BRCA2</i>mutations and childhood cancer: a developmental perspective from clinical and epidemiological observations with implications for genetic counselling
Spectrum of<i>RB1</i>mutations identified in 403 retinoblastoma patients
Colorectal cancer risk variants on 11q23 and 15q13 are associated with unexplained adenomatous polyposis
Mutations in the T (brachyury) gene cause a novel syndrome consisting of sacral agenesis, abnormal ossification of the vertebral bodies and a persistent notochordal canal
Different mutations in<i>PDE4D</i>associated with developmental disorders with mirror phenotypes
Combined exome and whole-genome sequencing identifies mutations in<i>ARMC4</i>as a cause of primary ciliary dyskinesia with defects in the outer dynein arm
Paralogue annotation identifies novel pathogenic variants in patients with Brugada syndrome and catecholaminergic polymorphic ventricular tachycardia
A novel heterozygous<i>OPA3</i>mutation located in the mitochondrial target sequence results in altered steady-state levels and fragmented mitochondrial network
3q27.3 microdeletional syndrome: a recognisable clinical entity associating dysmorphic features, marfanoid habitus, intellectual disability and psychosis with mood disorder
Identification of pathogenic gene variants in small families with intellectually disabled siblings by exome sequencing
Somatic point mutations occurring early in development: a monozygotic twin study
Autism traits in the RASopathies
A new face of Borjeson–Forssman–Lehmann syndrome? De novo mutations in<i>PHF6</i>in seven females with a distinct phenotype
Reporting results from whole-genome and whole-exome sequencing in clinical practice: a proposal for Canada?
Genetic variants in<i>CHI3L1</i>influencing YKL-40 levels: resequencing 900 individuals and genotyping 9000 individuals from the general population
<i>CDKN1C</i>mutation affecting the PCNA-binding domain as a cause of familial Russell Silver syndrome
A novel mutation in<i>KIAA0196</i>: identification of a gene involved in Ritscher–Schinzel/3C syndrome in a First Nations cohort
Genome-wide association study of chronic hepatitis B virus infection reveals a novel candidate risk allele on 11q22.3
Association analyses identifying two common susceptibility loci shared by psoriasis and systemic lupus erythematosus in the Chinese Han population Chinese Han population
Novel cases of D-2-hydroxyglutaric aciduria with<i>IDH1</i>or<i>IDH2</i>mosaic mutations identified by amplicon deep sequencing
Genome-wide association study of sex hormones, gonadotropins and sex hormone–binding protein in Chinese men
Mutations in SLC35A3 cause autism spectrum disorder, epilepsy and arthrogryposis
Exome sequencing of Bardet–Biedl syndrome patient identifies a null mutation in the BBSome subunit<i>BBIP1</i>(<i>BBS18</i>)
The busy physician's guide to genetics, genomics and personalized medicine
Deletion of the 5′exons of<i>COL4A6</i>is not needed for the development of diffuse leiomyomatosis in patients with Alport syndrome
Criteria and prediction models for mismatch repair gene mutations: a review
Alternative splicing modifies the effect of mutations in COL11A1 and results in recessive type 2 Stickler syndrome with profound hearing loss
A systematic review on screening for Fabry disease: prevalence of individuals with genetic variants of unknown significance
Victor McKusick and the History of Medical Genetics
CAG size-specific risk estimates for intermediate allele repeat instability in Huntington disease
Delineating the genetic heterogeneity of ALS using targeted high-throughput sequencing
Advances in osteoarthritis genetics: Table 1
Comprehensive molecular diagnosis of 179 Leber congenital amaurosis and juvenile retinitis pigmentosa patients by targeted next generation sequencing
Prevalence of rare mitochondrial DNA mutations in mitochondrial disorders
Confirmation of papillary thyroid cancer susceptibility loci identified by genome-wide association studies of chromosomes 14q13, 9q22, 2q35 and 8p12 in a Chinese population
An overview on molecular biology of KIT/PDGFRA wild type (WT) gastrointestinal stromal tumours (GIST)
Large-scale genotyping identifies a new locus at 22q13.2 associated with female breast size
