Clinical Genetics - 2014

212 articles | Last updated: 2025-12-03 14:12:56
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Identification of a recurrent <i><scp>BRCA1</scp></i> exon 21‐22 genomic rearrangement in Malay breast cancer patients Malay
Expanding the phenotype of a recurrent <i>de novo</i> variant in <i><scp>PACS1</scp></i> causing intellectual disability
Mutations in <i><scp>FA2H</scp></i> in three Arab families with a clinical spectrum of neurodegeneration and hereditary spastic paraparesis
Disclosure of incidental findings in cancer genomic research: investigators' perceptions on obligations and barriers
<i><scp>BRCA1</scp></i> genetic testing in a Pakistani breast‐ovarian cancer family with multiple consanguineous marriages
Genetic features of Lynch syndrome in the Israeli population
Mutations in <scp>C2CD3</scp> cause oral‐facial‐digital syndrome through deregulation of centriole length
Homozygosity mapping reveals founder <i><scp>SEC23B</scp></i>‐<scp>Y462C</scp> mutations in Indian congenital dyserythropoietic anemia type <scp>II</scp>
Setleis syndrome: clinical, molecular and structural studies of the first <scp>TWIST2</scp> missense mutation
A mutation creating an out‐of‐frame alternative translation initiation site in the <scp><i>GRHPR</i></scp> 5′<scp>UTR</scp> causing primary hyperoxaluria type <scp>II</scp>
<i><scp>MICU1</scp></i> mutation: a genetic cause for a type of neuromuscular disease in children
A duplication of the whole <i><scp>KIAA2022</scp></i> gene validates the gene role in the pathogenesis of intellectual disability and autism
Insights into genotype–phenotype correlations from <i><scp>CREBBP</scp></i> point mutation screening in a cohort of 46 Rubinstein–Taybi syndrome patients
Response to Cragun et al.
Identification of eight novel mutations and transcript analysis of two splicing mutations in Chinese newborns with <scp>MCC</scp> deficiency Chinese
Autosomal recessive congenital cataract, intellectual disability phenotype linked to <i><scp>STX3</scp></i> in a consanguineous Tunisian family
Genetics of human isolated hereditary hair loss disorders
Mismatch repair genes founder mutations and cancer susceptibility in Lynch syndrome
Exome sequencing as a differential diagnosis tool: resolving mild trichohepatoenteric syndrome
Mutations predisposing to breast cancer in 12 candidate genes in breast cancer patients from Poland Polish women; Polish founder mutations
Characterization of a novel founder <i><scp>MSH6</scp></i> mutation causing Lynch syndrome in the French Canadian population French Canadian; first French settlers; Quebec (population)
Frequent occurrence of gastric cancer in Asian kindreds with Li–Fraumeni syndrome Asian (Asian families)
Use of panel tests in place of single gene tests in the cancer genetics clinic
Fanconi anaemia: genetics, molecular biology, and cancer – implications for clinical management in children and adults
Collagen type <scp>IV</scp>‐related nephropathies in Portugal: pathogenic <i><scp>COL4A5</scp></i> mutations and clinical characterization of 22 families Portuguese families
The gastrointestinal manifestation of constitutional mismatch repair deficiency syndrome: from a single adenoma to polyposis‐like phenotype and early onset cancer
Genetics of sudden cardiac death in the young
Patient reported outcomes and patient empowerment in clinical genetics services
Collagen type <scp>IV</scp>‐related nephropathies in Portugal: pathogenic <i><scp>COL4A3</scp></i> and <i><scp>COL4A4</scp></i> mutations and clinical characterization of 25 families
Low utilization of prenatal and pre‐implantation genetic diagnosis in Huntington disease – risk discounting in preventive genetics
Long‐term psychosocial and behavioral adjustment in individuals receiving genetic test results in Lynch syndrome
Consanguinity and founder effect for Gaucher disease mutation <scp>G377S</scp> in a population from Tabuleiro do Norte, Northeastern Brazil
Germline <scp><i>TP5</i>3</scp> mutations is common in patients with two early‐onset primary malignancies
Autism spectrum disorders: perceptions of genetic etiology and recurrence risk among Taiwanese parents of affected children
‘It had to be done’: genetic testing decisions for arrhythmogenic right ventricular cardiomyopathy
Autism spectrum disorders: a qualitative study of attitudes toward prenatal genetic testing and termination decisions of affected pregnancies Asians
<i>SOX10</i> mutations mimic isolated hearing loss
Next‐generation <scp>DNA</scp> sequencing of a Swedish malignant hyperthermia cohort
Prevalence of <i><scp>BRCA1</scp></i> and <i><scp>BRCA2</scp></i> mutations in unselected breast cancer patients from Peru Hispanic; Peruvian; Peruvian women; Peru; HISPANEL
p.<scp>L18P</scp>: a novel <scp>IDUA</scp> mutation that causes a distinct attenuated phenotype in mucopolysaccharidosis type I patients
Response to Cragun et al.
