Journal of Medical Genetics - 2002

306 articles | Last updated: 2025-12-03 14:12:57
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T A T A T A T A
Mismatch repair gene analysis in Catalonian families with colorectal cancer
Mutation analysis in the candidate Mobius syndrome genes PGT and GATA2 on chromosome 3 and EGR2 on chromosome 10
SHOX point mutations and deletions in Leri-Weill dyschondrosteosis
Serrated adenoma and APC gene mutation
Deletion of 9p associated with gonadal dysfunction in 46,XY but not in 46,XX human fetuses
Predictive testing in the context of pregnancy: experience in Huntington's disease and autosomal dominant cerebellar ataxia
Management of women with a family history of breast cancer in the North West Region of England: training for implementing a vision of the future
Human T and risk for neural tube defects
Molecular changes in skin predict predisposition to breast cancer
Genetics for Haematologists: The Molecular Genetic Basis of Haematological Disorders: Wadie F Bahou. ReMEDICA Genetics for . . . Series. Series Editor, Eli Hatchwell. (Pp 142; pound25.) London: ReMEDI
Exclusion of PTEN, CTNNB1, and PTCH as candidate genes for Birt-Hogg-Dube syndrome
Cancers of the small and large bowel originate differently
Reproductive counselling for women with myotonic dystrophy
A variant of osteogenesis imperfecta type IV with resolving kyphomelia is caused by a novel COL1A2 mutation
Molecular characterisation of a ring chromosome 22 in a patient with severe language delay: a contribution to the refinement of the subtelomeric 22q deletion syndrome
Familial adult renal neoplasia
Dysmorphic Syndromes and Constitutional Diseases of the Skeleton : G Canepa, P Maroteaux, V Pietrogrande. Padova: Piccin Nuova Libraria, 2001.
Association of Crohn's disease and ulcerative colitis with haplotypes of the MLH1 gene in Italian inflammatory bowel disease patients Italian
A novel locus for brachydactyly type A1 on chromosome 5p13.3-p13.2
Hirschsprung disease and L1CAM: is the disturbed sex ratio caused by L1CAM mutations?
Antiepileptic drug therapy during pregnancy: the neurologist's perspective
Dominant X linked retinitis pigmentosa is frequently accounted for by truncating mutations in exon ORF15 of the RPGR gene
Major difference in aetiology and phenotypic abnormalities between transient and permanent neonatal diabetes
An aetiological study of 25 mentally retarded adults with autism
A new MRXS locus maps to the X chromosome pericentromeric region: a new syndrome or narrow definition of Sutherland-Haan genetic locus?
Genetic characterisation of patients with multiple colonic polyps
Association of polymorphisms and allelic combinations in the tumour necrosis factor-alpha-complement MHC region with coronary artery disease
Craniofacial anomalies, cataracts, congenital heart disease, sacral neural tube defects, and growth and developmental retardation in two sisters: a new autosomal recessive MCA/MR syndrome?
ATM mutations in Finnish breast cancer patients Finnish
Adrenal Disease in Childhood. Clinical and Molecular Aspects : Editors Ieuan A Hughes, Adrian J L Clark. Basle: Karger. 2001. ISBN 1421/7082.
Limited role for HLA DR15 in MS
Mutations in the pancreatic secretory trypsin inhibitor gene (PSTI/SPINK1) rather than the cationic trypsinogen gene (PRSS1) are significantly associated with tropical calcific pancreatitis
Robinow syndrome
Corpus callosum hypoplasia and associated brain anomalies in Nijmegen breakage syndrome
Paternal contribution to the risk for pre-eclampsia
A common ancestral haplotype in carrier chromosomes from different ethnic backgrounds in vacuolating megalencephalic leucoencephalopathy with subcortical cysts
Absence of 22q11 deletions in 211 patients with developmental delay analysed using PCR
In vitro analysis of aminoglycoside therapy for the Arg120stop nonsense mutation in RP2 patients
Colour Atlas of Genetics: 2nd edition. Eberhard Passarge. (Pp 457.) Stuttgart: Thieme. 2001. ISBN 3-13-100362-6. New York: Thieme. 2001. ISBN 0-86577-958-9.
