Clinical Genetics - 2012

158 articles | Last updated: 2025-12-03 14:12:56
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Article Title Cauc. White Euro. Other Phrases Used
T A T A T A T A
Cardiac characterization of 16 patients with large <i><scp>NF1</scp></i> gene deletions
Homozygosity for a <i><scp>FBN1</scp></i> missense mutation causes a severe Marfan syndrome phenotype
<scp>cDNA</scp> analysis of the <i><scp>BRCA1</scp></i> unclassified variant c.5194‐<scp>12G</scp>&gt;<scp>A</scp>
Mental retardation, short stature and synpolydactyly in a manifesting heterozygote of Bartsocas–Papas syndrome
A germline mosaic <i><scp>BRCA1</scp></i> exon deletion in a woman with bilateral basal‐like breast cancer
Mutation identification of Fabry disease in families with other lysosomal storage disorders Italian
Syndromic non‐compaction of the left ventricle: associated chromosomal anomalies
A novel syndrome of abnormal striatum and congenital cataract: evidence for linkage to chromosomes 11
New evidence for, and challenges in, linking small <scp>CGG</scp> repeat expansion <scp>FMR1</scp> alleles with Parkinson's disease
Novel <i><scp>OTOA</scp></i> mutations cause autosomal recessive non‐syndromic hearing impairment in Pakistani families
The <i><scp>MSH2</scp></i> c.388_389del mutation shows a founder effect in Portuguese Lynch syndrome families Portuguese ancestry; originating from the north of Portugal; mentions families from Germany, Scotlan
Whole exome sequencing in a patient with uniparental disomy of chromosome 2 and a complex phenotype
Knowledge of the Genetic Information Nondiscrimination act among individuals affected by Huntington disease
Perspectives of clinical genetics professionals toward genome sequencing and incidental findings: a survey study
Aging in Rett syndrome: a longitudinal study Dutch
Homozygous truncation of SIX6 causes complex microphthalmia in humans
Nucleotide variants of genes encoding components of the Wnt signalling pathway and the risk of non‐syndromic tooth agenesis
Prenatal diagnostic conundrum involving a novel <i><scp>ATP7A</scp></i> duplication
Incomplete nonsense‐mediated decay facilitates detection of a multi‐exonic deletion mutation in <i><scp>SGCE</scp></i>
Homozygosity mapping identifies genetic defects in four consanguineous families with retinal dystrophy from Pakistan
Neurodevelopmental consequences of maternal distress: what do we really know?
Evaluation of exclusion prenatal and exclusion preimplantation genetic diagnosis for Huntington's disease in the Netherlands
Protein expression studies of desmoplakin mutations in cardiomyopathy patients reveal different molecular disease mechanisms
To tell or not to tell – what to do about p.<scp>C282Y</scp> heterozygotes identified by <i><scp>HFE</scp></i> screening
Comparison of X‐chromosome inactivation in Duchenne muscle/myocardium‐manifesting carriers, non‐manifesting carriers and related daughters
Cross‐sectional assessment of pain and physical function in skeletal dysplasia patients
Inheritance of the chronic myeloproliferative neoplasms. A systematic review
Association of <scp>HLA</scp> locus variant in Parkinson's disease
Monozygotic twins discordant for submicroscopic chromosomal anomalies in 2p25.3 region detected by array <scp>CGH</scp>
Parental origin of mutation and the risk of breast cancer in a prospective study of women with a <i><scp>BRCA1</scp></i> or <i><scp>BRCA2</scp></i> mutation
Beckwith–Wiedemann syndrome and long <scp>QT</scp> syndrome due to familial‐balanced translocation t(11;17)(p15.5;q21.3) involving the <i><scp>KCNQ1</scp></i> gene
Hereditary trichilemmal cysts: a proposal for the assessment of diagnostic clinical criteria
<i><scp>BMPR1A</scp></i> mutations in early‐onset colorectal cancer with mismatch repair proficiency
Is multiple<scp>SNP</scp>testing in<i><scp>BRCA2</scp></i>and<i><scp>BRCA1</scp></i>female carriers ready for use in clinical practice? Results from a large Genetic Centre in the<scp>UK</scp>
CCMG statement on gene patents
<i><scp>OCA5,</scp></i> a novel locus for non‐syndromic oculocutaneous albinism, maps to chromosome 4q24
The <scp>M694V</scp> mutation in Armenian‐Americans: a 10‐year retrospective study of <i><scp>MEFV</scp></i> mutation testing for familial Mediterranean fever at <scp>UCLA</scp>
<i><scp>OFD1</scp></i> mutations in males: phenotypic spectrum and ciliary basal body docking impairment
Early‐onset epileptic encephalopathy in a girl carrying a truncating mutation of the <i><scp>ARX</scp></i> gene: rethinking the <i><scp>ARX</scp></i> phenotype in females
Providing predictive testing for Huntington disease via telehealth: results of a pilot study in British Columbia, Canada
Reduced cancer incidence in Huntington's disease: record linkage study clue to an evolutionary trade‐off?
