Journal of Medical Genetics - 2009

159 articles | Last updated: 2025-12-03 14:12:57
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T A T A T A T A
FCGR2A functional genetic variant associated with susceptibility to severe malarial anaemia in Ghanaian children
Phenotype-genotype correlation in a familial IGF1R microdeletion case
Functional evidence implicating a novel TOR1A mutation in idiopathic, late-onset focal dystonia
Mutations in ZIC2 in human holoprosencephaly: description of a Novel ZIC2 specific phenotype and comprehensive analysis of 157 individuals
Correction
Correction
Correction
Genomic microarrays in mental retardation: from copy number variation to gene, from research to diagnosis
Non-recurrent SEPT9 duplications cause hereditary neuralgic amyotrophy
Constitutional translocation breakpoint mapping by genome-wide paired-end sequencing identifies HACE1 as a putative Wilms tumour susceptibility gene
Assisted reproductive technologies do not enhance the variability of DNA methylation imprints in human
Comprehensive genotype-phenotype analysis in 230 patients with tetralogy of Fallot
Updating the profile of C-terminal MECP2 deletions in Rett syndrome
Information for genetic management of mtDNA disease: sampling pathogenic mtDNA mutants in the human germline and in placenta
Recurrent reciprocal 16p11.2 rearrangements associated with global developmental delay, behavioural problems, dysmorphism, epilepsy, and abnormal head size
Partial 7q11.23 deletions further implicate GTF2I and GTF2IRD1 as the main genes responsible for the Williams-Beuren syndrome neurocognitive profile
Phenotypic spectrum of MFN2 mutations in the Spanish population
Mutations of FUS gene in sporadic amyotrophic lateral sclerosis
An update on age related mosaic and offspring risk in neurofibromatosis 2 (NF2)
Karyomapping: a universal method for genome wide analysis of genetic disease based on mapping crossovers between parental haplotypes
Coexistence of two different pseudohypoparathyroidism subtypes (Ia and Ib) in the same kindred with independent Gs  coding mutations and GNAS imprinting defects
Unexpected myopathy associated with a mutation in MYBPC3 and misplacement of the cardiac myosin binding protein C
Clinical and molecular characterisation of Bardet-Biedl syndrome in consanguineous populations: the power of homozygosity mapping
Genotype-phenotype correlations in L1 syndrome: a guide for genetic counselling and mutation analysis
Clinical and cellular characterisation of Hermansky-Pudlak syndrome type 6
Disruption of ST5 is associated with mental retardation and multiple congenital anomalies
A case of Beckwith-Wiedemann syndrome caused by a cryptic 11p15 deletion encompassing the centromeric imprinted domain of the BWS locus
Renal tumour suppressor function of the Birt-Hogg-Dube syndrome gene product folliculin
Incomplete penetrance of the predisposition to medulloblastoma associated with germ-line SUFU mutations
Multiple single nucleotide polymorphisms in the human urate transporter 1 (hURAT1) gene are associated with hyperuricaemia in Han Chinese
Duplications of the critical Rubinstein-Taybi deletion region on chromosome 16p13.3 cause a novel recognisable syndrome
A novel splice variant of the DNA-PKcs gene is associated with clinical and cellular radiosensitivity in a patient with xeroderma pigmentosum
Screening for Fabry disease in high-risk populations: a systematic review
Identification of 11 novel mutations in eight BBS genes by high-resolution homozygosity mapping
A gain-of-function TBX20 mutation causes congenital atrial septal defects, patent foramen ovale and cardiac valve defects
Submicroscopic chromosomal imbalances in idiopathic Silver-Russell syndrome (SRS): the SRS phenotype overlaps with the 12q14 microdeletion syndrome
Array comparative genomic hybridisation of 52 subjects with a Smith-Magenis-like phenotype: identification of dosage sensitive loci also associated with schizophrenia, autism, and developmental delay
Epilepsy and mental retardation limited to females with PCDH19 mutations can present de novo or in single generation families
A cohort study of recurrence patterns among more than 54 000 relatives of oral cleft cases in Denmark: support for the multifactorial threshold model of inheritance
Small-molecule signal-transduction inhibitors: targeted therapeutic agents for single-gene disorders
A triplication of the Williams-Beuren syndrome region in a patient with mental retardation, a severe expressive language delay, behavioural problems and dysmorphisms
Submicroscopic genomic alterations in Silver-Russell syndrome and Silver-Russell-like patients
Heritability of metabolic syndrome traits among healthy younger adults: a population based study in China
Can our understanding of epigenetics assist with primary prevention of congenital defects?