Okihiro syndrome and acro-renal-ocular syndrome: clinical overlap, expansion of the phenotype, and absence of PAX2 mutations in two new families
Spinocerebellar ataxia and the A3243G and A8344G mtDNA mutations
National study of microphthalmia, anophthalmia, and coloboma (MAC) in Scotland: investigation of genetic aetiology
Identification of a 52 kb deletion downstream of the SOST gene in patients with van Buchem disease
Novel mutations in the gamma-crystallin genes cause autosomal dominant congenital cataracts
ARC syndrome is not so rare
Sensitivity of conformation sensitive gel electrophoresis in detecting mutations in Marfan syndrome and related conditions
Supportive evidence for the DYX3 dyslexia susceptibility gene in Canadian families
Corneal dystrophy and perceptive deafness (Harboyan syndrome): CDPD1 maps to 20p13
Dysmorphism, variable overgrowth, normal bone age, and severe developmental delay: a "Sotos-like" syndrome?
Omphalocele in three generations with autosomal dominant transmission
A T to C mutation in the polypyrimidine tract of the exon 9 splicing site of the RB1 gene responsible for low penetrance hereditary retinoblastoma
High frequency of skewed X inactivation in young breast cancer patients
Severe phenotype in Angelman syndrome resulting from paternal isochromosome 15
Leber hereditary optic neuropathy
Organisation of the pericentromeric region of chromosome 15: at least four partial gene copies are amplified in patients with a proximal duplication of 15q
A genome wide scan for familial high myopia suggests a novel locus on chromosome 7q36
Specific haplotypes of the RET proto-oncogene are over-represented in patients with sporadic papillary thyroid carcinoma
Naturally occurring mutations and functional polymorphisms in multidrug resistance 1 gene: correlation with microsatellite instability and lymphoid infiltration in colorectal cancers
Genes versus environment in ocular refraction
Gene carriers face other risks
A single base alteration in the CGG repeat region of FMR1: possible effects on gene expression and phenotype
Mosaicism for FMR1 and FMR2 deletion: a new case
Genome screening of coeliac disease
Guilt by association?
Alport syndrome and mental retardation: clinical and genetic dissection of the contiguous gene deletion syndrome in Xq22.3 (ATS-MR)
A locus for isolated cataract on human Xp
Constitutional de novo interstitial deletion of 8 Mb on chromosome 22q12.1-12.3 encompassing the neurofibromatosis type 2 (NF2) locus in a dysmorphic girl with severe malformations
Catalogue of Unbalanced Chromosome Aberrations in Man: 2nd edition. Albert Schinzel. (Pp 966; SFr 397.) Berlin: Walter de Gruyter, 2001. ISBN 3-11-011607-3.
