Journal of Medical Genetics - 2016

128 articles | Last updated: 2025-12-03 14:12:57
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A genome-wide interaction analysis of tricyclic/tetracyclic antidepressants and RR and QT intervals: a pharmacogenomics study from the Cohorts for Heart and Aging Research in Genomic Epidemiology (CHA
Outcome of 24 years national surveillance in different hereditary colorectal cancer subgroups leading to more individualised surveillance
Compound heterozygosity for severe and hypomorphic<i>NDUFS2</i>mutations cause non-syndromic LHON-like optic neuropathy
Genetics of idiopathic pulmonary fibrosis: from mechanistic pathways to personalised medicine
Single synonymous mutation in factor IX alters protein properties and underlies haemophilia B
Decreased telomere length in children with cartilage-hair hypoplasia
Precise long non-coding RNA modulation in visual maintenance and impairment
The performance of deleteriousness prediction scores for rare non-protein-changing single nucleotide variants in human genes
Recessive progressive symmetric erythrokeratoderma results from a homozygous loss-of-function mutation of<i>KRT83</i>and is allelic with dominant monilethrix
Genome-wide association study identifies variants in<i>HORMAD2</i>associated with tonsillectomy
A novel somatic mutation achieves partial rescue in a child with Hutchinson-Gilford progeria syndrome
The importance of dynamic re-analysis in diagnostic whole exome sequencing
Diagnostic value of exome and whole genome sequencing in craniosynostosis
Hypersuccinylacetonaemia and normal liver function in maleylacetoacetate isomerase deficiency
Genetic determinants of myocardial dysfunction
Risk assessment of maternally inherited<i>SDHD</i>paraganglioma and phaeochromocytoma
Oral pharmacological chaperone migalastat compared with enzyme replacement therapy in Fabry disease: 18-month results from the randomised phase III ATTRACT study
The UCL low-density lipoprotein receptor gene variant database: pathogenicity update
ACBD5 deficiency causes a defect in peroxisomal very long-chain fatty acid metabolism
Congenital valvular defects associated with deleterious mutations in the<i>PLD1</i>gene
The cerebellum and embodied semantics: evidence from a case of genetic ataxia due to<i>STUB1</i>mutations
<i>AMMECR1</i>: a single point mutation causes developmental delay, midface hypoplasia and elliptocytosis
The impact of a panel of 18 SNPs on breast cancer risk in women attending a UK familial screening clinic: a case–control study
A<i>de novo</i>missense mutation of<i>GABRB2</i>causes early myoclonic encephalopathy
Impact of subsidies on cancer genetic testing uptake in Singapore
Chitayat syndrome: hyperphalangism, characteristic facies, hallux valgus and bronchomalacia results from a recurrent c.266A&gt;G p.(Tyr89Cys) variant in the<i>ERF</i>gene
Erratum: The kinetochore protein, <i>CENPF</i> , is mutated in human ciliopathy and microcephaly phenotypes
Genotype–phenotype correlation and functional studies in patients with cystic fibrosis bearing CFTR complex alleles
A novel<i>TRAPPC11</i>mutation in two Turkish families associated with cerebral atrophy, global retardation, scoliosis, achalasia and alacrima
Mutations in the phosphatidylinositol glycan C (<i>PIGC</i>) gene are associated with epilepsy and intellectual disability
<i>COA7</i>(<i>C1orf163</i><i>/RESA1</i>) mutations associated with mitochondrial leukoencephalopathy and cytochrome c oxidase deficiency
New paradigms for<i>BRCA1</i>/<i>BRCA2</i>testing in women with ovarian cancer: results of the Genetic Testing in Epithelial Ovarian Cancer (GTEOC) study
Anxiety delivered direct-to-consumer: are we asking the right questions about the impacts of DTC genetic testing?