Application of chromosomal microarray in the evaluation of abnormal prenatal findings
Absence of <i><scp>PMS2</scp></i> mutations in colon‐<scp>CFR</scp> participants whose colorectal cancers demonstrate unexplained loss of <scp>MLH1</scp> expression
Identification of the <scp><i>CFTR</i></scp> p.<scp>Phe508Del</scp> founder mutation in the absence of a polythymidine <scp>9T</scp> allele in a Hispanic patient Hispanic
Fragile X‐associated tremor/ataxia syndrome (<scp>FXTAS</scp>) in grey zone carriers
Combining fetal sonography with genetic and allele pathogenicity studies to secure a neonatal diagnosis of Bardet–Biedl syndrome
First successful double‐factor <scp>PGD</scp> for Lynch syndrome: monogenic analysis and comprehensive aneuploidy screening
Caught in the AKT: identification of a <i>de novo</i> pathway in MCAP and MPPH and its therapeutic implications
Investigation of primary microcephaly in Bushehr province of Iran: novel <i><scp>STIL</scp></i> and <i><scp>ASPM</scp></i> mutations
Mutation type and position varies between mosaic and inherited <scp>NF2</scp> and correlates with disease severity
Molecular characterization and clinical presentation of <scp>HK</scp>αα and anti‐<scp>HK</scp>αα alleles in southern Chinese subjects southern Chinese; Guangxi Zhuang Autonomous Region
The effect of fetal androgen metabolism‐related gene variants on external genitalia virilization in congenital adrenal hyperplasia
Genetics of the Charcot‐Marie‐Tooth disease in the Spanish Gypsy population: the hereditary motor and sensory neuropathy‐Russe in depth Gypsy
Evaluating rare coding variants as contributing causes to non‐syndromic cleft lip and palate
Recurrent mutations in<i><scp>DNAJC5</scp></i>cause autosomal dominant Kufs disease
A study of the <scp><i>SCN5A</i></scp> gene in a cohort of 76 patients with Brugada syndrome
Mutation spectrum of <i><scp>RB1</scp></i> gene in Korean bilateral retinoblastoma patients using direct sequencing and gene dosage analysis Korean
Clinical and genetic analysis of patients with X‐linked hyper‐<scp>IgM</scp> syndrome
Exome sequencing identifies <i><scp>UPF3B</scp></i> as the causative gene for a Chinese non‐syndrome mental retardation pedigree Chinese
Craniofacial and dental development in cardio‐facio‐cutaneous syndrome: the importance of Ras signaling homeostasis
Novel missense mutations of <scp>WNK1</scp> in patients with hypokalemic salt‐losing tubulopathies
Novel <scp><i>SLC9A6</i></scp> mutations in two families with Christianson syndrome
Whole exome sequencing identifies a novel <scp><i>DFNA9</i></scp> mutation, <scp>C162Y</scp>
Characterization of <i><scp>NF1</scp></i> allele containing two nonsense mutations in exon 37 that segregates with neurofibromatosis type 1
Physicians’ Perspectives on the Uncertainties and Implications of Chromosomal Microarray Testing of Children and Families
Caught in the AKT: identification of a de novo pathway in MCAP and MPPH and its therapeutic implications
<i>PIK3CA</i>, a hotspot for postzygotic mutations in nonhereditary overgrowth syndromes
Intellectual disability and craniofacial anomalies explained: one more gene associated with Potocki‐Shaffer syndrome
Exploring the utility of whole‐exome sequencing as a diagnostic tool in a child with atypical episodic muscle weakness
<i><scp>MLL2</scp></i> mosaic mutations and intragenic deletion–duplications in patients with Kabuki syndrome
Misdiagnosis of familial Mediterranean fever in patients with Anderson–Fabry disease
Genotype–phenotype studies of <scp>VCP</scp>‐associated inclusion body myopathy with Paget disease of bone and/or frontotemporal dementia
Hereditary hyperferritinemia cataract syndrome in four patients with mutations in the <scp>IRE</scp> of the <i><scp>FTL</scp></i> gene
Detection of genomic deletions of <i><scp>PKP2</scp></i> in arrhythmogenic right ventricular cardiomyopathy
