Journal of Medical Genetics - 2015

205 articles | Last updated: 2025-12-03 14:12:57
Caucasian
1
White
0
European
0
Other
5
Article Title Cauc. White Euro. Other Phrases Used
T A T A T A T A
MG-121 Five new patients with pure distal 1q trisomy, review of the literature and phenotype redefinition
MG-126 A<i>de novo</i>truncating mutation in the chromatin remodeler chd8 in a patient with autism, macrocephaly and overgrowth
MG-127 Diagnostic accuracy of chromosome microarray in children with epilepsy and neurological abnormalities of unknown aetiology
MG-106 Global developmental delay and characteristic facial features associated with pacs1 gene mutation – report of two cases
MG-134 Update on novel treatments for pyridoxine-dependent epilepsy due to antiquitin deficiency
MG-141 A further report of paediatric cancer and cleidocranial dysplasia raises the possibility of a causative association of weak effect
MG-142 Improved motor function with 5-hydroxytryptophan in a family with systemic serotonin deficiency, hemiplegic migraines and neurodegenerative course
MG-144 When rare happens: Characterising atypical breakpoints in CML
MG-115 Colorectal cancer patients with BRCA1 and BRCA2 mutations: Preparing for unexpected results
MG-116 Determining a genetic cause for familial intracranial aneurysms
MG-117 Clinical features of prc2 complex-related overgrowth due to mutations in eed
MG-118 Developing a multidisciplinary fragile X and related conditions clinic in victoria, BC
MG-101 Mutations preventing regulated exon skipping of a receptor tyrosine kinase cause a developmental disorder of osteogenesis
MG-123 Exonic and intronic NRXN1 deletions: Novel genotype-phenotype correlations
MG-125 CYP21A2 mutation spectrum in congenital adrenal hyperplasia identified from molecular genetic testing
MG-129 Our experience of<i>in silico</i>gene panel testing for clinically heterogeneous disorders using exome sequencing
MG-104 Fetal jugular lymph sacs – what is the significance?: Abstract MG-104 Table 1
MG-122 Cytogenetic characterisation of 3 small supernumerary chromosomal markers in a 1 year-old girl
MG-123 Genomics of early pregnancy loss
MG-137 Autosomal recessive disorders are common in the old order amish population of southwestern ontario
MG-138 Co-occurrence of cohen syndrome with 16p11.2 duplication: The exome sequencing approach
MG-139 Non-penetrance, variable expressivity or non pathogenicity of abcc9 dilated cardiomyopathy (DCM) mutation in 3 generation kindred
MG-107 Congenital sucrase-isomaltase deficiency: Identification of the common inuit founder mutation
MG-146 Potential biological explanations for no results for sex chromosome aneuploidy assessment using directed cell-free DNA analysis: A summary of three cases
MG-116 Report of 2 families with emberger syndrome (GATA2 mutation): Recognition of variance in clinical phenotype allows detection prior to malignant presentation
MG-100 Review of a large family with schwannomatosis identifies an expanded phenotype
MG-111 Clinical genetics education: Building foundations using e-modules for paediatric residents
MG-112 A KMT2D mutation segregating in a family presenting with autosomal dominant choanal atresia reinforces the kabuki/charge connexion
MG-119 Genetic polymorphism in the vitamin D receptor gene and 25-hydroxyvitamin D serum levels in east indian women with polycystic ovary syndrome east indian
MG-124 The investicate project: Identification of new variation, establishment of stem cells, and tissue collection advancing treatment efforts
MG-126 Data sharing and variant classification consensus building in the canadian open genetics repository (COGR)
MG-105 Delineating the phenotypes associated with the 15q11.2 BP1-BP2 deletion: Preliminary trends in psychometric evaluation
MG-145 Importance of fetal fraction analysis for CFDNA testing in the general pregnancy population: Abstract MG-145 Table 1
MG-147 Canadian open genetics repository (COGR): A unified clinical genome database as a community resource for standardising and sharing genetic interpretations
MG-112 Ten new cases further delineate the syndromic intellectual disability phenotype caused by mutations in DYRK1A
