Journal of Medical Genetics - 2012

120 articles | Last updated: 2025-12-03 14:12:57
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T A T A T A T A
Riboflavin transporter 3 involvement in infantile Brown-Vialetto-Van Laere disease: two novel mutations
Paternal deletion of the 11p15.5 centromeric-imprinting control region is associated with alteration of imprinted gene expression and recurrent severe intrauterine growth restriction
Mutation of HERC2 causes developmental delay with Angelman-like features
Variants of Uncertain Significance in<i>BRCA1</i>and<i>BRCA2</i>assessment of in silico analysis and a proposal for communication in genetic counselling
Hemizygous mutations in SNAP29 unmask autosomal recessive conditions and contribute to atypical findings in patients with 22q11.2DS
Mutation in<i>MPDZ</i>causes severe congenital hydrocephalus
<i>IL12B</i>SNPs and copy number variation in<i>IL23R</i>gene associated with susceptibility to leprosy
Osteogenesis imperfecta type V: marked phenotypic variability despite the presence of the<i>IFITM5</i>c.−14C&gt;T mutation in all patients
Pancreatic cancer risk in Peutz-Jeghers syndrome patients: a large cohort study and implications for surveillance
Human diseases versus mouse models: insights into the regulation of genomic imprinting at the human 11p15/mouse distal chromosome 7 region
High-throughput mutation analysis in patients with a nephronophthisis-associated ciliopathy applying multiplexed barcoded array-based PCR amplification and next-generation sequencing
Corrections
<i>EFTUD2</i>haploinsufficiency leads to syndromic oesophageal atresia
A homozygous mutation of<i>C12orf65</i>causes spastic paraplegia with optic atrophy and neuropathy (SPG55)
Corrections
Identification of a functional variant in the<i>KIF5A-CYP27B1-METTL1-FAM119B</i>locus associated with multiple sclerosis
Whole exome sequencing identified a novel zinc-finger gene<i>ZNF141</i>associated with autosomal recessive postaxial polydactyly type A
Non-heritable genetics of human disease: spotlight on post-zygotic genetic variation acquired during lifetime
An Italian association study and meta-analysis with previous GWAS confirm<i>WNT4</i>,<i>CDKN2BAS</i>and<i>FN1</i>as the first identified susceptibility loci for endometriosis
Comprehensive genomic analyses associate<i>UGT8</i>variants with musical ability in a Mongolian population
Novel<i>KIF7</i>mutations extend the phenotypic spectrum of acrocallosal syndrome
A multi-centre clinico-genetic analysis of the VPS35 gene in Parkinson disease indicates reduced penetrance for disease-associated variants
Risk of malignant paraganglioma in SDHB-mutation and SDHD-mutation carriers: a systematic review and meta-analysis
The<i>DYRK1A</i>gene is a cause of syndromic intellectual disability with severe microcephaly and epilepsy
Exome sequencing identified a missense mutation of<i>EPS8L3</i>in Marie Unna hereditary hypotrichosis
De novo copy number variants are associated with congenital diaphragmatic hernia
Study of autosomal recessive osteogenesis imperfecta in Arabia reveals a novel locus defined by<i>TMEM38B</i>mutation
Corrections
Rare variants in XRCC2 as breast cancer susceptibility alleles: Table 1
A 600 kb deletion syndrome at 16p11.2 leads to energy imbalance and neuropsychiatric disorders
A balanced translocation truncates Neurotrimin in a family with intracranial and thoracic aortic aneurysm
Use of targeted exome sequencing as a diagnostic tool for Familial Hypercholesterolaemia
Distinct and replicable genetic risk factors for acute respiratory distress syndrome of pulmonary or extrapulmonary origin
Corrections
Epigenetic state and expression of imprinted genes in umbilical cord correlates with growth parameters in human pregnancy
Co-occurrence of recurrent duplications of the DiGeorge syndrome region on both chromosome 22 homologues due to inherited and de novo events
Two novel CCDC88C mutations confirm the role of DAPLE in autosomal recessive congenital hydrocephalus
The speech gene<i>FOXP2</i>is not imprinted
SNVerGUI: a desktop tool for variant analysis of next-generation sequencing data
Antenatal spectrum of CHARGE syndrome in 40 fetuses with<i>CHD7</i>mutations
Mutations in<i>TMEM231</i>cause Joubert syndrome in French Canadians
Multiple sequence variants of<i>BRCA2</i>exon 7 alter splicing regulation
Dominantly inherited diabetes mellitus caused by GATA6 haploinsufficiency: variable intrafamilial presentation
Exome sequencing identifies a<i>COL14A1</i>mutation in a large Chinese pedigree with punctate palmoplantar keratoderma
Closing the case of<i>APOE</i>in multiple sclerosis: no association with disease risk in over 29 000 subjects: Figure 1
Gene expression as a quantitative trait: what about translation?