Unique de novo mutation of BRCA2 in a woman with early onset breast cancer
Spastic paraparesis and atypical dementia caused by PSEN1 mutation (P264L), responsible for Alzheimer's disease
Refined mapping of the gene for otopalatodigital syndrome type I
Inositol and folate resistant neural tube defects
Branchio-oculo-facial syndrome and branchio-otic/branchio-oto-renal syndromes are distinct entities
A single amino acid substitution (D1441Y) in the carboxyl-terminal propeptide of the proalpha1(I) chain of type I collagen results in a lethal variant of osteogenesis imperfecta with features of dense
Prevalence of SDHB, SDHC, and SDHD germline mutations in clinic patients with head and neck paragangliomas
Genetics for Dermatologists: The Molecular Genetic Basis of Dermatological Disorders: Sherri J Bale. (pound25, $40.) London: ReMEDICA Publishing. 2000. ISBN 0 901346 10 2
Genes other than BRCA1 and BRCA2 involved in breast cancer susceptibility
Investigation of the GRB2, GRB7, and CSH1 genes as candidates for the Silver-Russell syndrome (SRS) on chromosome 17q
Molecular genetic analysis of the NF2 gene in young patients with unilateral vestibular schwannomas
Malignant peripheral nerve sheath tumours in neurofibromatosis 1
Cystatin C gene and exudative age related macular degeneration are linked
Comparative genomic hybridisation shows a partial de novo deletion 16p11.2 in a neonate with multiple congenital malformations
Identification and characterisation of mutations underlying Sanfilippo syndrome type B (mucopolysaccharidosis type IIIB)
A case of Costello syndrome and glycogen storage disease type III
Prader-Willi syndrome and a deletion/duplication within the 15q11-q13 region
Covert operations
Changes in frequencies of heterozygous thermolabile 5,10-methylenetetrahydrofolate reductase gene in fetuses with neural tube defects
Current status of human chromosome 14
DTDST mutations are not a frequent cause of idiopathic talipes equinovarus (club foot)
The fibulin-1 gene (FBLN1) is disrupted in a t(12;22) associated with a complex type of synpolydactyly
Human gene mutations causing infertility
A Val227Ala polymorphism in the peroxisome proliferator activated receptor alpha (PPARalpha) gene is associated with variations in serum lipid levels
Correction
Phenotypic effects of mosaicism for a 47,XXX cell line in Turner syndrome
Another risk factor for MI?
Hereditary non-polyposis colorectal cancer: current risks of colorectal cancer largely overestimated
Familial pericentric inversion of chromosome 5 in a family with benign neonatal convulsions
The null oncogene hypothesis and protection from cancer
A school based study of children with learning disability indicates poor levels of genetic investigation
Maternal MTHFR genotype contributes to the risk of non-syndromic cleft lip and palate
Preconceptional couple screening for cystic fibrosis carrier status: couples prefer full disclosure of test results
Testing for osteogenesis imperfecta in cases of suspected non-accidental injury
An investigation of ACE as a risk factor for dementia and cognitive decline in the general population
Relationship between genotype and phenotype for the CFTR gene W846X mutation
Novel autosomal dominant mandibulofacial dysostosis with ptosis: clinical description and exclusion of TCOF1
Localisation of the Y chromosome stature gene to a 700 kb interval in close proximity to the centromere
When HFE gene mutation doesn't make sense
Infantile spinal muscular atrophy variant with congenital fractures in a female neonate: evidence for autosomal recessive inheritance
Contribution of BRCA2 germline mutations to hereditary breast/ovarian cancer in Germany
Four novel mutations in the OFD1 (Cxorf5) gene in Finnish patients with oral-facial-digital syndrome 1 Finnish
An MLH1 haplotype is over-represented on chromosomes carrying an HNPCC predisposing mutation in MLH1
A patient with a supernumerary marker chromosome (15), Angelman syndrome, and uniparental disomy resulting from paternal meiosis II non-disjunction
Identification of cryptic splice site, exon skipping, and novel point mutations in type I CD36 deficiency
Assessing epidemiological evidence for the teratogenic effects of anticonvulsant medications
CTLA-4/CD28 gene region is associated with genetic susceptibility to coeliac disease in UK families
Preimplantation genetic diagnosis in clinical practice
Partial trisomy 9p12p21.3 with a normal phenotype
The frequency of mtDNA 8994 polymorphism and detection of the NARP 8993 mutation
