Gender of the embryo contributes to CAG instability in transgenic mice containing a Huntington's disease gene

Human Molecular Genetics

Volumn 9, Issue 18, 2000, Pages 2767-2775

  Kovtun, Irina V ^a   Therneau, Terrence M ^a   McMurray, Cynthia T ^a  

^a MAYO CLINIC   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

ADENINE; CYTOSINE; DNA; GUANINE; TRINUCLEOTIDE;

ANIMAL TISSUE; ARTICLE; CONTROLLED STUDY; DNA REPAIR; DNA REPLICATION; EMBRYO; EXON; FATHER; FEMALE; GENDER; GENE MUTATION; GENETIC CODE; GENETIC STABILITY; GERM LINE; HUNTINGTON CHOREA; MALE; MOUSE; MUTANT; NONHUMAN; PRIORITY JOURNAL; PROGENY; TRANSGENE; TRANSGENIC MOUSE; TRINUCLEOTIDE REPEAT;

EID: 0034326903     PISSN: 09646906     EISSN: None     Source Type: Journal    
DOI: 10.1093/hmg/9.18.2767     Document Type: Article

References (40)

1. Anticipation in Huntington's disease is inherited through the male line but may originate in the female
2. The relationship between trinucleotide repeat (CAG) length and clinical features of Huntington disease
3. A study of the Huntington's disease associated trinucleotide repeat in the Scottish population
4. Molecular analysis of new mutations for Huntington's disease: Intermediate alleles and sex of origin effects
5. Contribution of DNA sequence and CAG size to mutation frequencies of intermediate alleles for Huntington disease: Evidence from single sperm analysis
6. Trinucleotide repeat length instability and age of onset in Huntington's disease
7. Instability of CAG repeats in Huntington's disease: Relation to parental transmission and age of onset
8. Mitotic stability and meiotic variability of the (CAG)(n) repeat in the Huntington disease gene
9. Correlation between magnitude of CAG repeat length alterations and length of the paternal repeat in parentally inherited Huntington's disease
10. Evidence for a mechanism predisposing to intergenerational CAG repeat instability in spinocerebellar ataxia type I
11. Expansion of an unstable trinucleotide CAG repeat in spinocerebellar ataxia type 1
12. Unstable expansion of CAG repeat in hereditary dentatorubral-pallidoluysian atrophy (DRPLA)
13. Dentatorubral and pallidoluysian atrophy expansion of an unstable CAG trinucleotide on chromosome 12p
14. Intergenerational instability of the CAG repeat of the gene for Machado-Joseph disease (MJD1) is affected by the genotype of the normal chromosome: Implication for the molecular mechanisms of the instability of the CAG repeat
15. Non-Mendelian transmission in dentatrorubral-pallidoluysian atrophy and Machado-Joseph disease: The mutant allele is preferentially transmitted in male meiosis
16. Meiotic stability and genotype-phenotype correlation of the trinucleotide repeat in X-linked spinal and bulbar atrophy
17. The marker (X) syndrome: A cytogenetic analysis
18. Further segregation analysis of the fragile X syndrome with special reference to premutation males
19. Characterization of the full fragile X syndrome mutation in fetal gametes
20. Segregation distortion of the CTG repeats at the myotonic dystrophy locus
21. Segregation distortion in myotonic dystrophy
22. An untranslated CTG expansion causes a novel form of spinocerebellar ataxia (SCA 8)
23. Fragile X syndrome and the (CGG)(n) mutation: Two families with discordant MZ twins
24. Somatic and gonadal mosaicism of the Huntington disease gene CAG repeat in brain and sperm
25. Instability of highly expanded CAG in mice transgenic for the Huntington's disease mutation
26. Transgenic mice harboring a full-length human mutant DRPLA gene exhibit age-dependent intergenerational and somatic instabilities of CAG repeats comparable with those in DRPLA patients
27. Gametic but not somatic instability of CAG repeat length in Huntington's disease
28. CAG repeat length variation in sperm from a patient with Kennedy's disease
29. Analysis of (CAG)(n) size heterogeneity in somatic and sperm cell DNA from intermediate and expanded Huntington Disease gene carriers
30. Analysis of germline mutation spectra at the Huntington's disease locus supports a mitotic mutation mechanism
31. Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice
32. Anticipation and instability of IT-15 (CAG)(n) repeats in parent-offspring pairs with Huntington disease
33. The appropriateness of analysis of variance and multiple-comparison procedures
34. Testing the statistical model
35. Increased trinucleotide repeat instability with advanced maternal age
36. DNA secondary structure: A common and causative factor for expansion in human disease
37. Different mechanisms underlie DNA instability in Huntington disease and colorectal cancer