Clinical Genetics - 2007

210 articles | Last updated: 2025-12-03 14:12:56
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11
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3
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35
Article Title Cauc. White Euro. Other Phrases Used
T A T A T A T A
Cytogenetically invisible microdeletions involving <i>PITX2 </i>in Rieger syndrome
A novel <i>DFNA5 </i>mutation, IVS8+4 A&gt;G, in the splice donor site of intron 8 causes late‐onset non‐syndromic hearing loss in a Chinese family Chinese; Chinese origin
<i>TBX22 </i>mutations are a frequent cause of non‐syndromic cleft palate in the Thai population Thai population; ethnic‐matched control chromosomes
Colorectal cancer survivors undergoing genetic testing for hereditary non‐polyposis colorectal cancer: motivational factors and psychosocial functioning
Identities, frequencies and origins of <i>TMC1</i> mutations causing DFNB7/B11 deafness in Pakistan northern European origins; African‐American
Characterization of <i>de novo </i>microdeletions involving 17q11.2q12 identified through chromosomal comparative genomic hybridization
A novel duplication in the <i>FMR1 </i>gene: implications for molecular analysis in fragile X syndrome and repeat instability
The methylenetetrahydrofolate dehydrogenase (MTHFD1) 1958G&gt;A variant is not associated with spina bifida risk in the Dutch population Dutch population
Contiguous gene deletions involving <i>EFNB1</i>, <i>OPHN1</i>, <i>PJA1</i> and <i>EDA</i> in patients with craniofrontonasal syndrome
Genetic defects underlying Peutz–Jeghers syndrome (PJS) and exclusion of the polarity‐associated <i>MARK/Par1</i> gene family as potential PJS candidates
Do carriers of POLG mutation W748S have disease manifestations?
Susceptibility genes in breast cancer: more is less?
Survival estimates for patients with Machado–Joseph disease (SCA3)
Prenatal diagnosis of Miller‐Dieker syndrome by ultrasound and molecular cytogenetic analysis
Amino acid changes in the amino terminus of the Na,K‐adenosine triphosphatase alpha‐2 subunit associated to familial and sporadic hemiplegic migraine
β‐Globin gene cluster polymorphisms are strongly associated with severity of HbE/β<sup>0</sup>‐thalassemia
Association of the estrogen receptor α gene polymorphisms with osteoporosis in the Mexican population Mexican population; subpopulations
<i>GFAP </i>mutations and polymorphisms in 13 unrelated Italian patients affected by Alexander disease
<i>DMPK</i>‐associated myotonic dystrophy and CTG repeats in Alabama African Americans whites of European descent
Novel VCP mutations in inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia
The role of common genetic risk variants in Parkinson disease
Pallister–Killian syndrome: tetrasomy of 12pter→12p11.22 in a boy with an analphoid, inverted duplicated marker chromosome
Non‐neuronopathic Gaucher disease due to saposin C deficiency
Germline mutation prevalence in the base excision repair gene, <i>MYH</i>, in patients with endometrial cancer
Transcriptional explorations of <i>CAPN3</i> identify novel splicing mutations, a large‐sized genomic deletion and evidence for messenger RNA decay
Genetics of Noonan syndrome – a new gene, and the search is still on
Interstitial deletion of chromosome 4p associated with mild mental retardation, epilepsy and polymicrogyria of the left temporal lobe
Germline<i> hMSH2 </i>promoter mutation in a Chinese HNPCC kindred: evidence for dual role of LOH
The use of genetic testing in hereditary colorectal cancer syndromes: genetic testing in HNPCC, (A)FAP and MAP
Sex‐linked deafness
Sotos syndrome caused by a paracentric inversion disrupting the NSD1 gene
Disruption of the <i>IL1RAPL1</i> gene associated with a pericentromeric inversion of the X chromosome in a patient with mental retardation and autism
Haplogroup analysis supports a pathogenic role for the 7510T&gt;C mutation of mitochondrial tRNA<sup>Ser(UCN)</sup> in sensorineural hearing loss Spanish
A novel arginine‐to‐cysteine substitution in the triple helical region of the α1(I) collagen chain in a family with an osteogenesis imperfecta/Ehlers–Danlos phenotype
Pediatric restrictive cardiomyopathy associated with a mutation in β‐myosin heavy chain
Identification of a novel TGFBR1 mutation in a Loeys–Dietz syndrome type II patient with vascular Ehlers–Danlos syndrome phenotype