Chromosome 10p13-14 and 22q11 deletion screening in 100 patients with isolated and syndromic conotruncal heart defects
Association between markers in chromosomal region 17q23 and young onset hypertension: a TDT study
Acrofacial dysostosis in a patient with the TSC2-PKD1 contiguous gene syndrome
Dilated cardiomyopathy, sudden cardiac death, hypoplastic discs, and retinal detachment: a new autosomal dominant syndrome
Distinctive audiometric features between USH2A and USH2B subtypes of Usher syndrome
A late onset variant of ataxia-telangiectasia with a compound heterozygous genotype, A8030G/7481insA
Automated fluorescent genotyping detects 10% of cryptic subtelomeric rearrangements in idiopathic syndromic mental retardation
Partial deletion of 4p and 4q in a fetus with ring chromosome 4: phenotype and molecular mapping of the breakpoints
Functional characterisation of MeCP2 mutations found in male patients with X linked mental retardation
Long term health and neurodevelopment in children exposed to antiepileptic drugs before birth
Identification of mutations in the gene encoding sterol regulatory element binding protein (SREBP)-2 in hypercholesterolaemic subjects
TNF2 turns up in non-ischaemic heart failure
Abnormal expression of the KLF8 (ZNF741) gene in a female patient with an X;autosome translocation t(X;21)(p11.2;q22.3) and non-syndromic mental retardation
The teratogenicity of anticonvulsant drugs: a progress report
Genes predict outcome in multiple sclerosis
New mutations in hereditary pancreatitis
HLA-DR4 and risk of spondyloarthropathy
Chromosome 2 aberrations in clinical cases characterised by high resolution multicolour banding and region specific FISH probes
Close relatives: distant relations
Molecular studies in 10 cases of Rubinstein-Taybi syndrome, including a mild variant showing a missense mutation in codon 1175 of CREBBP
Q829X, a novel mutation in the gene encoding otoferlin (OTOF), is frequently found in Spanish patients with prelingual non-syndromic hearing loss
Maternal uniparental disomy 12 in a healthy girl with a 47,XX,+der(12)(:p11->q11:)/46,XX karyotype
Risk assessment and management of high risk familial breast cancer
Transient neonatal diabetes, a disorder of imprinting
Thrombocytopenia-absent radius syndrome: a clinical genetic study
Mutations of the UMOD gene are responsible for medullary cystic kidney disease 2 and familial juvenile hyperuricaemic nephropathy
Sorting nexin 3 (SNX3) is disrupted in a patient with a translocation t(6;13)(q21;q12) and microcephaly, microphthalmia, ectrodactyly, prognathism (MMEP) phenotype
Genetic variants of NHEJ DNA ligase IV can affect the risk of developing multiple myeloma, a tumour characterised by aberrant class switch recombination
Unlinked genes influence severity of FAP within families
Clinical and genetic studies of Birt-Hogg-Dube syndrome
SNP S1103Y in the cardiac sodium channel gene SCN5A is associated with cardiac arrhythmias and sudden death in a white family
Evolution and expression of FOXL2
Longevity in Schimke immuno-osseous dysplasia
Relationship between clinical and genetic diagnosis of Prader-Willi syndrome
Report of a new case of "genitopatellar" syndrome which challenges the importance of absent patellae as a defining feature
Calculating predictive values for the large repeat alleles at the SCA8 locus in patients with ataxia
Germline mutation of the tumour suppressor PTEN in Proteus syndrome
Low rate of TP53 germline mutations in breast cancer/sarcoma families not fulfilling classical criteria for Li-Fraumeni syndrome
Physical and psychomotor development of 1799 children born after second trimester amniocentesis for maternal serum positive triple test screening and normal prenatal karyotype
Behavioural phenotype of Bardet-Biedl syndrome
Autosomal dominant (AD) pure spastic paraplegia (HSP) linked to locus SPG4 affects almost exclusively males in a large pedigree
PRKAR1A, one of the Carney complex genes, and its locus (17q22-24) are rarely altered in pituitary tumours outside the Carney complex
Hereditary duplication of proximal chromosome 1q (q11q22) in a patient with T lymphoblastic lymphoma/leukaemia: a family study using G banding and comparative genomic hybridisation
A comparative study between infertile males and patients with Turner syndrome to determine the influence of sex chromosome mosaicism and the breakpoints of structurally abnormal Y chromosomes on pheno
Frequency of mutations in the early growth response 2 gene associated with peripheral demyelinating neuropathies