Common cancers share familial susceptibility: implications for cancer genetics and counselling
Carriers of a<i>VEGFA</i>enhancer polymorphism selectively binding CHOP/DDIT3 are predisposed to increased circulating levels of thyroid-stimulating hormone
<i>CEP78</i>is mutated in a distinct type of Usher syndrome
High diagnostic yield of syndromic intellectual disability by targeted next-generation sequencing
Targeted massively parallel sequencing and histological assessment of skeletal muscles for the molecular diagnosis of inherited muscle disorders
<i>SLC13A5</i>is the second gene associated with Kohlschütter–Tönz syndrome
<i>PALB2</i>,<i>CHEK2</i>and<i>ATM</i>rare variants and cancer risk: data from COGS
New insights into the genetic component of non-infectious uveitis through an Immunochip strategy
Gastric cancer: somatic genetics as a guide to therapy
<i>KCNA4</i>deficiency leads to a syndrome of abnormal striatum, congenital cataract and intellectual disability
Homozygous and compound heterozygous mutations in the<i>FBN1</i>gene: unexpected findings in molecular diagnosis of Marfan syndrome
Meta-analysis of genome-wide association studies of HDL cholesterol response to statins
<i>FOXP2</i>variants in 14 individuals with developmental speech and language disorders broaden the mutational and clinical spectrum
Novel asymptomatic CNS findings in patients with<i>ACVR1/ALK2</i>mutations causing fibrodysplasia ossificans progressiva
Erratum:<i>Genetic and neurodevelopmental spectrum of<i>SYNGAP1</i>-associated intellectual disability and epilepsy</i>
Congenital heart defects and left ventricular non-compaction in males with loss-of-function variants in<i>NONO</i>
Amelogenesis imperfecta in familial hypomagnesaemia and hypercalciuria with nephrocalcinosis caused by<i>CLDN19</i>gene mutations
Cystathionine β-synthase genetic variant rs2124459 is associated with a reduced risk of cleft palate in French and Belgian populations French and Belgian populations
Survival and causes of death in patients with von Hippel-Lindau disease
Phenotypic spectrum of<i>POLR3B</i>mutations: isolated hypogonadotropic hypogonadism without neurological or dental anomalies
Copy-number variations are enriched for neurodevelopmental genes in children with developmental coordination disorder
New insights in the molecular signature of advanced medullary thyroid cancer: evidence of a bad outcome of cases with double<i>RET</i>mutations
De novo mtDNA point mutations are common and have a low recurrence risk
Genetic and neurodevelopmental spectrum of<i>SYNGAP1</i>-associated intellectual disability and epilepsy
Eight further individuals with intellectual disability and epilepsy carrying bi-allelic<i>CNTNAP2</i>aberrations allow delineation of the mutational and phenotypic spectrum
Mutations specific to the Rac-GEF domain of<i>TRIO</i>cause intellectual disability and microcephaly
The clinical, biochemical and genetic features associated with<i>RMND1</i>-related mitochondrial disease
Novel myosin-based therapies for congenital cardiac and skeletal myopathies
De novo missense variants in<i>HECW2</i>are associated with neurodevelopmental delay and hypotonia
Mutations in<i>MYT1</i>, encoding the myelin transcription factor 1, are a rare cause of OAVS
De novo mutations of<i>KIAA2022</i>in females cause intellectual disability and intractable epilepsy
Specifying the ovarian cancer risk threshold of ‘premenopausal risk-reducing salpingo-oophorectomy’ for ovarian cancer prevention: a cost-effectiveness analysis
Mutations in<i>LTBP3</i>cause acromicric dysplasia and geleophysic dysplasia
Correction
Gain-of-function mutation in<i>TRPV4</i>identified in patients with osteonecrosis of the femoral head
Novel<i>LMNA</i>mutations cause an aggressive atypical neonatal progeria without progerin accumulation
Mutations in<i>HECW2</i>are associated with intellectual disability and epilepsy
<i>SETD2</i>and<i>DNMT3A</i>screen in the Sotos-like syndrome French cohort
Phenome-wide association study maps new diseases to the human major histocompatibility complex region
Complex clonal mosaicism within microdissected intestinal metaplastic glands without concurrent gastric cancer
Kinetic mutations in argininosuccinate synthetase deficiency: characterisation and in vitro correction by substrate supplementation
Mutations in<i>TUBB8</i>cause a multiplicity of phenotypes in human oocytes and early embryos
A splicing mutation in<i>VPS4B</i>causes dentin dysplasia I
Genes associated with common variable immunodeficiency: one diagnosis to rule them all?
