Journal of Medical Genetics - 2010

111 articles | Last updated: 2025-12-03 14:12:57
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T A T A T A T A
The battle of replication fork
Molecular diagnosis for heterogeneous genetic diseases with targeted high-throughput DNA sequencing applied to retinitis pigmentosa
TMEM70 mutations are a common cause of nuclear encoded ATP synthase assembly defect: further delineation of a new syndrome
The inversa type of recessive dystrophic epidermolysis bullosa is caused by specific arginine and glycine substitutions in type VII collagen
CFTR transcription defects in pancreatic sufficient cystic fibrosis patients with only one mutation in the coding region of CFTR
Colorectal cancer susceptibility loci on chromosome 8q23.3 and 11q23.1 as modifiers for disease expression in lynch syndrome
Constitutional mosaic genome-wide uniparental disomy due to diploidisation: an unusual cancer-predisposing mechanism
Cancer and neurologic degeneration in xeroderma pigmentosum: long term follow-up characterises the role of DNA repair
Mosaic trisomy 13: understanding origin using SNP array
Adult-onset hereditary pulmonary alveolar proteinosis caused by a single-base deletion in CSF2RB
Mutation analysis of 18 nephronophthisis associated ciliopathy disease genes using a DNA pooling and next generation sequencing strategy
2q31.1 microdeletion syndrome: redefining the associated clinical phenotype
Genetic architecture of open angle glaucoma and related determinants
CANT1 mutation is also responsible for Desbuquois dysplasia, type 2 and Kim variant
GeneScreen: a program for high-throughput mutation detection in DNA sequence electropherograms
Reduced expression by SETBP1 haploinsufficiency causes developmental and expressive language delay indicating a phenotype distinct from Schinzel-Giedion syndrome
Association of the CBLB gene with multiple sclerosis: new evidence from a replication study in an Italian population Italian population
Germline DICER1 mutations and familial cystic nephroma
CDKN2BAS is associated with periodontitis in different European populations and is activated by bacterial infection European populations
A clinical and molecular genetic study of 112 Iranian families with primary microcephaly
A novel NGF mutation clarifies the molecular mechanism and extends the phenotypic spectrum of the HSAN5 neuropathy
Rare familial 16q21 microdeletions under a linkage peak implicate cadherin 8 (CDH8) in susceptibility to autism and learning disability
A common pattern of brain MRI imaging in mitochondrial diseases with complex I deficiency
Molecular analysis of ring chromosome 20 syndrome reveals two distinct groups of patients
Genetic loci associated with lipid concentrations and cardiovascular risk factors in the Korean population
Quantification of the methylation at the GNAS locus identifies subtypes of sporadic pseudohypoparathyroidism type Ib
Variants in CFTR untranslated regions are associated with congenital bilateral absence of the vas deferens
Adaptor protein complex-4 (AP-4) deficiency causes a novel autosomal recessive cerebral palsy syndrome with microcephaly and intellectual disability
Uptake of breast cancer prevention and screening trials
Correction
Corrections
Germline SMARCB1 mutation and somatic NF2 mutations in familial multiple meningiomas
Multiexon deletions account for 15% of congenital myasthenic syndromes with RAPSN mutations after negative DNA sequencing
Ancestry informative markers for fine-scale individual assignment to worldwide populations
Recurrent microdeletions of 15q25.2 are associated with increased risk of congenital diaphragmatic hernia, cognitive deficits and possibly Diamond-Blackfan anaemia
Cohen syndrome diagnosis using whole genome arrays
Germline mutations in CDH1 are infrequent in women with early-onset or familial lobular breast cancers
Wide spectrum of desmosomal mutations in Danish patients with arrhythmogenic right ventricular cardiomyopathy