Maternally inherited non-syndromic hearing impairment in a Spanish family with the 7510T>C mutation in the mitochondrial tRNASer(UCN) gene
Correlation between a specific Wilms tumour suppressor gene (WT1) mutation and the histological findings in Wilms tumour (WT)
Comparison of genetic services with and without genetic registers: knowledge, adjustment, and attitudes about genetic counselling among probands referred to three genetic clinics
Comparison of genetic services with and without genetic registers: access and attitudes to genetic counselling services among relatives of genetic clinic patients
Screening for microsatellite instability target genes in colorectal cancers
High throughput screening of human subtelomeric DNA for copy number changes using multiplex amplifiable probe hybridisation (MAPH)
De novo GLI3 mutation in acrocallosal syndrome: broadening the phenotypic spectrum of GLI3 defects and overlap with murine models
Two novel frameshift mutations in NKX2.5 result in novel features including visceral inversus and sinus venosus type ASD
Childhood onset mitochondrial myopathy and lactic acidosis caused by a stop mutation in the mitochondrial cytochrome c oxidase III gene
Bar code screening on combed DNA for large rearrangements of the BRCA1 and BRCA2 genes in French breast cancer families
Identification of novel CLN2 mutations shows Canadian specific NCL2 alleles
Family history of breast cancer as a determinant of the risk of developing endometrial cancer: a nationwide cohort study
Genetic testing in hereditary non-polyposis colorectal cancer families with a MSH2, MLH1, or MSH6 mutation
Linkage to 18qter differentiates two clinically overlapping syndromes: congenital cataracts-facial dysmorphism-neuropathy (CCFDN) syndrome and Marinesco-Sjogren syndrome
Promoter polymorphisms in the CD14 receptor gene and their potential association with the severity of chronic periodontitis
Protein losing enteropathy-hepatic fibrosis syndrome in Saguenay-Lac St-Jean, Quebec is a congenital disorder of glycosylation type Ib
Severe digital abnormalities in a patient heterozygous for both a novel missense mutation in HOXD13 and a polyalanine tract expansion in HOXA13
Behavioural complaints in participants who underwent predictive testing for Huntington's disease
Your Genetic Destiny: Know Your Genes, Secure your Health, Save Your Life: Aubrey Milunsky. Oxford: Perseus Publishing. 2001. ISDN 0738203777.
Hereditary ovarian cancer resulting from a non-ovarian cancer cluster region (OCCR) BRCA2 mutation: is the OCCR useful clinically?
Continuous medical education approaches for clinical genetics: a postal survey of general practitioners
No association between HOXA1 and HOXB1 genes and autism spectrum disorders (ASD)
Monozygotic twins with chromosome 22q11 deletion and discordant phenotypes: updates with an epigenetic hypothesis
Splice mutation 1811+1.6kbA>G causes severe cystic fibrosis with pancreatic insufficiency: report of 11 compound heterozygous and two homozygous patients
CYS127S (FH-Kairouan) and D245N (FH-Tozeur) mutations in the LDL receptor gene in Tunisian families with familial hypercholesterolaemia Tunisian families
Novel ATP6V1B1 and ATP6V0A4 mutations in autosomal recessive distal renal tubular acidosis with new evidence for hearing loss
Mosaicism for duplication 12q (12q13->12q21.2) accompanied by a pericentric inversion in a dysmorphic female infant
Coffin-Lowry syndrome: clinical and molecular features
Homozygosity mapping of a Dyggve-Melchior-Clausen syndrome gene to chromosome 18q21.1
Autosomal recessive primary microcephaly: an analysis of locus heterogeneity and phenotypic variation
Variant of endothelial nitric oxide synthase gene determines severity of atherosclerosis
Genotype-phenotype relationships in Berardinelli-Seip congenital lipodystrophy
"Molecular rulers" for calibrating phenotypic effects of telomere imbalance
Genetic testing and genetic counselling in hypertrophic cardiomyopathy: the French experience
Description and functional analysis of a novel in frame mutation linked to hereditary non-polyposis colorectal cancer
Constitutional alterations of the ATM gene in early onset sporadic breast cancer
A novel aberrant splice site mutation in the APC gene
Prevalence of mutations in the short stature homeobox containing gene (SHOX) in Madelung deformity of childhood
Heterozygous P250L mutation of fibroblast growth factor receptor 3 in a case of isolated craniosynostosis
Streptococcal infection distinguishes different types of psoriasis
8p23 duplication reconsidered: is it a true euchromatic variant with no clinical manifestation?