The <i>RBMX</i> gene as a candidate for the Shashi X‐linked intellectual disability syndrome
<scp>APC</scp> promoter <scp>1B</scp> deletion in seven American families with familial adenomatous polyposis
A recurrent germline <i><scp>BAP1</scp></i> mutation and extension of the <i><scp>BAP1</scp></i> tumor predisposition spectrum to include basal cell carcinoma
Detecting somatic mosaicism: considerations and clinical implications
The natural history of spinal neurofibromatosis: a critical review of clinical and genetic features
Cornelia de Lange syndrome
Frequencies and geographic distributions of genetic mutations in transthyretin‐ and non‐transthyretin‐related familial amyloidosis
<i><scp>BRCA1</scp></i> and <i><scp>BRCA2</scp></i> mutations and the risk for colorectal cancer
Genetic regulation of vertebrate eye development
Mutations within the spliceosomal gene <i>SNRPB</i> affect its auto‐regulation and are causative for classic cerebro‐costo‐mandibular syndrome
Autosomal recessive cystinuria caused by genome‐wide paternal uniparental isodisomy in a patient with Beckwith–Wiedemann syndrome
Targeted next‐generation sequencing in the diagnosis of neurodevelopmental disorders
<i><scp>FERMT1</scp></i> promoter mutations in patients with Kindler syndrome
The first mutation in <i><scp>CNGA2</scp></i> in two brothers with anosmia
Immunologic assessment and <scp>KMT2D</scp> mutation detection in Kabuki syndrome
Novel treatment strategies for schizophrenia from improved understanding of genetic risk
Translational genomics and head and neck cancer: toward precision medicine
Bioinformatics evaluation of <i><scp>NPHS2</scp></i> deletion mutation associated with congenital nephrotic syndrome in a consanguineous Pakistani family
Pregnant couples at increased risk for common aneuploidies choose maximal information from invasive genetic testing
Beneficial metabolic phenotypes caused by loss‐of‐function <i><scp>APOC3</scp></i> mutations
Cumulative effects of genetic markers and the detection of advanced colorectal neoplasias by population screening
Prostate cancer in a man with a <i><scp>BRCA2</scp></i> mutation and a personal history of bilateral breast cancer
Phenotypic spectrum associated with <i><scp>PTCHD1</scp></i> deletions and truncating mutations includes intellectual disability and autism spectrum disorder
Routine assessment of psychosocial problems after cancer genetic counseling: results from a randomized controlled trial
Role of the Nrf2 signaling system in health and disease
Focal dysplasia of the cerebral cortex and infantile spasms associated with somatic 1q21.1‐q44 duplication including the <i><scp>AKT3</scp></i> gene
Intellectual disability: novel mutations in <scp>DEAF1</scp> cause speech impairment and behavioral problems
<scp>DYRK1B</scp> variant linked to autosomal dominant metabolic syndrome
Molecular and computational analyses of genes involved in mannose 6‐phosphate independent trafficking
Germline <i><scp>BAP1</scp></i> mutations predispose also to multiple basal cell carcinomas
Autosomal dominant <i>IFIH1</i> gain‐of‐function mutations cause Aicardi–Goutières syndrome
Phosphoserine phosphatase (<i><scp>PSPH</scp></i>) gene mutation in an intellectual disability family from Pakistan
Challenges and opportunities in the investigation of unexplained intellectual disability using family‐based whole‐exome sequencing
From the era of genome analysis to the era of genomic drug discovery: a pioneering example of rheumatoid arthritis