Correction
Prediction of breast cancer risk by genetic risk factors, overall and by hormone receptor status
Deletion of the 3q26 region including the EVI1 and MDS1 genes in a neonate with congenital thrombocytopenia and subsequent aplastic anaemia
Recessive germline<i>SDHA</i>and<i>SDHB</i>mutations causing leukodystrophy and isolated mitochondrial complex II deficiency
Complex I deficiency: clinical features, biochemistry and molecular genetics
Miglustat as a therapeutic agent: prospects and caveats
X-exome sequencing identifies a<i>HDAC8</i>variant in a large pedigree with X-linked intellectual disability, truncal obesity, gynaecomastia, hypogonadism and unusual face
Quantitative trait locus analysis for next-generation sequencing with the functional linear models
A new mutation resulting in the truncation of the TRAF6-interacting domain of XEDAR: a possible novel cause of hypohidrotic ectodermal dysplasia: Figure 1
Neurofibromatosis type 1: from genotype to phenotype
Public funding for genomics: where does Canada stand?
BRCA1 R1699Q variant displaying ambiguous functional abrogation confers intermediate breast and ovarian cancer risk
A systematic review of associated structural and chromosomal defects in oral clefts: when is prenatal genetic analysis indicated?
Clinical significance of copy number variations in the 11p15.5 imprinting control regions: new cases and review of the literature
<i>PAPSS2</i>mutations cause autosomal recessive brachyolmia
Targeted high-throughput sequencing for diagnosis of genetically heterogeneous diseases: efficient mutation detection in Bardet-Biedl and Alström Syndromes
Genotypic and phenotypic analysis of 396 individuals with mutations in<i>Sonic Hedgehog</i>
Identification of the first recurrent PAR1 deletion in Léri-Weill dyschondrosteosis and idiopathic short stature reveals the presence of a novel<i>SHOX</i>enhancer
Homozygous deletion of Tenascin-R in a patient with intellectual disability
Targeting branch sites of new exons?
Microduplications upstream of<i>MSX2</i>are associated with a phenocopy of cleidocranial dysplasia
A founder mutation in Vps37A causes autosomal recessive complex hereditary spastic paraparesis
Germline<i>DICER1</i>mutation and associated loss of heterozygosity in a pineoblastoma: Figure 1
wANNOVAR: annotating genetic variants for personal genomes via the web
Translational genomic medicine: common metabolic traits and ancestral components of Mexican Americans
Rare<i>TP53</i>genetic variant associated with glioma risk and outcome
Novel mutations consolidate<i>KCTD7</i>as a progressive myoclonus epilepsy gene
Intragenic<i>CAMTA1</i>rearrangements cause non-progressive congenital ataxia with or without intellectual disability
Delineation of<i>CCDC39/CCDC40</i>mutation spectrum and associated phenotypes in primary ciliary dyskinesia
A Finnish founder mutation in<i>RAD51D</i>: analysis in breast, ovarian, prostate, and colorectal cancer: Table 1
Mutation in<i>RAB33B</i>, which encodes a regulator of retrograde Golgi transport, defines a second Dyggve–Melchior–Clausen locus
Germline mutations in the DNA damage response genes<i>BRCA1</i>,<i>BRCA2</i>,<i>BARD1</i>and<i>TP53</i>in patients with therapy related myeloid neoplasms
Indication for<i>CDKN2A</i>-mutation analysis in familial pancreatic cancer families without melanomas
Further delineation of the phenotype of chromosome 14q13 deletions: (positional) involvement of<i>FOXG1</i>appears the main determinant of phenotype severity, with no evidence for a holoprosencephaly
Clinical application of exome sequencing in undiagnosed genetic conditions
Disruption of RAB40AL function leads to Martin–Probst syndrome, a rare X-linked multisystem neurodevelopmental human disorder