Corrigendum
Nerve growth factor and the physiology of pain: lessons from congenital insensitivity to pain with anhidrosis
<i>Drosophila</i> as a tool for studying the conserved genetics of pain
Painful gains: missense mutations in <i><scp>SCN9A</scp></i> and idiopathic small nerve fibre neuropathy
PS: Pain and sodium channels
Heritability of pain sensitivity and opioid analgesia
Fabry disease in children: agalsidase‐beta enzyme replacement therapy
The <i>BRCA1</i><scp>S1715N</scp> mutation segregates with breast and ovarian cancer in an extended family pedigree
Human Mendelian pain disorders: a key to discovery and validation of novel analgesics
<scp>SOX2</scp> anophthalmia syndrome in adulthood – a neurodegenerative picture?
Clinical and molecular characterization of neonatal diabetes and monogenic syndromic diabetes in Asian Indian children
Twin studies of pain
Genetic aspects of sodium channelopathy in small fiber neuropathy
Corrigendum
Obituary: Dr. David L. Rimoin
Regulating the communication of genetic risk information: the Italian legal approach to questions of confidentiality and disclosure
Pharmacogenetics of pain and analgesia
Opioid genetics: the key to personalized pain control?
Genetic heterogeneity in Pakistani microcephaly families Pakistani; Iran; India; Pakistani founder mutation; Pakistani population
From mouse to humans: discovery of the<scp><i>CACNG2</i></scp>pain susceptibility gene
Primary care physicians' knowledge of and experience with pharmacogenetic testing
A phase 1 enzyme replacement study offers promise for severe hypophospahatasia
Rethinking the genetic basis and inheritance of fascioscapulohumeral muscular dystrophy
New insights into familial diarrhea syndrome
Effects of informed consent for individual genome sequencing on relevant knowledge non‐Hispanic Whites; minority racial/ethnic groups
Parent–child exome sequencing identifiesa <i>de novo</i> truncating mutation in <i>TCF4</i> in non‐syndromic intellectual disability
A prevalent founder mutation and genotype–phenotype correlations of <i>OTOF</i> in Japanese patients with auditory neuropathy Japanese
Autosomal dominant amelogenesis imperfecta associated with <i>ENAM</i> frameshift mutation p.Asn36Ilefs56
Whole genomes in the clinic: uncovering <i>de novo</i> mutations in sporadic infantile epilepsy
Paraspinal ganglioneuroma in the proband of a large family with mild cutaneous manifestations of NF1, carrying a deep <i>NF1</i> intronic mutation
Corrigendum
A role for genetic counsellors and clinical geneticists in pharmacogenetics?
Response to Newman et al.
Detection of <i>BRCA1/2</i> mutations in breast cancer patients from Thailand and Pakistan
An intronic mutation causes severe LGMD2A in a large inbred family belonging to a genetic isolate in the Alps
Two double heterozygotes in a South African Afrikaner family: implications for <i>BRCA1</i> and <i>BRCA2</i> predictive testing Afrikaner
Deficiency of the Y14 protein is a critical factor underlying the etiology of thrombocytopenia with absent radii syndrome
Phenotype in 18 Danish subjects with genetically verified CHARGE syndrome Danish
Co‐morbidity of complex genetic disorders and hypersomnias of central origin: lessons from the underlying neurobiology of wake and sleep
Exome sequencing in a family with an X‐linked lethal malformation syndrome: clinical consequences of hemizygous truncating <i>OFD1</i> mutations in male patients
<i>MEF2C</i> mutations are a rare cause of Rett or severe Rett‐like encephalopathies
A novel frameshift mutation of the <i>GLI3</i> gene in a family with broad thumbs with/without big toes, postaxial polydactyly and variable syndactyly of the hands/feet
Results of Duchenne muscular dystrophy family screening in practice: leaks rather than cascades?