MG-114 First 2 years of experience of an integrated multidisciplinary clinic for adults with aortopathies in a canadian context
MG-110 Intravenous neonatal gene therapy corrects GM2 gangliosidoses in sandhoff mice for ‘long-term’, by using an aav expressing a new hexosaminidase variant
MG-113 A case ‘out of the blue’
MG-114 Clinical actionability of multi-gene panel tests for hereditary breast and ovarian cancer (HBOC)
MG-120 Chorionic villus sampling: 3 cases with discrepancies between ifish, array-cgh and karyotype
MG-122 Atypical fish pattern consistent with insertion of 5’ CBFB into 16p13 in a case of therapy-related acute myeloid leukaemia
MG-127 Residual disease monitoring in a retinoblastoma patient by pcr of a novel deletion breakpoint
MG-130 Utilising whole exome sequencing to identify causative variants in genetically heterogeneous disorders
MG-107 Multiple pathogenic variants identified by next-generation sequencing hereditary cancer panel testing – a case report
MG-108 Beyond the ACMG 56: Parental choices and initial results from a comprehensive whole genome sequencing-based search for predictive genomic variants in children
MG-105 Facial dysmorphism, skeletal abnormalities and central nervous system abnormalities in two sibs born to a consanguineous couple: A new autosomal recessive condition
MG-118 Towards understanding phenotypic variability using exome sequencing
MG-124 Zebrafish as an emerging model for human disease
MG-131 Incidental germline findings in tumour molecular profiling by next generation sequencing
MG-135 Metabolic diet app suite: Digital medicine to support families with inborn errors of metabolism
MG-140 17Q21.31 microdeletion syndrome: A description of two cases
MG-143 Age is significantly associated with the bone marrow engraftment in patients with allogeneic stem cell transplantation
MG-108 Agenesis of the corpus callosum and autism associated with zeb1 gene deletion – a case report
MG-109 A novel 0.34 MB microduplication of 9Q34.3 in a patient with congenital cardiac defects and learning disabilities
MG-115 Compound heterozygous SCN4A mutation underlies severe congenital hypotonia and biophysical alteration in the encoded voltage-gated NAV1.4 sodium channel
Author Index
MG-102 Coffin-siris syndrome caused by a missense mutation in arid1a
MG-131 ZNF259 is a candidate gene for alopecia-primordial dwarfism-renal syndrome (APDRS)
MG-133 Development and launch of an expanded pan-ethnic carrier screening panel
MG-136 Functional analysis of the autism and intellectual disability gene PTCHD1 reveals hedgehog receptor-like functions and pdz-binding domain-specific regulation of CNTNAP1 and NLGN1
MG-109 Revisiting a clinical diagnosis 15 years later with the aid of whole exome sequencing: Osteopetrosis versus harderophorphyria
MG-117 Chromosome microarray and non-coding DNA copy number variants – a case of alveolar capillary dysplasia at FOXF1 locus
MG-119 The evolving features of nicolaides-baraitser syndrome – a case report of a twenty-years follow-up
MG-128 Use of prenatal array comparative genomic hybridization in cases of fetal structural cardiac anomalies: New cases and review of the literature
MG-129 The development of a genetic newborn screening assay for permanent hearing loss using blood spots – a collaboration between newborn screening ontario (NSO) and the infant hearing program (IHP)
MG-130 Pure duplication of 1q42.11-q44(QTER): Further clinical delineation of a rare terminal duplication syndrome
MG-132 Next-generation sequencing in the neonatal intensive care unit: Pilot data from 12 newborns
MG-121 Complexity of phenotypes of females with unbalanced x-autosomal translocations exemplified by a case with 46, x,der (x)t (x;16)(p11.2;p13.2) karyotype
MG-132 Diagnostic utility of whole genome sequencing in paediatric medicine
MG-134 Validation of a customizable next generation sequencing panel for ear and eye diseases
MG-135 P300 regulates glucose homeostasis by maintaining islet mass
MG-103 Determining genetics referral eligibility for hereditary breast/ovarian cancer risk assessment: An electronic solution
Author index
Local genotype influences DNA methylation at two asthma-associated regions, 5q31 and 17q21, in a founder effect population