Autosomal recessive mutations in the <scp><i>COL2A1</i></scp> gene cause severe spondyloepiphyseal dysplasia
Detailed characterization of <scp>MLH1</scp> p.<scp>D41H</scp> and p.<scp>N710D</scp> variants coexisting in a Lynch syndrome family with conserved <scp>MLH1</scp> expression tumors
Strategies for recruitment of relatives of <scp>BRCA</scp> mutation carriers to a genetic testing program in the Bahamas
Predictive genetic testing for adult‐onset disorders in minors: a critical analysis of the arguments for and against the 2013 <scp>ACMG</scp> guidelines
Living laboratory: whole‐genome sequencing as a learning healthcare enterprise
Use of genetic technologies to compare medicines
<scp>CHIPS</scp> for genetic testing to improve a regional clinical genetic service
Platelets are not all gray in <scp>GFI1B</scp> disease
Whole‐exome sequencing broadens the phenotypic spectrum of rare pediatric epilepsy: a retrospective study
Carrier screening of <i>RTEL1</i> mutations in the Ashkenazi Jewish population Ashkenazi Jewish; Ashkenazi Orthodox; Ashkenazi population
Expanding the spectrum of Grik2 mutations: intellectual disability, behavioural disorder, epilepsy and dystonia
Progressive microcephaly is caused by compound‐heterozygous mutations in <i><scp>QARS</scp></i>
Characterization of large deletions in the <i><scp>DHCR7</scp></i> gene
Genetic epidemiology of tooth agenesis in Japan: a population‐ and family‐based study
Diagnostic dilemmas in Fabry disease: a case series study on <scp>GLA</scp> mutations of unknown clinical significance
Ataxia telangiectasia: more variation at clinical and cellular levels
<scp>SNaPshot</scp> reveals high mutation and carrier frequencies of 15 common hearing loss mutants in a Chinese newborn cohort Chinese; Chinese population; Chinese newborns
Finnish Fanconi anemia mutations and hereditary predisposition to breast and prostate cancer Finns; Finland; founder populations
A founder mutation in the <i><scp>TCIRG1</scp></i> gene causes osteopetrosis in the Ashkenazi Jewish population Ashkenazi Jewish (AJ) population
A review of mismatch repair gene transcripts: issues for interpretation of <scp>mRNA</scp> splicing assays
A mutation in the Z‐line Cypher/ZASP protein is associated with arrhythmogenic right ventricular cardiomyopathy
Atypical giant axonal neuropathy arising from a homozygous mutation by uniparental isodisomy
<i><scp>EIF2AK4</scp></i> genetic mutations cause a recessive form of rare and deadly lung disease, pulmonary veno‐occlusive disease
Phenylalanine hydroxylase deficiency in Mexico: genotype–phenotype correlations, <scp>BH<sub>4</sub></scp> responsiveness and evidence of a founder effect
Changes in hematological parameters in α‐thalassemia individuals co‐inherited with erythroid Krüppel‐like factor mutations
Identification of novel mutations in the <i><scp>VPS33B</scp></i> gene involved in arthrogryposis, renal dysfunction, and cholestasis syndrome
The role of targeted <i><scp>BRCA1</scp></i>/<i><scp>BRCA2</scp></i> mutation analysis in hereditary breast/ovarian cancer families of Portuguese ancestry Portuguese ancestry
Developmental Biology: Frontiers for Clinical Genetics
Help or hindrance: young people's experiences of predictive testing for Huntington's disease
<i><scp>COL1A1</scp></i> C‐propeptide cleavage site mutation causes high bone mass, bone fragility and jaw lesions: a new cause of gnathodiaphyseal dysplasia?
