Journal of Medical Genetics - 2014

111 articles | Last updated: 2025-12-03 14:12:57
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<i>ISCA2</i>mutation causes infantile neurodegenerative mitochondrial disorder
Novel role for non-homologous end joining in the formation of double minutes in methotrexate-resistant colon cancer cells
Homozygous missense mutation in<i>MED25</i>segregates with syndromic intellectual disability in a large consanguineous family
Functionally distinct groups of inherited PTEN mutations in autism and tumour syndromes
Mitochondrial mutations associated with aminoglycoside ototoxicity and hearing loss susceptibility identified by meta-analysis
Mendelian randomisation applied to drug development in cardiovascular disease: a review
Neuropathy target esterase impairments cause Oliver–McFarlane and Laurence–Moon syndromes
<i>CTNND2</i>—a candidate gene for reading problems and mild intellectual disability
<i>WWOX</i>-related encephalopathies: delineation of the phenotypical spectrum and emerging genotype-phenotype correlation
Mutations in<i>SPECC1L</i>, encoding sperm antigen with calponin homology and coiled-coil domains 1-like, are found in some cases of autosomal dominant Opitz G/BBB syndrome
Current novel-gene-finding strategy for autosomal-dominant hypercholesterolaemia needs refinement
Exhaustive methylation analysis revealed uneven profiles of methylation at<i>IGF2/</i>ICR1<i>/H19</i>11p15 loci in Russell Silver syndrome
Next-generation sequencing of nine atrial fibrillation candidate genes identified novel de novo mutations in patients with extreme trait of atrial fibrillation
Pseudogene in cancer: real functions and promising signature
Case-only exome sequencing and complex disease susceptibility gene discovery: study design considerations
<i>EIF4G1</i>is neither a strong nor a common risk factor for Parkinson's disease: evidence from large European cohorts: Table1 large European cohorts
Genomic variations integrated database for<i>MUTYH</i>-associated adenomatous polyposis
Differential increases of specific<i>FMR1</i>mRNA isoforms in premutation carriers
Recent advances in primary ciliary dyskinesia genetics
Mutations in the tricarboxylic acid cycle enzyme, aconitase 2, cause either isolated or syndromic optic neuropathy with encephalopathy and cerebellar atrophy
Intake levels of dietary polyunsaturated fatty acids modify the association between the genetic variation in<i>PCSK5</i>and HDL cholesterol
International requirements for consent in biobank research: qualitative review of research guidelines
Whole exome sequencing in family trios reveals<i>de novo</i>mutations in<i>PURA</i>as a cause of severe neurodevelopmental delay and learning disability
Clinical, histological and genetic characterisation of patients with tubular aggregate myopathy caused by mutations in STIM1
Absent expression of the osteoblast-specific maternally imprinted genes,<i>DLX5</i>and<i>DLX6,</i>causes split hand/split foot malformation type I
Mutation in<i>PLK4</i>, encoding a master regulator of centriole formation, defines a novel locus for primordial dwarfism
<i>OTX2</i>mutations cause autosomal dominant pattern dystrophy of the retinal pigment epithelium
Tumour<i>MLH1</i>promoter region methylation testing is an effective prescreen for Lynch Syndrome (HNPCC)
Clinical interpretation of CNVs with cross-species phenotype data
Factors determining penetrance in familial atypical haemolytic uraemic syndrome
<i>De novo</i>and rare inherited mutations implicate the transcriptional coregulator TCF20/SPBP in autism spectrum disorder
Defining and managing incidental findings in genetic and genomic practice
Targeted gene panel sequencing in children with very early onset inflammatory bowel disease—evaluation and prospective analysis
Rare and low-frequency variants in human common diseases and other complex traits
Exome sequencing identifies<i>SLC17A9</i>pathogenic gene in two Chinese pedigrees with disseminated superficial actinic porokeratosis
Efficient strategy for the molecular diagnosis of intellectual disability using targeted high-throughput sequencing
Titin and desmosomal genes in the natural history of arrhythmogenic right ventricular cardiomyopathy
Moving beyond genetics: is<i>FAM13A</i>a major biological contributor in lung physiology and chronic lung diseases?