Bicuspid aortic valve and aortic coarctation are linked to deletion of the X chromosome short arm in Turner syndrome
Support of the histaminergic hypothesis in Tourette Syndrome: association of the histamine decarboxylase gene in a large sample of families
<i>FGFR1</i>mutations cause Hartsfield syndrome, the unique association of holoprosencephaly and ectrodactyly
Can the diagnosis of NF1 be excluded clinically? A lack of pigmentary findings in families with spinal neurofibromatosis demonstrates a limitation of clinical diagnosis
Comparison of the clinical scoring systems in Silver–Russell syndrome and development of modified diagnostic criteria to guide molecular genetic testing
West syndrome, microcephaly, grey matter heterotopia and hypoplasia of corpus callosum due to a novel ARFGEF2 mutation
Advancing genetic testing for deafness with genomic technology
Associations of common variants in<i>HFE</i>and<i>TMPRSS6</i>with iron parameters are independent of serum hepcidin in a general population: a replication study
Genome-wide association study identifies ephrin type A receptors implicated in paclitaxel induced peripheral sensory neuropathy
Targeted sequence capture and GS-FLX Titanium sequencing of 23 hypertrophic and dilated cardiomyopathy genes: implementation into diagnostics
Digenic inheritance in medical genetics
A<i>de novo</i>X;8 translocation creates a<i>PTK2</i>-<i>THOC2</i>gene fusion with<i>THOC2</i>expression knockdown in a patient with psychomotor retardation and congenital cerebellar hypoplasia
Recessive truncating<i>NALCN</i>mutation in infantile neuroaxonal dystrophy with facial dysmorphism
Identification of well-differentiated gene expressions between Han Chinese and Japanese using genome-wide microarray data analysis
<i>CNVinspector:</i>a web-based tool for the interactive evaluation of copy number variations in single patients and in cohorts
<i>CDH1</i>germline mutations and the hereditary diffuse gastric and lobular breast cancer syndrome: a multicentre study
Whole exome sequencing identifies<i>FGF16</i>nonsense mutations as the cause of X-linked recessive metacarpal 4/5 fusion
Refining the role of<i>pms2</i>in Lynch syndrome: germline mutational analysis improved by comprehensive assessment of variants
WNT1 mutation with recessive osteogenesis imperfecta and profound neurological phenotype
New and recurrent gain-of-function<i>STAT1</i>mutations in patients with chronic mucocutaneous candidiasis from Eastern and Central Europe
Loss of function of the E3 ubiquitin-protein ligase UBE3B causes Kaufman oculocerebrofacial syndrome
A novel desmin mutation leading to autosomal recessive limb-girdle muscular dystrophy: distinct histopathological outcomes compared with desminopathies
Next generation diagnostics of cystic fibrosis and<i>CFTR</i>-related disorders by targeted multiplex high-coverage resequencing of<i>CFTR</i>
Disruption of TBC1D7, a subunit of the TSC1-TSC2 protein complex, in intellectual disability and megalencephaly
An X chromosome-wide association analysis identifies variants in<i>GPR174</i>as a risk factor for Graves’ disease
No evidence for locus heterogeneity in Knobloch syndrome
<i>GATAD2B</i>loss-of-function mutations cause a recognisable syndrome with intellectual disability and are associated with learning deficits and synaptic undergrowth in<i>Drosophila</i>
Upregulation of<i>RCAN1</i>causes Down syndrome-like immune dysfunction
Phenotype and genotype in 101 males with X-linked creatine transporter deficiency
A novel intellectual disability syndrome caused by GPI anchor deficiency due to homozygous mutations in<i>PIGT</i>
Mutation in<i>ADAT3</i>, encoding adenosine deaminase acting on transfer RNA, causes intellectual disability and strabismus
A novel syndrome of hypohidrosis and intellectual disability is linked to COG6 deficiency
Risk of thyroid cancer in first-degree relatives of patients with non-medullary thyroid cancer by histology type and age at diagnosis: a joint study from five Nordic countries
<i>TBX4</i>mutations (small patella syndrome) are associated with childhood-onset pulmonary arterial hypertension