Mutation update and uncommon phenotypes in a French cohort of 96 patients with <i><scp>WFS1</scp></i>‐related disorders French cohort
<scp>R705H</scp> mutation of <i><scp>MYH9</scp></i> is associated with <i><scp>MYH9</scp></i>‐related disease and not only with non‐syndromic deafness <scp>DFNA17</scp>
An assessment of Canadian systems for triaging referred out genetic testing
Fetal skeletal dysplasias in a tertiary care center: radiology, pathology, and molecular analysis of 112 cases
Cardiovascular manifestations in Marfan syndrome and related diseases; multiple genes causing similar phenotypes
Genetic testing for sporadic hearing loss using targeted massively parallel sequencing identifies 10 novel mutations
Incidental findings on array comparative genomic hybridization: detection of carrier females of dystrophinopathy without any family history
Interactive e‐counselling for genetics pre‐test decisions: where are we now?
A cohort study of <i><scp>MFN2</scp></i> mutations and phenotypic spectrums in Charcot–Marie–Tooth disease <scp>2A</scp> patients
Patient experiences with gene panels based on exome sequencing in clinical diagnostics: high acceptance and low distress
Methylmalonic aciduria <i>cblB</i> type: characterization of two novel mutations and mitochondrial dysfunction studies
<i><scp>BRCA2</scp></i> gene: a candidate for clinical testing in familial colorectal cancer type X
Monoallelic <i><scp>ABCC8</scp></i> mutations are a common cause of diazoxide‐unresponsive diffuse form of congenital hyperinsulinism
Systematic review of the psychosocial aspects of living with Marfan syndrome
Determination of the allelic frequency in Smith–Lemli–Opitz syndrome by analysis of massively parallel sequencing data sets
Alpha‐thalassemia intellectual disability: variable phenotypic expression among males with a recurrent nonsense mutation – c.<scp>109C</scp>&gt;T (p.<scp>R37X</scp>)
Cross‐border reprogenetic services
Influence of individual differences in disease perception on consumer response to direct‐to‐consumer genomic testing
Mutations in <i><scp>POT1</scp></i> predispose to familial cutaneous malignant melanoma
Mutations in <i><scp>COG2</scp></i> encoding a subunit of the conserved oligomeric golgi complex cause a congenital disorder of glycosylation
Preferences for results from genomic microarrays: comparing parents and health care providers
Excessively redundant umbilical skin as a potential early clinical feature of Morquio syndrome and <i><scp>FKBP14</scp></i>‐related Ehlers–Danlos syndrome
Evaluating a unique, specialist psychiatric genetic counseling clinic: uptake and impact
Protein truncating variants of <i><scp>SLC30A8</scp></i> reduce type 2 diabetes mellitus risk in humans
Spondyloepimetaphyseal dysplasia with joint laxity (Beighton type); mutation analysis in eight affected South African families Afrikaans-speaking community of South Africa; South African families; Iranian persons; Japanese fami
Preimplantation genetic diagnosis for cystic fibrosis: the Montpellier center's 10‐year experience
A cross‐sectional multicenter study of osteogenesis imperfecta in North America – results from the linked clinical research centers
The importance of genetic susceptibility in Dupuytren's disease
Innovative personalized medicine in gastric cancer: time to move forward
Intragenic <i><scp>CAMTA1</scp></i> deletions are associated with a spectrum of neurobehavioral phenotypes
Pre‐test genetic counseling services for hereditary breast and ovarian cancer delivered by non‐genetics professionals in the state of Florida
Genetic and bioinformatics analysis of four novel <i><scp>GCK</scp></i> missense variants detected in Caucasian families with <scp>GCK‐MODY</scp> phenotype
Hypertrophic pyloric stenosis in twins; genetic or environmental factors
Phenotypic heterogeneity and mutational spectrum in a cohort of 45 Italian males subjects with X‐linked ectodermal dysplasia Italian
The impact of multiplex genetic testing on disease risk perceptions