Salbutamol increases survival motor neuron (SMN) transcript levels in leucocytes of spinal muscular atrophy (SMA) patients: relevance for clinical trial design
Association between a common mitochondrial DNA D-loop polycytosine variant and alteration of mitochondrial copy number in human peripheral blood cells
Genes that determine immunology and inflammation modify the basic defect of impaired ion conductance in cystic fibrosis epithelia
Mosaicism for oncogenic G12D KRAS mutation associated with epidermal nevus, polycystic kidneys and rhabdomyosarcoma
Mutations in PCDH21 cause autosomal recessive cone-rod dystrophy
BBS10 mutations are common in 'Meckel'-type cystic kidneys
Polymorphisms at 16p13 are associated with systemic lupus erythematosus in the Chinese population Chinese population
A novel HER2-positive breast cancer phenotype arising from germline TP53 mutations
Abnormal respiratory cilia in non-syndromic Leber congenital amaurosis with CEP290 mutations
Oesophageal squamous cell carcinoma in a young adult with IL-12R 1 deficiency
STXBP2 mutations in children with familial haemophagocytic lymphohistiocytosis type 5
FSH dystrophy and a subtelomeric 4q haplotype: a new assay and associations with disease
The severity of phenotype linked to SUCLG1 mutations could be correlated with residual amount of SUCLG1 protein
Epigenotype-phenotype correlations in Silver-Russell syndrome
Developmental delay and connective tissue disorder in four patients sharing a common microdeletion at 6q13-14
The GST domain of GDAP1 is a frequent target of mutations in the dominant form of axonal Charcot Marie Tooth type 2K
Germline APC mutation spectrum derived from 863 genomic variations identified through a 15-year medical genetics service to French patients with FAP
Critical consequences of finding three pathogenic mutations in an individual with recessive disease
WTX mutations can occur both early and late in the pathogenesis of Wilms tumour
Guanosine diphosphate-mannose:GlcNAc2-PP-dolichol mannosyltransferase deficiency (congenital disorders of glycosylation type Ik): five new patients and seven novel mutations
ABCC8 and KCNJ11 molecular spectrum of 109 patients with diazoxide-unresponsive congenital hyperinsulinism
Co-segregation of Norrie disease and idiopathic pulmonary hypertension in a family with a microdeletion of the NDP region at Xp11.3-p11.4
Parthenogenetic chimaerism/mosaicism with a Silver-Russell syndrome-like phenotype
Genetics for the health sciences: a handbook for clinical healthcare
Search for the best indicators for the presence of a VPS13B gene mutation and confirmation of diagnostic criteria in a series of 34 patients genotyped for suspected Cohen syndrome
Identification of an unusual variant peroxisome biogenesis disorder caused by mutations in the PEX16 gene
Methylation of the CpG sites in the myotonic dystrophy locus does not correlate with CTG expansion size or with the congenital form of the disease
Genomic rearrangements of the GREM1-FMN1 locus cause oligosyndactyly, radio-ulnar synostosis, hearing loss, renal defects syndrome and Cenani-Lenz-like non-syndromic oligosyndactyly
MERRF/MELAS overlap syndrome: a double pathogenic mutation in mitochondrial tRNA genes
A dominant mesomelic dysplasia associated with a 1.0-Mb microduplication of HOXD gene cluster at 2q31.1
The c.859G>C variant in the SMN2 gene is associated with types II and III SMA and originates from a common ancestor
Transcobalamin II receptor polymorphisms are associated with increased risk for neural tube defects
Neuroblastoma amplified sequence gene is associated with a novel short stature syndrome characterised by optic nerve atrophy and Pelger-Huet anomaly
Novel and recurrent TRPV4 mutations and their association with distinct phenotypes within the TRPV4 dysplasia family
Identification of clinically significant, submicroscopic chromosome alterations and UPD in fetuses with ultrasound anomalies using genome-wide 250k SNP array analysis