Genetic polymorphisms of matrix metalloproteinases in lung, breast and colorectal cancer
Predictors of psychological distress among individuals with a strong family history of malignant melanoma
Novel mutations of the ferroportin gene (<i>SLC40A1</i>): analysis of 56 consecutive patients with unexplained iron overload
A case of lethal hypophosphatasia providing new insights into the perinatal benign form of hypophosphatasia and expression of the ALPL gene
<i>De novo</i> occurrence of the 730insG recurrent mutation in an Italian family with the ichthyotic variant of Vohwinkel syndrome, loricrin keratoderma Italian family
Genetic heterogeneity of autosomal dominant hypercholesterolemia
Analysis of the <i>HFE</i> gene (H63D and C282Y) mutations in patients with iron overload, family members and controls from Antioquia, Northwest Colombia
Polymorphisms spanning the <i>0N</i> exon and promoter of the estrogen receptor‐beta (ERβ) gene <i>ESR2</i> are associated with venous ulceration
High frequency of T130I mutation of <i>HNF4A</i> gene in Mexican patients with early‐onset type 2 diabetes
Restoring reproductive confidence in families with X‐linked mental retardation by finding the causal mutation
<i>Myocardin</i> gene regulatory variants as surrogate markers of cardiac hypertrophy – study in a genetically homogeneous population Cretan ("native Cretan individuals", "Cretan population")
Skeletal dysplasias and the growth plate
Perinatal lethal Gaucher’s disease without prenatal complications
Cultural enhancement of a clinical service to meet the needs of indigenous people; genetic service development in response to issues for New Zealand Maori Eurocentric model; Maori people
Array‐based genotype–phenotype correlation in a case of supernumerary ring chromosome 12
Two novel functional mutations in the Na<sup>+</sup>,K<sup>+</sup>‐ATPase α2‐subunit <i>ATP1A2</i> gene in patients with familial hemiplegic migraine and associated neurological phenotypes
A life without pain? Hedonists take note
When adaptive processes go awry: gain‐of‐function in<i> SCN9A</i>
Dyskeratosis congenita: a genetic disorder of many faces
Use of multiplex ligation‐dependent probe amplification increases the detection rate for 11p15 epigenetic alterations in Silver–Russell syndrome
Mutation and phenotypic spectrum in patients with cardio‐facio‐cutaneous and Costello syndrome
Homozygosity for mutation G212A of the gene for aspartoacylase is associated with atypical form of Canavan’s disease
Synpolydactyly: clinical and molecular advances
Two new patients bearing mutations in the <i>fukutin</i> gene confirm the relevance of this gene in Walker–Warburg syndrome
Restless legs syndrome: an update on genetics and future perspectives
Familial 22q11.2 duplication: a three‐generation family with a 3‐Mb duplication and a familial 1.5‐Mb duplication
Mutations in the familial Mediterranean fever gene of patients with IgA nephropathy and other forms of glomerulonephritis ethnic extraction; ethnically adjusted general population; Jewish extraction
Stickler and branchio‐oto‐renal syndromes in a patient with mutations in<i> EYA1 </i>and<i> COL2A1 </i>genes
Familial Mediterranean Fever in Crete: a genetic and structural biological approach in a population of ‘intermediate risk’ Mediterranean ancestry; Greeks; natives of Crete; Cretan(s); Western/Eastern populations of the Medi
Increased dental caries in people with neurofibromatosis 1
Pure subtelomeric microduplications as a cause of mental retardation
A disease causing deletion of 29 base pairs in intron 15 in the <i>MKS1 </i>gene is highly associated with the campomelic variant of the Meckel–Gruber syndrome individuals of European origin; Finnish, German
<i>Parkin </i>polymorphisms: risk for Parkinson’s disease in Indian population Indian population
Lissencephaly and LIS1: insights into the molecular mechanisms of neuronal migration and development
Cytogenetic and molecular characterization of the derivative Y chromosome: a case study of an azoospermic patient
The genetics of mitral valve prolapse
Insights into the genetics of severe congenital neutropenia
The homozygous deletion of the 3′ enhancer of the <i>SHOX </i>gene causes Langer mesomelic dysplasia
Crouzon with acanthosis nigricans. Further delineation of the syndrome
The impact of array genomic hybridization on mental retardation research: a review of current technologies and their clinical utility