Supernumerary marker chromosomes (SMC) and uniparental disomy (UPD): coincidence or consequence?
Education improves general practitioner (GP) management of familial breast/ovarian cancer: findings from a cluster randomised controlled trial
The Century of Mendelism: Editors Robert A Peel, John Timson. London: The Galton Institute. 2000. ISBN 0 950 40666 X.
Myotonic Dystrophy: 3rd ed. Major Problems in Neurology Series. Peter S Harper. (pound55.00.) London: Harcourt. 2001. ISBN 0 7020 21520
Oxford Medical Databases: London Dysmorphology Database Version 3.0. London Neurogenetics Database Version 3.0. Dysmorphology Photo Library on CD-ROM Version 3.0: R M Winter, M Baraitser. (CD, pound15
Fragile X Syndrome - Diagnosis, Treatment and Research: Third edition. Editors Randi Jenssen Hagerman, Paul J Hagerman. pound65.50 HB, pound31.00 PB. Baltimore: The Johns Hopkins University Press. 200
Molecular screening for Smith-Magenis syndrome among patients with mental retardation of unknown cause
Pure terminal duplication of the short arm of chromosome 19 in a boy with mild microcephaly
A rare case of a de novo dup(19q) associated with a mild phenotype
A novel atypical 22q11.2 distal deletion in father and son
Cytogenetic analysis of spermatozoa from males aged between 47 and 71 years
SDHB mutation analysis in familial and sporadic phaeochromocytoma identifies a novel mutation
Germline MSH2 and MLH1 mutational spectrum in HNPCC families from Poland and the Baltic States
Novel homozygous mutation in the alpha subunit of the rod cGMP gated channel (CNGA1) in two Spanish sibs affected with autosomal recessive retinitis pigmentosa
Familial expansile osteolysis in a large Spanish kindred resulting from an insertion mutation in the TNFRSF11A gene
APC gene mutation does not predict duodenal cancer
Deletion of the OPA1 gene in a dominant optic atrophy family: evidence that haploinsufficiency is the cause of disease
Twelve novel FBN1 mutations in Marfan syndrome and Marfan related phenotypes test the feasibility of FBN1 mutation testing in clinical practice
A significant response to neoadjuvant chemotherapy in BRCA1/2 related breast cancer
Counseling About Cancer: Strategies for Genetic Counseling: 2nd edition. Katherine A Schneider. (Pp 333; $39.95.) New York: Wiley-Liss. 2002. ISDN 0-471-37036-3.