Scalp fibroblasts have a shared expression profile in monogenic craniosynostosis
Phenotypic spectrum associated with de novo and inherited deletions and duplications at 16p11.2 in individuals ascertained for diagnosis of autism spectrum disorder
Associations of folate and choline metabolism gene polymorphisms with orofacial clefts
MECP2 mutation in one of Rett's original patients
Fragile X syndrome: from molecular genetics to therapy
What is paradominant inheritance?
A newly recognised microdeletion syndrome of 2p15-16.1 manifesting moderate developmental delay, autistic behaviour, short stature, microcephaly, and dysmorphic features: a new patient with 3.2 Mb del
Mutation in IFT80 in a fetus with the phenotype of Verma-Naumoff provides molecular evidence for Jeune-Verma-Naumoff dysplasia spectrum
Correction
Correction
"New microdeletion syndromes: complex, but no new paradigms"
A functional haplotype variant in the TBX22 promoter is associated with cleft palate and ankyloglossia
Molecular analysis of pericentrin gene (PCNT) in a series of 24 Seckel/microcephalic osteodysplastic primordial dwarfism type II (MOPD II) families
De novo apparently balanced translocations in man are predominantly paternal in origin and associated with a significant increase in paternal age
Deletion of YWHAE in a patient with periventricular heterotopias and pronounced corpus callosum hypoplasia
Survival in women with MMR mutations and ovarian cancer: a multicentre study in Lynch syndrome kindreds
Phenomic determinants of genomic variation in autism spectrum disorders
Differences in SMN1 allele frequencies among ethnic groups within North America
Breast cancer susceptibility variants alter risks in familial disease
Phenotype and genotype in mucolipidoses II and III alpha/beta: a study of 61 probands
DARS2 mutations in mitochondrial leucoencephalopathy and multiple sclerosis
MEF2C haploinsufficiency caused by either microdeletion of the 5q14.3 region or mutation is responsible for severe mental retardation with stereotypic movements, epilepsy and/or cerebral malformations
Microdeletions including YWHAE in the Miller-Dieker syndrome region on chromosome 17p13.3 result in facial dysmorphisms, growth restriction, and cognitive impairment
Distal limb deficiencies, micrognathia syndrome, and syndromic forms of split hand foot malformation (SHFM) are caused by chromosome 10q genomic rearrangements
ABCB4 gene mutations and single-nucleotide polymorphisms in women with intrahepatic cholestasis of pregnancy
OPA1 increases the risk of normal but not high tension glaucoma
Association between a polymorphism in the human programmed death-1 (PD-1) gene and cytomegalovirus infection after kidney transplantation
De novo SCN1A mutations in Dravet syndrome and related epileptic encephalopathies are largely of paternal origin
Physicochemical property changes of amino acid residues that accompany missense mutations in SCN1A affect epilepsy phenotype severity
Admixture mapping of ankle-arm index: identification of a candidate locus associated with peripheral arterial disease
Large scale calcium channel gene rearrangements in episodic ataxia and hemiplegic migraine: implications for diagnostic testing
Phenotype and genotype in 17 patients with Goltz-Gorlin syndrome
Encephalocraniocutaneous lipomatosis
The unfolding clinical spectrum of POLG mutations
Enhancer deletions of the SHOX gene as a frequent cause of short stature: the essential role of a 250 kb downstream regulatory domain
Novel FOXG1 mutations associated with the congenital variant of Rett syndrome
Segregation of enlarged vestibular aqueducts in families with non-diagnostic SLC26A4 genotypes
Mutations in 3 genes (MKS3, CC2D2A and RPGRIP1L) cause COACH syndrome (Joubert syndrome with congenital hepatic fibrosis)
Phenotypic variability in 49 cases of ESCO2 mutations, including novel missense and codon deletion in the acetyltransferase domain, correlates with ESCO2 expression and establishes the clinical criter