Clinical course of sly syndrome (mucopolysaccharidosis type VII)
Erratum: A specific mutation in TBL1XR1 causes Pierpont syndrome
Complementation of hypersensitivity to DNA interstrand crosslinking agents demonstrates that<i>XRCC2</i>is a Fanconi anaemia gene
GATOR1 complex: the common genetic actor in focal epilepsies
Deficiency of HTRA2/Omi is associated with infantile neurodegeneration and 3-methylglutaconic aciduria
Molecular findings from 537 individuals with inherited retinal disease
Mutations in<i>CEP120</i>cause Joubert syndrome as well as complex ciliopathy phenotypes
When chromatin organisation floats astray: the<i>Srcap</i>gene and Floating–Harbor syndrome
Identification of genomic deletions causing inherited retinal degenerations by coverage analysis of whole exome sequencing data
No evidence that protein truncating variants in<i>BRIP1</i>are associated with breast cancer risk: implications for gene panel testing
Cystic cerebellar dysplasia and biallelic<i>LAMA1</i>mutations: a lamininopathy associated with tics, obsessive compulsive traits and myopia due to cell adhesion and migration defects
The genetic landscape and clinical implications of vertebral anomalies in VACTERL association
A recurrent mitochondrial p.Trp22Arg<i>NDUFB3</i>variant causes a distinctive facial appearance, short stature and a mild biochemical and clinical phenotype
A detailed clinical analysis of 13 patients with AUTS2 syndrome further delineates the phenotypic spectrum and underscores the behavioural phenotype
Functional and genetic epidemiological characterisation of the<i>FFAR4</i>(<i>GPR120</i>) p.R270H variant in the Danish population
Microduplications at the pseudoautosomal<i>SHOX</i>locus in autism spectrum disorders and related neurodevelopmental conditions
Naturally occurring<i>BRCA2</i>alternative mRNA splicing events in clinically relevant samples
Evidence of mutations in<i>RIC3</i>acetylcholine receptor chaperone as a novel cause of autosomal-dominant Parkinson's disease with non-motor phenotypes
Identification of biallelic<i>LRRK1</i>mutations in osteosclerotic metaphyseal dysplasia and evidence for locus heterogeneity
Germline<i>RRAS2</i>mutations are not associated with Noonan syndrome
Meta-analysis of 49 549 individuals imputed with the 1000 Genomes Project reveals an exonic damaging variant in<i>ANGPTL4</i>determining fasting TG levels
Deletions of 5′<i>HOXC</i>genes are associated with lower extremity malformations, including clubfoot and vertical talus
Time to treatment benefit for adult patients with Fabry disease receiving agalsidase β: data from the Fabry Registry
Cluster-randomised non-inferiority trial comparing DVD-assisted and traditional genetic counselling in systematic population testing for BRCA1/2 mutations
Improving diagnosis and broadening the phenotypes in early-onset seizure and severe developmental delay disorders through gene panel analysis
<i>AKAP2</i>identified as a novel gene mutated in a Chinese family with adolescent idiopathic scoliosis
Clinical features for diagnosis and management of patients with PRDM12 congenital insensitivity to pain
Circular RNAs: a new frontier in the study of human diseases
Prevalence of<i>BRCA1/2</i>germline mutations in 21 401 families with breast and ovarian cancer
Risky business: getting a grip on BRIP
Epigenetic inheritance of telomere length obscures identification of causative<i>PARN</i>locus
COLD-PCR and microarray: two independent highly sensitive approaches allowing the identification of fetal paternally inherited mutations in maternal plasma
Deficiency of the myogenic factor MyoD causes a perinatally lethal fetal akinesia
Discovery of a frameshift mutation in podocalyxin-like (PODXL) gene, coding for a neural adhesion molecule, as causal for autosomal-recessive juvenile Parkinsonism
Genetic spectrum of Saudi Arabian patients with antenatal cystic kidney disease and ciliopathy phenotypes using a targeted renal gene panel
Mutations in<i>EXOSC2</i>are associated with a novel syndrome characterised by retinitis pigmentosa, progressive hearing loss, premature ageing, short stature, mild intellectual disability and distinc
A study of common Mendelian disease carriers across ageing British cohorts: meta-analyses reveal heterozygosity for alpha 1-antitrypsin deficiency increases respiratory capacity and height
<i>HSP110</i>T17 simplifies and improves the microsatellite instability testing in patients with colorectal cancer
Allele-specific DNA hypomethylation characterises FSHD1 and FSHD2
An interstitial deletion within 9p21.3 and extending beyond<i>CDKN2A</i>predisposes to melanoma, neural system tumours and possible haematological malignancies
Deficiency of the alkaline ceramidase ACER3 manifests in early childhood by progressive leukodystrophy
Disturbed mitochondrial and peroxisomal dynamics due to loss of MFF causes Leigh-like encephalopathy, optic atrophy and peripheral neuropathy
Multigene testing of moderate-risk genes: be mindful of the missense
Molecular insights into development in humans:<i>Studies in Normal Development and Birth Defects</i>
A specific mutation in<i>TBL1XR1</i>causes Pierpont syndrome
The importance of genetic diagnosis for Duchenne muscular dystrophy
Chromosomal microarray testing identifies a 4p terminal region associated with seizures in Wolf–Hirschhorn syndrome
Gene editing of<i>DNAH11</i>restores normal cilia motility in primary ciliary dyskinesia
<i>KCNT1</i>mutations in seizure disorders: the phenotypic spectrum and functional effects
Current needs for human and medical genomics research infrastructure in low and middle income countries: Table 1
Identification of novel genetic causes of Rett syndrome-<i>like</i>phenotypes