Antisense‐mediated therapeutic pseudoexon skipping in <scp>TMEM165‐CDG</scp>
Clinical, imaging, and molecular findings in a sample of Mexican families with pantothenate kinase‐associated neurodegeneration Mexican, Mexico
<i>De novo <scp>WNT5A</scp></i>‐associated autosomal dominant Robinow syndrome suggests specificity of genotype and phenotype
Genetic evaluation based on family history and Her2 status correctly identifies <i><scp>TP53</scp></i> mutations in very early onset breast cancer cases
A homozygous <i><scp>SIX6</scp></i> mutation is associated with optic disc anomalies and macular atrophy and reduces retinal ganglion cell differentiation
Clinical application of genetics to guide prevention and treatment of oral diseases
Prevalence and penetrance of <i><scp>ZFPM2</scp></i> mutations and deletions causing congenital diaphragmatic hernia
<i><scp>D</scp>e novo <scp>EEF1A2</scp></i> mutations in patients with characteristic facial features, intellectual disability, autistic behaviors and epilepsy
Comprehensive genetic testing can save lives in hereditary hearing loss
Hypotrichosis‐lymphedema‐telangiectasia‐renal defect associated with a truncating mutation in the <scp>SOX18</scp> gene
Charcot–Marie–Tooth disease and pathways to molecular based therapies
Novel gene mutations and clinical features in patients with pantothenate kinase‐associated neurodegeneration
The emerging era of pharmacogenomics: current successes, future potential, and challenges
Olfaction evaluation and correlation with brain atrophy in Bardet‐Biedl syndrome
Views of primary care providers regarding the return of genome sequencing incidental findings
Personalized ophthalmology
The homozygous p.<scp>V37I</scp> variant of <i><scp>GJB2</scp></i> is associated with diverse hearing phenotypes East Asians; Chinese Han
The kidney in Fabry's disease
Informed consent for human genetic and genomic studies: a systematic review
Personalized gene silencing therapeutics for Huntington disease
Reevaluation of the <scp>CMT1A</scp> duplication frequency in Greek Charcot‐Marie‐Tooth type 1 patients Greek
<i><scp>GFI1B</scp></i> mutation causes autosomal dominant gray platelet syndrome
Personalized therapy for breast cancer
Pharmacogenetics of beta2 adrenergic receptor agonists in asthma management
Autosomal dominant polycystic kidney disease caused by somatic and germline mosaicism
Analysis of current testing practices for biallelic <i><scp>MUTYH</scp></i> mutations in <i><scp>MUTYH</scp></i>‐associated polyposis
Using a combination of whole‐exome sequencing and homozygosity mapping to identify a novel mutation of <scp>SCARB2</scp>
Using pharmacogenetics in real time to guide therapy: the warfarin example
Whole exome analysis identifies dominant <i><scp>COL4A1</scp></i> mutations in patients with complex ocular phenotypes involving microphthalmia
A comprehensive search for mutations in the <i><scp>PKD1</scp></i> and <i><scp>PKD2</scp></i> in Japanese subjects with autosomal dominant polycystic kidney disease European or American ADPKD
Genetic predictors of cardiovascular morbidity in Bardet–Biedl syndrome
<i><scp>SLC26A2</scp></i> disease spectrum in Sweden – high frequency of recessive multiple epiphyseal dysplasia (<scp>rMED</scp>) Finnish founder mutation; Sweden
Genotype–phenotype correlation of contiguous gene deletions of <i><scp>SLC6A8</scp>, <scp>BCAP31</scp></i> and <scp><i>ABCD1</i></scp>
Biobanks and personalized medicine
Dawning of the epigenetic era in hereditary cancer
Myhre syndrome
Strabismus genetics across a spectrum of eye misalignment disorders
The beginning of personalized medicine in sepsis: small steps to a bright future
Genotype/phenotype analysis in Chinese laminin‐α2 deficient congenital muscular dystrophy patients
A novel <i><scp>ACAD8</scp></i> mutation in asymptomatic patients with isobutyryl‐<scp>CoA</scp> dehydrogenase deficiency and a review of the <i><scp>ACAD8</scp></i> mutation spectrum
A ‘cure’ for Down syndrome: what do parents want?