Phenotypic variability in Rett syndrome associated with FOXG1 mutations in females
The very low penetrance of cystic fibrosis for the R117H mutation: a reappraisal for genetic counselling and newborn screening
Constitutional (germline) MLH1 epimutation as an aetiological mechanism for hereditary non-polyposis colorectal cancer
New challenges for informed consent through whole genome array testing
High frequency of de novo mutations in Li-Fraumeni syndrome
A de novo 1p34.2 microdeletion identifies the synaptic vesicle gene RIMS3 as a novel candidate for autism
A common UCP2 polymorphism predisposes to stress hyperglycaemia in severe sepsis
Contribution of RET, NTRK3 and EDN3 to the expression of Hirschsprung disease in a multiplex family
Deregulation of EIF4E: a novel mechanism for autism
International guidelines for the diagnosis and management of hereditary haemorrhagic telangiectasia
Analysis of multigenerational families with thoracic aortic aneurysms and dissections due to TGFBR1 or TGFBR2 mutations
Penetrance of marked cognitive impairment in older male carriers of the FMR1 gene premutation
A common variant in the adiponutrin gene influences liver enzyme values
TP53 PIN3 and MDM2 SNP309 polymorphisms as genetic modifiers in the Li-Fraumeni syndrome: impact on age at first diagnosis
Association of IL18RAP and CCR3 with coeliac disease in the Spanish population Spanish population
Defective complex I assembly due to C20orf7 mutations as a new cause of Leigh syndrome
Addition of pathology and biomarker information significantly improves the performance of the Manchester scoring system for BRCA1 and BRCA2 testing
Mutation prediction models in Lynch syndrome: evaluation in a clinical genetic setting
Characterisation of psoriasis susceptibility locus 6 (PSORS6) in patients with early onset psoriasis and evidence for interaction with PSORS1
A new microduplication syndrome encompassing the region of the Miller-Dieker (17p13 deletion) syndrome
Apoptosis and cancer: mutations within caspase genes
A genome-wide association study suggests that a locus within the ataxin 2 binding protein 1 gene is associated with hand osteoarthritis: the Treat-OA consortium
Hypomorphic mutations in meckelin (MKS3/TMEM67) cause nephronophthisis with liver fibrosis (NPHP11)
Identification of loss-of-function mutations of SLC35D1 in patients with Schneckenbecken dysplasia, but not with other severe spondylodysplastic dysplasias group diseases
17q21.31 microduplication patients are characterised by behavioural problems and poor social interaction
Change in stage distribution observed with annual screening for ovarian cancer in BRCA carriers
Refining the critical region of the novel 19q13.11 microdeletion syndrome to 750 Kb
Long-range regulation at the SOX9 locus in development and disease
Natural course of Fabry disease: changing pattern of causes of death in FOS - Fabry Outcome Survey
Association of RASGRP1 with type 1 diabetes is revealed by combined follow-up of two genome-wide studies
High resolution array analysis: diagnosing pregnancies with abnormal ultrasound findings
Wild-type but not mutant huntingtin modulates the transcriptional activity of liver X receptors
Phenotypic expansion and further characterisation of the 17q21.31 microdeletion syndrome
The high frequency of complement factor H related CFHR1 gene deletion is restricted to specific subgroups of patients with atypical haemolytic uraemic syndrome
Functional evidence implicating FOXL2 in non-syndromic premature ovarian failure and in the regulation of the transcription factor OSR2
Genome-wide linkage scan for plasma high density lipoprotein cholesterol, apolipoprotein A-1 and triglyceride variation among American Indian populations: the Strong Heart Family Study
Genetic screening of 202 individuals with congenital limb malformations and requiring reconstructive surgery