Non‐random maternal X‐chromosome inactivation associated with PHACES
Screening for <i>BRCA1</i> and <i>BRCA2</i> mutations in Eastern Finnish breast/ovarian cancer families Finnish; Eastern Finland; Finnish founder mutations; Finnish breast/ovarian cancer families; relativ
Germinal mosaicism in Simpson‐Golabi‐Behmel syndrome
Identification of proximal 1p36 deletions using array‐CGH: a possible new syndrome
Formalin‐fixed paraffin‐embedded clinical tissues show spurious copy number changes in array‐CGH profiles
Auditory pathology in cri‐du‐chat (5p‐) syndrome: phenotypic evidence for auditory neuropathy
New insights into autism from a comprehensive genetic map
SORL1 adds another piece to the complex puzzle of Alzheimer disease genetics
Unraveling intrinsic genetic factors in type 2 diabetes
Of old and new diseases: genetics of pituitary ACTH excess (Cushing) and deficiency
Detection of copy number changes at the <i>NF1 </i>locus with improved high‐resolution array CGH
A distinct spectrum of<i> SLC26A4 </i>mutations in patients with enlarged vestibular aqueduct in China Chinese; different ethnic background and populations
Early and severe liver disease associated with homozygosity for an exon 7 mutation, G691R, in Wilson’s disease
Development and testing of a decision aid for breast cancer prevention for women with a BRCA1 or BRCA2 mutation
Molecular analysis and long‐term clinical evaluation of three siblings with Alström syndrome consanguineous Turkish family
Movement and mood disorder in two brothers with Gaucher disease
Genetics of autosomal recessive non‐syndromic mental retardation: recent advances
Negative genetic neonatal screening for cystic fibrosis caused by compound heterozygosity for two large <i>CFTR</i> rearrangements
Gene dosage influences the age at onset of SCA2 in a family from southern Italy
Utility of molecular analyses in the exploration of extreme intrafamilial variability in the Marfan syndrome
Frequency of constitutional <i>MSH6 </i>mutations in a consecutive series of families with clinical suspicion of HNPCC
Mosaic paternally derived inv dup(15) may partially rescue the Prader–Willi syndrome phenotype with uniparental disomy
Prevalence of <i>CYP1B1 </i>mutations in Australian patients with primary congenital glaucoma ethnic populations
Combined UGT1A1 and UGT1A7 variant alleles are associated with increased risk of Gilbert’s syndrome in Taiwanese adults
The importance of the fetal origins of adult disease for geneticists
Endocardial cushion defect in a patient with Crouzon syndrome carrying a mutation in the fibroblast growth factor receptor (<i>FGFR</i>)‐2 gene
Sweet successes in diabetes genetics
Frequency of hemochromatosis gene (<i>HFE</i>) mutations in Corsica (France)
Audioprofiling identifies <i>TECTA</i> and<i> GJB2</i>‐related deafness segregating in a single extended pedigree
Novel mutations in the pejvakin gene are associated with autosomal recessive non‐syndromic hearing loss in Iranian families
Cystic fibrosis in a southern Brazilian population: characteristics of 90% of the alleles among Europeans and their descendants; Mediterranean component
<i>CYR61 </i>polymorphisms are associated with plasma HDL‐cholesterol levels in obese individuals
Familial CHARGE syndrome because of <i>CHD7</i> mutation: clinical intra‐ and interfamilial variability
Fidelity of whole‐genome amplification of blood spot DNA for HLA typing and SNP analyses
Distribution and frequencies of <i>CDH23 </i>mutations in Japanese patients with non‐syndromic hearing loss Japanese; Eastern Asian populations
The effect of enzyme replacement therapy on bone crisis and bone pain in patients with type 1 Gaucher disease
Exceptional good cognitive and phenotypic profile in a male carrying a mosaic mutation in the <i>FMR1</i> gene
Clinical score of 62 Italian patients with Cornelia de Lange syndrome and correlations with the presence and type of <i>NIPBL</i> mutation
Response to Cohen
Mutations in the <i>CHX10</i> gene in non‐syndromic microphthalmia/anophthalmia patients from Qatar
Validation study of the <scp>lambda</scp> model for predicting the <i>BRCA1</i> or <i>BRCA2</i> mutation carrier status of North American Ashkenazi Jewish women Ashkenazi Jewish (AJ)
High‐resolution oligonucleotide array‐CGH applied to the detection and characterization of large rearrangements in the hereditary breast cancer gene <i>BRCA1</i>