Mutations in<i>WNT10A</i>are present in more than half of isolated hypodontia cases
A novel defect of peroxisome division due to a homozygous non-sense mutation in the<i>PEX11β</i>gene
Prostate cancer risk assessment model: a scoring model based on the Swedish Family-Cancer Database
A human laterality disorder associated with recessive<i>CCDC11</i>mutation
Whole exome sequencing identifies a splicing mutation in <i>NSUN2</i> as a cause of a Dubowitz-like syndrome
The immunogenetics of immune dysregulation, polyendocrinopathy, enteropathy, X linked (IPEX) syndrome
First PEX11β patient extends spectrum of peroxisomal biogenesis disorder phenotypes: Table 1
A paradigm shift in the delivery of services for diagnosis of inherited retinal disease
<i>OTX2</i>mutations contribute to the otocephaly-dysgnathia complex
Exome sequencing identifies mutations in<i>LZTFL1</i>, a BBSome and smoothened trafficking regulator, in a family with Bardet–Biedl syndrome with situs inversus and insertional polydactyly
Mutations in<i>GRIP1</i>cause Fraser syndrome
Genomic analysis of mitochondrial diseases in a consanguineous population reveals novel candidate disease genes
The Human Variome Project Beijing meeting
Phenotype difference between ALS patients with expanded repeats in<i>C9ORF72</i>and patients with mutations in other ALS-related genes
A recessive form of Marshall syndrome is caused by a mutation in the<i>COL11A1</i>gene: Figure 1
An<i>SNX10</i>mutation causes malignant osteopetrosis of infancy
<i>NEK1</i>and<i>DYNC2H1</i>are both involved in short rib polydactyly Majewski type but not in Beemer Langer cases
Identification of<i>YAP1</i>as a novel susceptibility gene for polycystic ovary syndrome
Genome-wide linkage and copy number variation analysis reveals 710 kb duplication on chromosome 1p31.3 responsible for autosomal dominant omphalocele
Keratinocytic epidermal nevi are associated with mosaic<i>RAS</i>mutations
Recessive mutations in<i>MCM4</i>/<i>PRKDC</i>cause a novel syndrome involving a primary immunodeficiency and a disorder of DNA repair
Molecular diagnosis in mitochondrial complex I deficiency using exome sequencing
Shorter telomere length is associated with increased ovarian cancer risk in both familial and sporadic cases
Correction
Haploinsufficiency of<i>COQ4</i>causes coenzyme Q<sub>10</sub>deficiency
Single nucleotide polymorphisms in the<i>NOS2</i>and<i>NOS3</i>genes are associated with exhaled nitric oxide
Mutations in<i>DYNC1H1</i>cause severe intellectual disability with neuronal migration defects
Correlation of tumour BRAF mutations and<i>MLH1</i>methylation with germline mismatch repair (MMR) gene mutation status: a literature review assessing utility of tumour features for MMR variant classi
<i>CDKN2A</i>is the main susceptibility gene in Italian pancreatic cancer families
Life expectancy in hereditary cancer predisposing diseases: an observational study
Promoter methylation in coagulation<i>F7</i>gene influences plasma FVII concentrations and relates to coronary artery disease
Fabry International Prognostic Index: a predictive severity score for Anderson-Fabry disease
Mutations in<i>NLRP7</i>are associated with diploid biparental hydatidiform moles, but not androgenetic complete moles
Thyroid cancer susceptibility polymorphisms: confirmation of loci on chromosomes 9q22 and 14q13, validation of a recessive 8q24 locus and failure to replicate a locus on 5q24
Genotype–phenotype correlation in<i>CC2D2A</i>-related Joubert syndrome reveals an association with ventriculomegaly and seizures
Unique author identifier; what are we waiting for?
Exome sequencing reveals a novel Fanconi group defined by XRCC2 mutation: Figure 1
Facioscapulohumeral muscular dystrophy: new insights from compound heterozygotes and implication for prenatal genetic counselling