The effect of the MHC locus on autoantibodies in type 1 diabetes
TSC1 and TSC2 mutations in patients with lymphangioleiomyomatosis and tuberous sclerosis complex
SPRED1 mutations (Legius syndrome): another clinically useful genotype for dissecting the neurofibromatosis type 1 phenotype
New surfactant protein C gene mutations associated with diffuse lung disease
Association of chromosome 2q36.1-36.3 and autosomal dominant transmission in ankylosing spondylitis: results of genetic studies across generations of Han Chinese families Han Chinese
Germline mutation of microRNA-125a is associated with breast cancer
Genetic diagnosis of familial hypercholesterolaemia: the importance of functional analysis of potential splice-site mutations
SPRED1 germline mutations caused a neurofibromatosis type 1 overlapping phenotype
Further delineation of the 15q13 microdeletion and duplication syndromes: a clinical spectrum varying from non-pathogenic to a severe outcome
A genome-wide association study identifies a novel locus on chromosome 18q12.2 influencing white cell telomere length
Molecular analyses of the LRRK2 gene in European and North African autosomal dominant Parkinson's disease European; North African
Clinical spectrum of SIX3-associated mutations in holoprosencephaly: correlation between genotype, phenotype and function
A large deletion in GPR98 causes type IIC Usher syndrome in male and female members of an Iranian family
Conotruncal heart defects in three patients with congenital disorder of glycosylation type Ia (CDG Ia)
GPR98 mutations cause Usher syndrome type 2 in males
Parkin and PINK1 mutations in early-onset Parkinson's disease: comprehensive screening in publicly available cases and control
Mutations in DNAH5 account for only 15% of a non-preselected cohort of patients with primary ciliary dyskinesia
Mutations in the urocanase gene UROC1 are associated with urocanic aciduria
U-type exchange is the most frequent mechanism for inverted duplication with terminal deletion rearrangements
Genetics of microtia and associated syndromes
Rhabdomyosarcoma in patients with constitutional mismatch-repair-deficiency syndrome
Recessive primary congenital lymphoedema caused by a VEGFR3 mutation
Microdeletion 15q13.3: a locus with incomplete penetrance for autism, mental retardation, and psychiatric disorders
Do mutations of the Pendred syndrome gene, SLC26A4, confer resistance to asthma and hypertension?
Large scale replication analysis of loci associated with lipid concentrations in a Japanese population
Evaluation of a surveillance programme for women with a family history of breast cancer
Replication of restless legs syndrome loci in three European populations European populations
Predictive diagnosis of the cancer prone Li-Fraumeni syndrome by accident: new challenges through whole genome array testing
Further clinical and molecular delineation of the 9q subtelomeric deletion syndrome supports a major contribution of EHMT1 haploinsufficiency to the core phenotype
The genetic basis of congenital hyperinsulinism
Germline mutation in DOK7 associated with fetal akinesia deformation sequence
Premature death in adults with 22q11.2 deletion syndrome
A homozygous deletion of a normal variation locus in a patient with hearing loss from non-consanguineous parents
Identification of 13 novel NLRP7 mutations in 20 families with recurrent hydatidiform mole; missense mutations cluster in the leucine-rich region
A new nonsense mutation of SMAD8 associated with pulmonary arterial hypertension
Point mutations and a large intragenic deletion in SPG11 in complicated spastic paraplegia without thin corpus callosum
Infantile cardiomyopathy caused by a mutation in the overlapping region of mitochondrial ATPase 6 and 8 genes
A pilot open label, single dose trial of fenobam in adults with fragile X syndrome
19q13.11 deletion syndrome: a novel clinically recognisable genetic condition identified by array comparative genomic hybridisation