FOXL2 mutation screening in a large panel of POF patients and XX males
A necropsy case of Denys-Drash syndrome with a WT1 mutation in exon 7
Cardiovascular manifestations in 75 patients with Williams syndrome
MECP2 gene nucleotide changes and their pathogenicity in males: proceed with caution
The phenotype of survivors of campomelic dysplasia
UGT1A7 and colorectal cancer susceptibility
Incidence of non-founder BRCA1 and BRCA2 mutations in high risk Ashkenazi breast and ovarian cancer families
A breast cancer family from Spain with germline mutations in both the BRCA1 and BRCA2 genes
Multifocal glomus tumours of the fingers in two patients with neurofibromatosis type 1
Spastin gene mutation in Japanese with hereditary spastic paraplegia Japanese
Characterisation of the growth regulating gene IMP3, a candidate for Silver-Russell syndrome
Deletion of the SIM1 gene (6q16.2) in a patient with a Prader-Willi-like phenotype
Analysis of the p63 gene in classical EEC syndrome, related syndromes, and non-syndromic orofacial clefts
Coeliac disease in twins
A novel locus for autosomal dominant non-syndromic deafness (DFNA41) maps to chromosome 12q24-qter
PTPN11 mutations in LEOPARD syndrome
Silver-Russell phenotype in a patient with pure trisomy 1q32.1-q42.1: further delineation of the pure 1q trisomy syndrome
Coffin-Lowry phenotype in a patient with a complex chromosome rearrangement
Clinical and molecular features of the immunodysregulation, polyendocrinopathy, enteropathy, X linked (IPEX) syndrome
Prospective screening for subtelomeric rearrangements in children with mental retardation of unknown aetiology: the Amsterdam experience
Dysosteosclerosis: a report of three new cases and evolution of the radiological findings
Interstitial telomeres of an inv(9)(p11.2;q34) involved in a jumping translocation found in a woman through a stable unbalanced translocation in her malformed child
Hereditary paraganglioma targets diverse paraganglia
Fraser syndrome and cryptophthalmos: review of the diagnostic criteria and evidence for phenotypic modules in complex malformation syndromes
Identification of a locus for a form of spondyloepiphyseal dysplasia on chromosome 15q26.1: exclusion of aggrecan as a candidate gene
Identification of candidate lung cancer susceptibility genes in mouse using oligonucleotide arrays
Mapping of a novel locus for achromatopsia (ACHM4) to 1p and identification of a germline mutation in the alpha subunit of cone transducin (GNAT2)
Vitreoretinopathy with phalangeal epiphyseal dysplasia, a type II collagenopathy resulting from a novel mutation in the C-propeptide region of the molecule
Familial chronic nail candidiasis with ICAM-1 deficiency: a new form of chronic mucocutaneous candidiasis
Expression of wild type and mutant TSC2, but not TSC1, causes an increase in the G1 fraction of the cell cycle in HEK293 cells
Mutation in KCNQ1 that has both recessive and dominant characteristics
Heterozygous truncating mutation in the human homeobox gene GSH2 has no discernable phenotypic effect
Genetic counselling for familial breast and ovarian cancer in Ontario
The use of audiotapes in consultations with women from high risk breast cancer families: a randomised trial
Parental attitude towards genetic testing for familial hypercholesterolaemia in children
Surviving campomelic dysplasia has the radiological features of the previously reported ischio-pubic-patella syndrome
Exceptionally mild Angelman syndrome phenotype associated with an incomplete imprinting defect
The intron 14 2140+5G>A variant in the low density lipoprotein receptor gene has no effect on plasma cholesterol levels
Apolipoprotein E4 is only a weak predictor of dementia and cognitive decline in the general population
Spondyloepimetaphyseal dysplasia with multiple dislocations (Hall type): three further cases and evidence of autosomal dominant inheritance
Coping style, psychological distress, risk perception, and satisfaction in subjects attending genetic counselling for hereditary cancer
Correction
Transmission of an unbalanced (Y;1) translocation in Brittany, France