CRISPR-Cas9 for medical genetic screens: applications and future perspectives
Variable brain phenotype primarily affects the brainstem and cerebellum in patients with osteogenesis imperfecta caused by recessive<i>WNT1</i>mutations
Disruption of Golgi morphology and altered protein glycosylation in PLA2G6-associated neurodegeneration
High-sensitivity sequencing reveals multi-organ somatic mosaicism causing DICER1 syndrome
Large-scale targeted sequencing comparison highlights extreme genetic heterogeneity in nephronophthisis-related ciliopathies
The regulatory element READ1 epistatically influences reading and language, with both deleterious and protective alleles
Targeted massively parallel sequencing of a panel of putative breast cancer susceptibility genes in a large cohort of multiple-case breast and ovarian cancer families
Low-level<i>APC</i>mutational mosaicism is the underlying cause in a substantial fraction of unexplained colorectal adenomatous polyposis cases
Intragenic<i>KANSL1</i>mutations and chromosome 17q21.31 deletions: broadening the clinical spectrum and genotype–phenotype correlations in a large cohort of patients
Antiproteinuric therapy and Fabry nephropathy: factors associated with preserved kidney function during agalsidase-beta therapy
Correction
Homozygous missense mutation in the<i>LMAN2L</i>gene segregates with intellectual disability in a large consanguineous Pakistani family
Identification of germline<i>DICER1</i>mutations and loss of heterozygosity in familial Wilms tumour
Mutation in cytochrome b gene of mitochondrial DNA in a family with fibromyalgia is associated with NLRP3-inflammasome activation
Fatal infantile mitochondrial encephalomyopathy, hypertrophic cardiomyopathy and optic atrophy associated with a homozygous<i>OPA1</i>mutation
Recurrent copy number variations as risk factors for neurodevelopmental disorders: critical overview and analysis of clinical implications
Leukoencephalopathy and early death associated with an Ashkenazi-Jewish founder mutation in the Hikeshi gene Ashkenazi-Jewish
<i>UNC80</i>mutation causes a syndrome of hypotonia, severe intellectual disability, dyskinesia and dysmorphism, similar to that caused by mutations in its interacting cation channel<i>NALCN</i>
Congenital hyperinsulinism in children with paternal 11p uniparental isodisomy and Beckwith–Wiedemann syndrome
Deletions and de novo mutations of<i>SOX11</i>are associated with a neurodevelopmental disorder with features of Coffin–Siris syndrome
Too many numbers and complexity: time to update the classifications of neurogenetic disorders?
MicroRNA-129-1 acts as tumour suppressor and induces cell cycle arrest of GBM cancer cells through targeting IGF2BP3 and MAPK1
A targeted next-generation sequencing assay for the molecular diagnosis of genetic disorders with orodental involvement
MKS1 regulates ciliary INPP5E levels in Joubert syndrome
Hypomyelination and developmental delay associated with<i>VPS11</i>mutation in Ashkenazi-Jewish patients Ashkenazi-Jewish
Genome-wide significant association with seven novel multiple sclerosis risk loci
Genomic analysis of HPV-positive versus HPV-negative oesophageal adenocarcinoma identifies a differential mutational landscape
Characterising the spectrum of autosomal recessive hereditary hearing loss in Iran
A human laterality disorder caused by a homozygous deleterious mutation in<i>MMP21</i>
<i>HACE1</i>deficiency causes an autosomal recessive neurodevelopmental syndrome
Intellectual ability in tuberous sclerosis complex correlates with predicted effects of mutations on TSC1 and TSC2 proteins
Combination of palmoplantar keratoderma and hair shaft anomalies, the warning signal of severe arrhythmogenic cardiomyopathy: a systematic review on genetic desmosomal diseases
Clinical, biochemical and metabolic characterisation of a mild form of human short-chain enoyl-CoA hydratase deficiency: significance of increased N-acetyl-S-(2-carboxypropyl)cysteine excretion
Clinical and molecular predictors of mortality in neurofibromatosis 2: a UK national analysis of 1192 patients
WGSA: an annotation pipeline for human genome sequencing studies