Arterial anomalies in arterial tortuosity syndrome: a sour‐sweet pathology?
Chinese Americans' views of prenatal genetic testing in the genomic era: a qualitative study
Clinical and molecular analysis of RASopathies in a group of Turkish patients "Turkish"; "ethnic population background"
Whole‐genome sequencing and the physician
Balearic archipelago: three islands, three beta‐thalassemia population patterns Iberian populations; Western Mediterranean population
A common tool for rare diseases
Next‐generation sequencing: ready for the clinics?
Direct to consumer genetic testing: a systematic review of position statements, policies and recommendations
Clinical application of 2.7M Cytogenetics array for CNV detection in subjects with idiopathic autism and/or intellectual disability
7q11.23 Microduplication: a recognizable phenotype
The Ehlers–Danlos syndrome, a disorder with many faces
Preimplantation and prenatal genetic diagnosis for androgen insensitivity syndrome resulting from a novel deletion/insertion mutation
Genotype–phenotype correlation in X‐linked Alport syndrome patients carrying missense mutations in the collagenous domain of <i>COL4A5</i>
Familial Breast Cancer
<i>PLP1</i> duplication at the breakpoint regions of an apparently balanced t(X;22) translocation causes Pelizaeus–Merzbacher disease in a girl
Rare occurrence of <i>PALB2</i> mutations in ovarian cancer patients from the Volga‐Ural region
Stüve–Wiedemann syndrome and related bent bone dysplasias
Integrating genomic and epigenomic information: a promising strategy for identifying functional DNA variants of human disease
A puzzle over several decades: eye anomalies with <i>FRAS1</i> and <i>STRA6</i> mutations in the same family
Survey of genetic counselors and clinical geneticists' use and attitudes toward pharmacogenetic testing
Mutations in the <i>NOG</i> gene are commonly found in congenital stapes ankylosis with symphalangism, but not in otosclerosis
46, XY gonadal dysgenesis: new <i>SRY</i> point mutation in two siblings with paternal germ line mosaicism
Hepatic manifestations of tuberous sclerosis complex: a genotypic and phenotypic analysis
Increased incidence of aberrant DNA methylation within diverse imprinted gene loci outside of <i>IGF2</i>/<i>H19</i> in Silver–Russell syndrome
Epigenetics of colorectal cancer
Colon‐specific phenotype in Lynch syndrome associated with <i>EPCAM</i> deletion
<i>CCBE1</i> mutations can cause a mild, atypical form of generalized lymphatic dysplasia but are not a common cause of non‐immune hydrops fetalis
The early‐life social environment and DNA methylation
Childhood adrenocortical carcinoma as a sentinel cancer for detecting families with germline <i>TP53</i> mutations
Female factor IX deficiency due to maternally inherited X‐inactivation
Schizophrenia as variation in the sapiens‐specific epigenetic instruction to the embryo
What does the nature of the <i>MECP2</i> mutation tell us about parental origin and recurrence risk in Rett syndrome?
Deletion of <i>CUL4B</i> leads to concordant phenotype in a monozygotic twin pair
Rapidly screening variants of uncertain significance in the MAP3K1 gene for phenotypic effects
Getting to the bottom of autism spectrum and related disorders: <i>MBD5</i> as a key contributor
It is ALL about the 6p22 histone gene cluster: characterizing the unique genetic features of Down's syndrome patients
A new era of non‐invasive prenatal genetic diagnosis: exploiting fetal epigenetic differences
A novel approach to the management and use of personal genome variants in clinical practice
Aicardi–Goutieres syndrome: from patients to genes and beyond
Epigenetic and genetic disturbance of the imprinted 11p15 region in Beckwith–Wiedemann and Silver–Russell syndromes
The Huntington's Disease health‐related Quality of Life questionnaire (HDQoL): a disease‐specific measure of health‐related quality of life