Subtelomeric rearrangements detected by FISH in three of 33 families with idiopathic mental retardation and minor physical anomalies
Survival in trisomy 13 and trisomy 18 cases ascertained from population based registers
Two novel aspartoacylase gene (ASPA) missense mutations specific to Norwegian and Swedish patients with Canavan disease
Molecular analysis of the aldolase B gene in patients with hereditary fructose intolerance from Spain
Allelic variants in the 5` non-coding region of the connexin32 gene: possible pitfalls in the diagnosis of X linked Charcot-Marie-Tooth neuropathy (CMTX)
Change in the penetrance of founder BRCA1/2 mutations? A retrospective cohort study
A novel 2 bp deletion in the TM4SF2 gene is associated with MRX58
Smith-Lemli-Opitz syndrome: carrier frequency and spectrum of DHCR7 mutations in Canada
Linkage of otosclerosis to a third locus (OTSC3) on human chromosome 6p21.3-22.3
Association of an interleukin 1B gene polymorphism (-511) with Parkinson's disease in Finnish patients
What do women really want to know? Motives for attending familial breast cancer clinics
The effect of a single BRCA2 mutation on cancer in Iceland
Novel BRCA2 mutation in a Polish family with hamartoma and two male breast cancers
Identification of 13 new mutations in the ACVRL1 gene in a group of 52 unselected Italian patients affected by hereditary haemorrhagic telangiectasia
The p63 gene in EEC and other syndromes
A cryptic deletion of 2q35 including part of the PAX3 gene detected by breakpoint mapping in a child with autism and a de novo 2;8 translocation
Towards a suggestive facial dysmorphism in adenylosuccinate lyase deficiency?
A new case of Yq microdeletion transmitted from a normal father to two infertile sons
Klinefelter-like phenotype and primary infertility in a male with a paracentric Xq inversion
Screening of TCOF1 in patients from different populations: confirmation of mutational hot spots and identification of a novel missense mutation that suggests an important functional domain in the prot
Germline mutations in the von Hippel-Lindau (VHL) gene in patients from Poland: disease presentation in patients with deletions of the entire VHL gene
Alkaptonuria in the Dominican Republic: identification of the founder AKU mutation and further evidence of mutation hot spots in the HGO gene
Molecular analysis of the CBP gene in 60 patients with Rubinstein-Taybi syndrome
Mutation screening of the PKD1 transcript by RT-PCR
Emery and Rimoin's Principles and Practice of Medical Genetics: 4th edition. Editors D L Rimoin, J M Connor, R E Pyeritz, B R Korf. (Pp 4936; pound350.00.) Edinburgh: Churchill-Livingstone. 2002. ISBN
Analysis of the phenotypic abnormalities in lymphoedema-distichiasis syndrome in 74 patients with FOXC2 mutations or linkage to 16q24
From errant enzymes to colon cancers
Genetic mapping of a susceptibility locus for disc herniation and spastic paraplegia on 6q23.3-q24.1
MICA-A5.1 weighs in with HLA-DR3/DQ2 in coeliac disease
Prenatal detection of cystic fibrosis by ultrasonography: a retrospective study of more than 346 000 pregnancies
Genetics for Cardiologists: The Molecular Genetic Basis of Cardiovascular Disorders: Ali J Marian. London: ReMedica Publishing. 2001. ISBN 1 901346 09 9.
Attitudes of deaf and hard of hearing subjects towards genetic testing and prenatal diagnosis of hearing loss
Catalog of Teratogenic Agents: 10th edition. Thomas H Shepard. Baltimore: The Johns Hopkins University Press. 2001. ISBN 0-8018-6722-3.
Genetic and functional analysis of the von Hippel-Lindau (VHL) tumour suppressor gene promoter
Linkage stratification and mutation analysis at the parkin locus identifies mutation positive Parkinson's disease families
Concerns of women presenting to a comprehensive cancer centre for genetic cancer risk assessment
Breakpoint analysis of a familial balanced translocation t(2;8)(q31;p21) associated with mesomelic dysplasia
Detection of large rearrangements of exons 13 and 22 in the BRCA1 gene in German families
Comorbid VHL and SCA2 mutations in a large kindred: confounding diagnosis of neurological dysfunction caused by CNS VHL vascular tumours versus SCA2 atrophic neurodegeneration