Genotype phenotype associations across the voltage-gated sodium channel family
Oculo-auriculo-vertebral spectrum: a review of the literature and genetic update
Correction
The clinical significance of small copy number variants in neurodevelopmental disorders
Genetic heterogeneity in Cornelia de Lange syndrome (CdLS) and CdLS-like phenotypes with observed and predicted levels of mosaicism
Juvenile myelomonocytic leukaemia and Noonan syndrome
Comprehensive genotype–phenotype correlations between<i>NLRP7</i>mutations and the balance between embryonic tissue differentiation and trophoblastic proliferation
Genetic architectures of ADME genes in five Eurasian admixed populations and implications for drug safety and efficacy
A novel missense mutation in<i>CCDC88C</i>activates the JNK pathway and causes a dominant form of spinocerebellar ataxia
Neurodevelopmental disorders associated with dosage imbalance of<i>ZBTB20</i>correlate with the morbidity spectrum of ZBTB20 candidate target genes
Human transgenerational responses to early-life experience: potential impact on development, health and biomedical research
Meta-analysis identifies loci affecting levels of the potential osteoarthritis biomarkers sCOMP and uCTX-II with genome wide significance
Expansion of the clinical phenotype associated with mutations in<i>activity-dependent neuroprotective protein</i>
TRMT10A dysfunction is associated with abnormalities in glucose homeostasis, short stature and microcephaly
Long-term prospective clinical follow-up after<i>BRCA1/2</i>presymptomatic testing:<i>BRCA2</i>risks higher than in adjusted retrospective studies
Clinical utilisation of a rapid low-pass whole genome sequencing technique for the diagnosis of aneuploidy in human embryos prior to implantation
Genetic variants within the second intron of the<i>KCNQ1</i>gene affect CTCF binding and confer a risk of Beckwith–Wiedemann syndrome upon maternal transmission
Mutations in<i>HAO1</i>encoding glycolate oxidase cause isolated glycolic aciduria
Whole exome sequencing of familial hypercholesterolaemia patients negative for<i>LDLR</i>/<i>APOB</i>/<i>PCSK9</i>mutations
A genome-wide association study of serum levels of prostate-specific antigen in the Japanese population
Correction
Correction
High risk of tobacco-related cancers in<i>CDKN2A</i>mutation-positive melanoma families
<i>SLC39A5</i>mutations interfering with the BMP/TGF-β pathway in non-syndromic high myopia
Temple syndrome: improving the recognition of an underdiagnosed chromosome 14 imprinting disorder: an analysis of 51 published cases
Mutations in<i>SETD2</i>cause a novel overgrowth condition
Involvement of the kinesin family members<i>KIF4A</i>and<i>KIF5C</i>in intellectual disability and synaptic function
Brain imaging in mitochondrial respiratory chain deficiency: combination of brain MRI features as a useful tool for genotype/phenotype correlations
Prediction of the age at onset in spinocerebellar ataxia type 1, 2, 3 and 6
A homozygous nonsense CEP250 mutation combined with a heterozygous nonsense C2orf71 mutation is associated with atypical Usher syndrome
Early postzygotic mutations contribute to de novo variation in a healthy monozygotic twin pair
HLA alleles as biomarkers of high-titre neutralising antibodies to interferon-β therapy in multiple sclerosis
A missense mutation in the splicing factor gene<i>DHX38</i>is associated with early-onset retinitis pigmentosa with macular coloboma
Diagnostic criteria for constitutional mismatch repair deficiency syndrome: suggestions of the European consortium ‘Care for CMMRD’ (C4CMMRD)
Pathogenic mutations in<i>GLI2</i>cause a specific phenotype that is distinct from holoprosencephaly
A novel stop mutation in the vascular endothelial growth factor-C gene (<i>VEGFC</i>) results in Milroy-like disease