Genotype phenotype correlations for hearing impairment: Approaches to management
Novel <i><scp>KDM6A</scp></i> (<scp>UTX</scp>) mutations and a clinical and molecular review of the X‐linked Kabuki syndrome (<scp>KS2</scp>)
Recurrent mutations of <scp>BRCA1</scp> and <scp>BRCA2</scp> in Poland: an update Polish women
Search for <i><scp>ReCQL4</scp></i> mutations in 39 patients genotyped for suspected Rothmund–Thomson/Baller‐Gerold syndromes
<scp>MicroRNAs</scp> in personalized cancer therapy
Panel‐based testing for inherited colorectal cancer: a descriptive study of clinical testing performed by a US laboratory
Hereditary haemochromatosis caused by homozygous <i><scp>HJV</scp></i> mutation evolved through paternal disomy
Novel <i><scp>LAMB3</scp></i> mutations cause non‐syndromic amelogenesis imperfecta with variable expressivity
A study of 133 Chinese children with mitochondrial respiratory chain complex I deficiency
Mosaic <i><scp>KCNJ2</scp></i> mutation in Andersen–Tawil syndrome: targeted deep sequencing is useful for the detection of mosaicism
The revised ghent nosology; reclassifying isolated ectopia lentis
The communication of secondary variants: interviews with parents whose children have undergone array‐<scp>CGH</scp> testing
Clinical and molecular characterization of Rubinstein‐Taybi syndrome patients carrying distinct novel mutations of the <i><scp>EP300</scp></i> gene
Novel mutations in <i><scp>PCYT1A</scp></i> are responsible for spondylometaphyseal dysplasia with cone‐rod dystrophy
Somatic mosaicism in a Cornelia de Lange syndrome patient with <i><scp>NIPBL</scp></i> mutation identified by different next generation sequencing approaches
<scp>BRCA1</scp> and <scp>BRCA2</scp> – update and implications on the genetics of breast cancer: a clinical perspective
Overview of Bardet–Biedl syndrome in Spain: identification of novel mutations in <i><scp>BBS1,</scp><scp>BBS10</scp></i> and <i><scp>BBS12</scp></i> genes
Microduplications encompassing the Sonic hedgehog limb enhancer <scp>ZRS</scp> are associated with Haas‐type polysyndactyly and Laurin‐Sandrow syndrome
Epigenetic mechanisms in the pathogenesis of Lynch syndrome
Clinical and molecular findings of chronic granulomatous disease in Oman: family studies
Psychological status in female carriers of premutation <scp>FMR1</scp> allele showing a complex relationship with the size of <scp>CGG</scp> expansion
‘When atlastin meets spastin’
Founder mutation for Huntington disease in Caucasus Jews The prevalence of HD is known for Europeans; Caucasus Jews (CJ), Azerbaijani, Ashkenazi Jewish, Israeli population
Mutation spectrum in <scp>BBS</scp> genes guided by homozygosity mapping in an Indian cohort
Attitudinal concordance toward uptake and disclosure of genetic testing for cancer susceptibility in patient–family member dyads
Mutation spectrum and prevalence of <i>BRCA</i>1 and <i>BRCA</i>2 genes in patients with familial and early‐onset breast/ovarian cancer from Tunisia Tunisian population; Tunisian women; our population
<scp>SPG35</scp> contributes to the second common subtype of <scp>AR‐HSP</scp> in China: frequency analysis and functional characterization of <i><scp>FA2H</scp></i> gene mutations Chinese
Active approach for breast cancer genetic counseling during radiotherapy: long‐term psychosocial and medical impact
<scp>DFNB16</scp> is a frequent cause of congenital hearing impairment: implementation of <i><scp>STRC</scp></i> mutation analysis in routine diagnostics
Ocular and craniofacial phenotypes in a large Brazilian family with congenital aniridia
Evidence‐based genetic counselling implications for Huntington disease intermediate allele predictive test results