Identification of a pathogenic<i>FTO</i>mutation by next-generation sequencing in a newborn with growth retardation and developmental delay
Mutations in human homologue of chicken<i>talpid3</i>gene (<i>KIAA0586</i>) cause a hybrid ciliopathy with overlapping features of Jeune and Joubert syndromes
ceRNA in cancer: possible functions and clinical implications
Bone marrow failure and developmental delay caused by mutations in poly(A)-specific ribonuclease (<i>PARN</i>)
Melanoma genetics
Genome-wide association meta-analyses to identify common genetic variants associated with hallux valgus in Caucasian and African Americans African Americans
Paraspinal neurofibromas and hypertrophic neuropathy in Noonan syndrome with multiple lentigines
Constitutional mismatch repair deficiency syndrome: clinical description in a French cohort
Neurogenetic evidence in the courtroom: a randomised controlled trial with German judges
Inherited predisposition to colorectal cancer: towards a more complete picture
Improving diagnostic precision, care and syndrome definitions using comprehensive next-generation sequencing for the inherited bone marrow failure syndromes
Mutations in<i>COQ4</i>, an essential component of coenzyme Q biosynthesis, cause lethal neonatal mitochondrial encephalomyopathy
<i>IFT81</i>, encoding an IFT-B core protein, as a very rare cause of a ciliopathy phenotype
Recommendations for somatic and germline genetic testing of single pheochromocytoma and paraganglioma based on findings from a series of 329 patients
<i>WAC</i>loss-of-function mutations cause a recognisable syndrome characterised by dysmorphic features, developmental delay and hypotonia and recapitulate 10p11.23 microdeletion syndrome
Old gene, new phenotype: mutations in heparan sulfate synthesis enzyme, EXT2 leads to seizure and developmental disorder, no exostoses
Charcot–Marie–Tooth diseases: an update and some new proposals for the classification
Cowden's syndrome with immunodeficiency
Buccal swab as a reliable predictor for X inactivation ratio in inaccessible tissues
Comprehensive spectrum of<i>BRCA1</i>and<i>BRCA2</i>deleterious mutations in breast cancer in Asian countries
Haplotype-based approach to known MS-associated regions increases the amount of explained risk
A homozygous missense variant in type I keratin<i>KRT25</i>causes autosomal recessive woolly hair
Whole exome sequencing identifies<i>LRP1</i>as a pathogenic gene in autosomal recessive keratosis pilaris atrophicans
Monogenic and chromosomal causes of isolated speech and language impairment
A<i>CASQ1</i>founder mutation in three Italian families with protein aggregate myopathy and hyperCKaemia
Multiple synchronous sites of origin of vestibular schwannomas in neurofibromatosis Type 2
Joubert syndrome: a model for untangling recessive disorders with extreme genetic heterogeneity
Lynch syndrome caused by<i>MLH1</i>mutations is associated with an increased risk of breast cancer: a cohort study
Continued lessons from the<i>INS</i>gene: an intronic mutation causing diabetes through a novel mechanism
Exploring genotype-phenotype relationships in Bardet-Biedl syndrome families
Rescue of primary ubiquinone deficiency due to a novel<i>COQ7</i>defect using 2,4–dihydroxybensoic acid
Combined mineralocorticoid and glucocorticoid deficiency is caused by a novel founder nicotinamide nucleotide transhydrogenase mutation that alters mitochondrial morphology and increases oxidative str
Recessive<i>DEAF1</i>mutation associates with autism, intellectual disability, basal ganglia dysfunction and epilepsy
Breakpoint mapping by whole genome sequencing identifies<i>PTH2R</i>gene disruption in a patient with midline craniosynostosis and a de novo balanced chromosomal rearrangement
Mutations in<i>SLC1A4</i>, encoding the brain serine transporter, are associated with developmental delay, microcephaly and hypomyelination
Constitutional or biallelic? Settling on a name for a recessively inherited cancer susceptibility syndrome
Streamlining review of research involving humans: Canadian models: Table 1
Prenatal genomic microarray and sequencing in Canadian medical practice: towards consensus
The<i>BRCA2</i>polymorphic stop codon: stuff or nonsense?