SOD1, ANG, VAPB, TARDBP, and FUS mutations in familial amyotrophic lateral sclerosis: genotype-phenotype correlations
Replication of KIF21B as a susceptibility locus for multiple sclerosis
Multiple cutaneous squamous cell carcinomas in a patient with interferon   receptor 2 (IFN R2) deficiency
The revised Ghent nosology for the Marfan syndrome
Hereditary diffuse gastric cancer: lifesaving total gastrectomy for CDH1 mutation carriers
Current clinical criteria for Lynch syndrome are not sensitive enough to identify MSH6 mutation carriers
Correction
Risk of breast cancer in male BRCA2 carriers
Mutational analysis of the PLCE1 gene in steroid resistant nephrotic syndrome
Hereditary diffuse gastric cancer: updated consensus guidelines for clinical management and directions for future research
Risk of urothelial bladder cancer in Lynch syndrome is increased, in particular among MSH2 mutation carriers
Clinical characterisation of 29 neurofibromatosis type-1 patients with molecularly ascertained 1.4 Mb type-1 NF1 deletions
Germline mutations of the CBL gene define a new genetic syndrome with predisposition to juvenile myelomonocytic leukaemia
Diagnosis, management, and complications of glomus tumours of the digits in neurofibromatosis type 1
Colorectal adenomas and cancer link to chromosome 13q22.1-13q31.3 in a large family with excess colorectal cancer
Mechanisms for variable expressivity of inherited SCN1A mutations causing Dravet syndrome
Novel CENPJ mutation causes Seckel syndrome
LBR mutation and nuclear envelope defects in a patient affected with Reynolds syndrome
Microdeletion at chromosome 4q21 defines a new emerging syndrome with marked growth restriction, mental retardation and absent or severely delayed speech
Genetic variant in the promoter of connective tissue growth factor gene confers susceptibility to nephropathy in type 1 diabetes
Investigation of the Birt-Hogg-Dube tumour suppressor gene (FLCN) in familial and sporadic colorectal cancer
TP53 germline mutation testing in 180 families suspected of Li-Fraumeni syndrome: mutation detection rate and relative frequency of cancers in different familial phenotypes
The BRCA1 c.5434C->G (p.Pro1812Ala) variant induces a deleterious exon 23 skipping by affecting exonic splicing regulatory elements
Functional evaluation of missense variations in the human MAD1L1 and MAD2L1 genes and their impact on susceptibility to lung cancer
Novel mutations in the long isoform of the USH2A gene in patients with Usher syndrome type II or non-syndromic retinitis pigmentosa
Identification of a prevalent founder mutation in an Israeli Muslim Arab village confirms the role of PRCD in the aetiology of retinitis pigmentosa in humans Israeli Muslim Arab village
The carrier clinic: an evaluation of a novel clinic dedicated to the follow-up of BRCA1 and BRCA2 carriers--implications for oncogenetics practice
Mutations in chaperonin-like BBS genes are a major contributor to disease development in a multiethnic Bardet-Biedl syndrome patient population
Nonsense mutation of the stereociliar membrane protein gene PTPRQ in human hearing loss DFNB84
SMARCB1 mutations are not a common cause of multiple meningiomas
BRCA1, BRCA2 and CHEK2 c.1100 delC mutations in patients with double primaries of the breasts and/or ovaries
Further molecular and clinical delineation of co-locating 17p13.3 microdeletions and microduplications that show distinctive phenotypes
Polyalanine expansion in the ZIC3 gene leading to X-linked heterotaxy with VACTERL association: a new polyalanine disorder?
Uncertainties in the classification of human cationic trypsinogen (PRSS1) variants as hereditary pancreatitis-associated mutations
Clinical and genetic spectrum of Birt-Hogg-Dube syndrome patients in whom pneumothorax and/or multiple lung cysts are the presenting feature
A BRCA1 promoter variant (rs11655505) and breast cancer risk
Epigenetic signatures of Silver-Russell syndrome
A novel FTL insertion causing neuroferritinopathy