A comparison of counselee and counselor satisfaction in reproductive genetic counseling
Frequency of Von Hippel‐Lindau germline mutations in classic and non‐classic Von Hippel‐Lindau disease identified by DNA sequencing, Southern blot analysis and multiplex ligation‐dependent probe ampli
Identification of a novel pseudodeficiency allele in the <i>GLB1</i> gene in a carrier of GM1 gangliosidosis Basque; non-Basque; Basque Country in the north of Spain
Screening of mutations in the <i>PHF8</i> gene and identification of a novel mutation in a Finnish family with XLMR and cleft lip/cleft palate Finnish
Mutation analysis of the genes involved in the Ras‐mitogen‐activated protein kinase (MAPK) pathway in Korean patients with Noonan syndrome
Executive functioning in children and adolescents with phenylketonuria
Congenital myasthenic syndrome caused by two non‐N88K rapsyn mutations
Exclusive cardiac dysfunction in familial primary carnitine deficiency cases: a genotype–phenotype correlation
Does cystic fibrosis neonatal screening detect atypical CF forms? Extended genetic characterization and 4‐year clinical follow‐up
17p11.2p12 triplication and del(17)q11.2q12 in a severely affected child with dup(17)p11.2p12 syndrome
A novel mutation in the <i>PHF8 </i>gene is associated with X‐linked mental retardation with cleft lip/cleft palate
Large deletions in the CFTR gene: clinics and genetics in Swiss patients with CF
Development of cortical GABAergic circuits and its implications for neurodevelopmental disorders
Cognition genes on autosomes: the paradox
Genetic testing for heart disease susceptibility: potential impact on motivation to quit smoking
Imiglucerase (Cerezyme<sup>®</sup>) improves quality of life in patients with skeletal manifestations of Gaucher disease
Constitutive deficiency in DNA mismatch repair: is it time for Lynch III?
Assessment of the prevalence of the 985A&gt;G MCAD mutation in the French‐Canadian population using allele‐specific PCR northwestern European populations; French-Canadians
The position of the mutation within the <i>LMNA </i>gene determines the type and extent of tissue involvement in laminopathies
Response to Landires et al.
Localization of a novel autosomal recessive hypotrichosis locus (LAH3) to chromosome 13q14.11–q21.32
Transmission of familial Mediterranean fever mutations following bone marrow transplantation
Multiplex ARMS analysis to detect 13 common mutations in familial hypercholesterolaemia
Gender, genotype, and phenotype differences in Smith–Magenis syndrome: a meta‐analysis of 105 cases
Constitutive deficiency in DNA mismatch repair
Abnormal brain structure in adults with Van der Woude syndrome
Glucose metabolism and insulin secretion in a patient with <i>ABCC8 </i>mutation and Fanconi–Bickel syndrome caused by maternal isodisomy of chromosome 3
A genome‐wide linkage scan for iron phenotype quantitative trait loci: the HEIRS Family Study race/ethnicity
Spectrum of mutations and variants/haplotypes of CFTR and genotype–phenotype correlation in idiopathic chronic pancreatitis and controls in Chinese by complete analysis Chinese; ethnically matched; other ethnic groups; population-specific; populations; Taiwan
Schizophrenia in a patient with subtelomeric duplication of chromosome 22q
The presence of germ line mosaicism in cleidocranial dysplasia
Aplasia cutis congenita, congenital heart lesions, and frontonasal cysts in four successive generations
<i>LRRK2</i> G6055A mutation in Italian patients with familial or sporadic Parkinson’s disease
The p.R1109X mutation in <i>SH3TC2</i> gene is predominant in Spanish Gypsies with Charcot–Marie–Tooth disease type 4 European Gypsy population; Gypsy; Spanish Gypsies
A novel homozygous frameshift deletion c.471del of <i>HFE</i> associated with hemochromatosis
Genetic counselling and consent for tumour testing in HNPCC
Psychopathology and personality aspects of adults with Möbius sequence
Current status of thalassemia in minority populations in Guangxi, China
Toward understanding the genetic basis of neural tube defects
Identification of novel <i>BRCA</i> large genomic rearrangements in Singapore Asian breast and ovarian patients with cancer Western European descent; Asian populations
Tuberous sclerosis complex and myocardial fat‐containing lesions: a report of four cases
Should NF2 mutation screening be undertaken in patients with an apparently isolated vestibular schwannoma?