Microdeletions on 6p22.3 are associated with mesomelic dysplasia Savarirayan type
Contribution of the low-frequency, loss-of-function p.R270H mutation in<i>FFAR4</i>(<i>GPR120</i>) to increased fasting plasma glucose levels
A risk prediction algorithm for ovarian cancer incorporating<i>BRCA1, BRCA2</i>, common alleles and other familial effects
Hereditary diffuse gastric cancer syndrome: improved performances of the 2015 testing criteria for the identification of probands with a<i>CDH1</i>germline mutation
Potential research participants support the return of raw sequence data
Hereditary diffuse gastric cancer: updated clinical guidelines with an emphasis on germline<i>CDH1</i>mutation carriers
Mutations in apoptosis-inducing factor cause X-linked recessive auditory neuropathy spectrum disorder
Rare genetic variants in Tunisian Jewish patients suffering from age-related macular degeneration Tunisian Jewish
A prospective study validating a clinical scoring system and demonstrating phenotypical-genotypical correlations in Silver-Russell syndrome
The clinical application of genome-wide sequencing for monogenic diseases in Canada: Position Statement of the Canadian College of Medical Geneticists
A mutation of<i>MET</i>, encoding hepatocyte growth factor receptor, is associated with human<i>DFNB97</i>hearing loss
Scientific Writing for Impact Factor Journals
Progressive influence of body mass index-associated genetic markers in rural Gambians
A germline mutation in<i>PBRM1</i>predisposes to renal cell carcinoma
Canadian Open Genetics Repository (COGR): a unified clinical genomics database as a community resource for standardising and sharing genetic interpretations
Prevalence of<i>MLH1</i>constitutional epimutations as a cause of Lynch syndrome in unselected versus selected consecutive series of patients with colorectal cancer
Pallister-Killian syndrome: a study of 22 British patients
Nonsense mutation in the<i>WDR73</i>gene is associated with Galloway-Mowat syndrome
Minichromosome maintenance complex component 8 (MCM8) gene mutations result in primary gonadal failure
Rare variants in<i>SOS2</i>and<i>LZTR1</i>are associated with Noonan syndrome
Ten-year outcome of enzyme replacement therapy with agalsidase beta in patients with Fabry disease
Loss-of-function de novo mutations play an important role in severe human neural tube defects
Mutations in the mitochondrial cysteinyl-tRNA synthase gene,<i>CARS2,</i>lead to a severe epileptic encephalopathy and complex movement disorder
The heritability of leucocyte telomere length dynamics
A novel syndrome of Klippel-Feil anomaly, myopathy, and characteristic facies is linked to a null mutation in<i>MYO18B</i>
Familial periventricular nodular heterotopia, epilepsy and Melnick–Needles Syndrome caused by a single<i>FLNA</i>mutation with combined gain-of-function and loss-of-function effects
A founder<i>MYBPC3</i>mutation results in HCM with a high risk of sudden death after the fourth decade of life
De novo gain-of-function and loss-of-function mutations of<i>SCN8A</i>in patients with intellectual disabilities and epilepsy
Bilateral vestibular schwannomas in older patients: NF2 or chance?
Positional mapping of<i>PRKD1</i>,<i>NRP1</i>and<i>PRDM1</i>as novel candidate disease genes in truncus arteriosus
Correction
Correction
CRISPR-Cas9: a new and promising player in gene therapy
A homozygous<i>PMS2</i>founder mutation with an attenuated constitutional mismatch repair deficiency phenotype
A novel<i>APC</i>mutation defines a second locus for Cenani–Lenz syndrome
Thyroid hormone resistance syndrome due to mutations in the thyroid hormone receptor α gene (<i>THRA</i>)
Metabolic abnormalities in Williams–Beuren syndrome
Disruption of<i>CLPB</i>is associated with congenital microcephaly, severe encephalopathy and 3-methylglutaconic aciduria
BRCA1 Circos: a visualisation resource for functional analysis of missense variants
Utility of next generation sequencing in genetic diagnosis of early onset neuromuscular disorders
<i>DCAF4</i>, a novel gene associated with leucocyte telomere length
Transmission of germline<i>TP53</i>mutations from male carriers to female partners
A novel X-linked trichothiodystrophy associated with a nonsense mutation in RNF113A
Evaluation of somatic mutations in tibial pseudarthrosis samples in neurofibromatosis type 1
<i>MuSK</i>: a new target for lethal fetal akinesia deformation sequence (FADS)
Copy number variation of two separate regulatory regions upstream of<i>SOX9</i>causes isolated 46,XY or 46,XX disorder of sex development
Genetic associations of the interleukin locus at 1q32.1 with clinical outcomes of cutaneous melanoma
Mutations in<i>COA3</i>cause isolated complex IV deficiency associated with neuropathy, exercise intolerance, obesity, and short stature
mirTrios: an integrated pipeline for detection of de novo and rare inherited mutations from trios-based next-generation sequencing
Plasma globotriaosylsphingosine in relation to phenotypes of Fabry disease
Mapping the functional landscape of frequent<i>phenylalanine hydroxylase</i>(<i>PAH</i>) genotypes promotes personalised medicine in phenylketonuria
Correction
Evidence of digenic inheritance in Alport syndrome
SeqHBase: a big data toolset for family based sequencing data analysis
The twisting tale of woolly hair: a trait with many causes
The kinetochore protein,<i>CENPF</i>, is mutated in human ciliopathy and microcephaly phenotypes
A tribute to Margaret W. Thompson