The PKU Paradox: A Short History of a Genetic Disease
Haplotypes of the inducible nitric oxide synthase gene are strongly associated with exhaled nitric oxide levels in adults: a population-based study
A familial disorder of altered DNA-methylation
A meta-analysis identifies adolescent idiopathic scoliosis association with<i>LBX1</i>locus in multiple ethnic groups
Comprehensive genotyping and clinical characterisation reveal 27 novel NKX2-1 mutations and expand the phenotypic spectrum
<i>ADAP2</i>in heart development: a candidate gene for the occurrence of cardiovascular malformations in NF1 microdeletion syndrome
A blinded international study on the reliability of genetic testing for GGGGCC-repeat expansions in<i>C9orf72</i>reveals marked differences in results among 14 laboratories
Mutations in the enzyme glutathione peroxidase 4 cause Sedaghatian-type spondylometaphyseal dysplasia
<i>RB1</i>mutation spectrum in a comprehensive nationwide cohort of retinoblastoma patients
Variable phenotypic expression of COG6 mutations
Genome-wide association study identifies new disease loci for isolated clubfoot
Expanding the clinical phenotype of COG6 deficiency
A comprehensive association analysis confirms<i>ZMIZ1</i>to be a susceptibility gene for vitiligo in Chinese population
Mutation in<i>KANK2</i>, encoding a sequestering protein for steroid receptor coactivators, causes keratoderma and woolly hair
Expanding the phenotype of mutations in DICER1: mosaic missense mutations in the RNase IIIb domain of<i>DICER1</i>cause GLOW syndrome
Mapping of hepatic expression quantitative trait loci (eQTLs) in a Han Chinese population
Genome-wide association study of smoking behaviours among Bangladeshi adults
CGG allele size somatic mosaicism and methylation in<i>FMR1</i>premutation alleles
<i>VPS53</i>mutations cause progressive cerebello-cerebral atrophy type 2 (PCCA2)
<i>POMK</i>mutation in a family with congenital muscular dystrophy with merosin deficiency, hypomyelination, mild hearing deficit and intellectual disability
Guidelines for surveillance of individuals with constitutional mismatch repair-deficiency proposed by the European Consortium “Care for CMMR-D” (C4CMMR-D)
Heterozygous mutations in the T (brachyury) gene
Correction
Genome-wide DNA methylation analysis of patients with imprinting disorders identifies differentially methylated regions associated with novel candidate imprinted genes
Functional analysis of<i>MSH2</i>unclassified variants found in suspected Lynch syndrome patients reveals pathogenicity due to attenuated mismatch repair
Next generation sequencing of chromosomal rearrangements in patients with split-hand/split-foot malformation provides evidence for<i>DYNC1I1</i>exonic enhancers of<i>DLX5/6</i>expression in humans
Normal and aberrant splicing of<i>LMNA</i>
A splice donor mutation in<i>NAA10</i>results in the dysregulation of the retinoic acid signalling pathway and causes Lenz microphthalmia syndrome
Truncating mutations in<i>TAF4B</i>and<i>ZMYND15</i>causing recessive azoospermia
A novel immunodeficiency syndrome associated with partial trisomy 19p13
Conotruncal malformations and absent thymus due to a deleterious NKX2-6 mutation
Recurrent X chromosome-linked deletions: discovery of new genetic factors in male infertility
A syndrome of congenital hyperinsulinism and rhabdomyolysis is caused by<i>KCNJ11</i>mutation
Prenylation defects in inherited retinal diseases
NECAP1 loss of function leads to a severe infantile epileptic encephalopathy
<i>Methyl-CpG-binding protein 2</i>(<i>MECP2</i>) mutation type is associated with disease severity in Rett syndrome
Whole exome sequencing identifies de novo mutations in<i>GATA6</i>associated with congenital diaphragmatic hernia