Association of <i>TCF7L2</i> polymorphisms with type 2 diabetes in Mexico City Spanish from Valencia; European and West African populations; Native Americans; Nigerians (Bini from the Edo region); East Asian and Native American populations;
The Ala53Thr mutation in the α‐synuclein gene in a Korean family with Parkinson disease
Niemann–Pick C disease: functional characterization of three NPC2 mutations and clinical and molecular update on patients with NPC2
Late‐onset GSDII with novel <i>GAA</i> gene mutation
The common genetic variant upstream of <i>INSIG2</i> gene is not associated with obesity in Indian population
A new familial mutation (R133G) in the <i>SRY</i> gene
Germline mutations in <i>APC</i> and <i>MUTYH</i> are responsible for the majority of families with attenuated familial adenomatous polyposis
Single‐nucleotide polymorphisms in the <i>COL1A1</i> regulatory regions are associated with otosclerosis
Mitochondrial deafness
Contribution of the <i>CFTR</i> gene, the pancreatic secretory trypsin inhibitor gene (<i>SPINK1</i>) and the cationic trypsinogen gene (<i>PRSS1</i>) to the etiology of recurrent pancreatitis Greek patients; healthy Greek subjects
Clinical findings and molecular characterization of six subtelomeric imbalances
The Huntington’s Disease quality of life battery for carers: reliability and validity
An overview of isolated and syndromic oesophageal atresia
<i>RMRP</i> mutations in hematological disorders
Isolated cystinuria (OMIM 238200) is not a separate entity but is caused by a mutation in the cystinuria gene <i>SLC7A9</i>
Newborn screening for mucopolysaccharidoses: opinions of patients and their families
<i>MEFV</i> alterations and population genetics analysis in a large cohort of Greek patients with familial Mediterranean fever Greeks; Mediterranean Basin populations; eastern rather than western populations of the Mediterranea
Loss‐of‐function mutations in the Na<sub>v</sub>1.7 gene underlie congenital indifference to pain in multiple human populations
Cystic fibrosis mutational spectrum and genotypic/phenotypic features in Greek‐Cypriots, with emphasis on dehydration as presenting symptom
Expanding the phenotypic spectrum of Caffey disease
Managing genetic discrimination: Strategies used by individuals found to have the Huntington disease mutation
Corrigendum
Mitochondrial GTPase mitofusin 2 mutations in Korean patients with Charcot‐Marie‐Tooth neuropathy type 2 Korean
The effect of enzyme replacement therapy on bone crisis and bone pain in patients with type 1 Gaucher disease
<i>SIL1 </i>and <i>SARA2 </i>mutations in Marinesco‐Sjögren and chylomicron retention diseases
Identification of 14 novel <i>GLB1 </i>mutations, including five deletions, in 19 patients with GM1 gangliosidosis from South America Gypsy
Associations between USF1 gene variants and cardiovascular risk factors in the Quebec Family Study French Canadians
Corrigendum
Corrigendum
<i>HRAS </i>and the Costello syndrome
A possible bichromatid mutation in a male gamete giving rise to a female mosaic for two different mutations in the X‐linked gene<i> WAS</i>
Gene discovery in craniofacial development and disease – cashing in your chips
Novel<i> LMNA </i>mutations seen in patients with familial partial lipodystrophy subtype 2 (FPLD2; MIM 151660)
A comparison of community, clinician, and patient preferences for naming a cancer‐related mutation
Three children with Milroy disease and<i> de novo </i>mutations in<i> VEGFR3</i>
Young people’s experiences of growing up in a family affected by Huntington’s disease
A novel mutation at the DFNA36 hearing loss locus reveals a critical function and potential genotype–phenotype correlation for amino acid‐572 of TMC1
A new locus for autosomal recessive non‐syndromic mental retardation maps to 1p21.1–p13.3
Interstitial deletions of chromosome 6q: genotype–phenotype correlation utilizing array CGH
<i>MLH1 </i>germline epimutations in selected patients with early‐onset non‐polyposis colorectal cancer
Disruption of Friend of GATA 2 gene (<i>FOG‐2</i>) by a <i>de novo </i>t(8;10) chromosomal translocation is associated with heart defects and gonadal dysgenesis
A family with lymphoedema‐distichiasis where identical twins have a discordant phenotype
Interactions and associations of paraoxonase gene cluster polymorphisms with myocardial infarction in a Pakistani population
Array comparative genomic hybridization for diagnosis of developmental delay – an exploratory cost‐consequences analysis
1.5 Mb<i> de novo </i>22q11.21 microduplication in a patient with cognitive deficits and dysmorphic facial features
A survey of preventive measures among<i> BRCA1 </i>mutation carriers from Poland Polish women
Germline novel<i> MSH2 </i>deletions and a founder<i> MSH2 </i>deletion associated with anticipation effects in HNPCC Italian; northeastern Italy
Effectiveness of the <scp>crcapro</scp> program in identifying patients suspected for HNPCC
Contribution of BRCA1 and BRCA2 germline mutations to the incidence of early‐onset breast cancer in Cyprus
Novel germline and somatic mutations of the<i> MSH2 </i>gene in hereditary non‐polyposis colorectal cancer