Meta-analysis of genome-wide studies identifies<i>MEF2C</i>SNPs associated with bone mineral density at forearm
Troponin activator augments muscle force in nemaline myopathy patients with nebulin mutations
Rhizomelic chondrodysplasia punctata and cardiac pathology
A new seipin-associated neurodegenerative syndrome
Evaluating the performance of the breast cancer genetic risk models BOADICEA, IBIS, BRCAPRO and Claus for predicting<i>BRCA1/2</i>mutation carrier probabilities: a study based on 7352 families from th
Impact of direct-to-consumer genomic testing at long term follow-up
Identification of chromosome 3q28 and<i>ALPK1</i>as susceptibility loci for chronic kidney disease in Japanese individuals by a genome-wide association study
Ovarian cancer among 8005 women from a breast cancer family history clinic: no increased risk of invasive ovarian cancer in families testing negative for<i>BRCA1</i>and<i>BRCA2</i>
Inheritance of autoinflammatory diseases: shifting paradigms and nomenclature
Recent advances in the genetics of sarcoidosis
High rate of mosaicism in individuals with Cornelia de Lange syndrome
Genomic study in Mexicans identifies a new locus for triglycerides and refines European lipid loci European; Mexicans
Correction
Recent advances in the molecular genetics of epilepsy
Genetics of arrhythmogenic right ventricular cardiomyopathy
Exome sequencing identifies<i>DYNC2H1</i>mutations as a common cause of asphyxiating thoracic dystrophy (Jeune syndrome) without major polydactyly, renal or retinal involvement
Mutations in WNT1 are a cause of osteogenesis imperfecta
<i>ARHGDIA</i>: a novel gene implicated in nephrotic syndrome
Exome sequencing identifies mutations in the gene<i>TTC7A</i>in French-Canadian cases with hereditary multiple intestinal atresia French-Canadian
From the periphery to centre stage: de novo single nucleotide variants play a key role in human genetic disease
Genetic complexity in hypertrophic cardiomyopathy revealed by high-throughput sequencing
Correction
Agenesis of corpus callosum and optic nerve hypoplasia due to mutations in<i>SLC25A1</i>encoding the mitochondrial citrate transporter
CFTR p.Arg117His associated with CBAVD and other CFTR-related disorders
Melanoma prone families with<i>CDK4</i>germline mutation: phenotypic profile and associations with<i>MC1R</i>variants
Mutations in<i>POLR3A</i>and<i>POLR3B</i>are a major cause of hypomyelinating leukodystrophies with or without dental abnormalities and/or hypogonadotropic hypogonadism
Whole exome sequencing identifies a mutation for a novel form of corneal intraepithelial dyskeratosis
Meta-analysis identifies a<i>MECOM</i>gene as a novel predisposing factor of osteoporotic fracture
Mutations in<i>TMEM231</i>cause Meckel–Gruber syndrome
Skewed X-inactivation patterns in ageing healthy and myelodysplastic haematopoiesis determined by a pyrosequencing based transcriptional clonality assay
Preimplantation genetic diagnosis in mitochondrial DNA disorders: challenge and success
Early infantile epileptic encephalopathy associated with a high voltage gated calcium channelopathy
Asphyxiating thoracic dysplasia: clinical and molecular review of 39 families
Deletions in 16q24.2 are associated with autism spectrum disorder, intellectual disability and congenital renal malformation
High cumulative risks of cancer in patients with<i>PTEN</i>hamartoma tumour syndrome
Mutations of<i>NANOS1</i>, a human homologue of the<i>Drosophila</i>morphogen, are associated with a lack of germ cells in testes or severe oligo-astheno-teratozoospermia
Heterogeneity of mutational mechanisms and modes of inheritance in auriculocondylar syndrome
Breakpoint mapping by next generation sequencing reveals causative gene disruption in patients carrying apparently balanced chromosome rearrangements with intellectual deficiency and/or congenital mal
Whole-exome sequencing identifies a mutation in the mitochondrial ribosome protein MRPL44 to underlie mitochondrial infantile cardiomyopathy
Genome-wide significant association of<i>ANKRD55</i>rs6859219 and multiple sclerosis risk
Role of<i>PRRT2</i>in common paroxysmal neurological disorders: a gene with remarkable pleiotropy
<i>TBC1D24</i>truncating mutation resulting in severe neurodegeneration