Transgenic models of Huntington's disease

Human Molecular Genetics

Volumn 6, Issue 10 REV. ISS., 1997, Pages 1633-1637

  Bates, Gillian P ^a   Mangiarini, Laura ^a   Mahal, Amarbirpal ^a   Davies, Stephen W ^b  

^a GUY S HOSPITAL   (United Kingdom)

^b UNIVERSITY COLLEGE LONDON   (United Kingdom)

Author keywords

[No Author keywords available]

Indexed keywords

CYTOSKELETON PROTEIN; HUNTINGTIN; MUTANT PROTEIN; POLYGLUTAMIC ACID; PROTEIN; REPETITIVE DNA;

AMINO ACID SEQUENCE; ANIMAL EXPERIMENT; ANIMAL MODEL; ARTICLE; AUTOSOME MOSAICISM; CELL DEATH; CELL SPECIFICITY; DEGENERATIVE DISEASE; GENE DISRUPTION; GENE FUNCTION; GENE STRUCTURE; GENETIC STABILITY; HUMAN; HUNTINGTON CHOREA; MOUSE; NERVE DEGENERATION; NONHUMAN; PRIORITY JOURNAL; PROTEIN PROTEIN INTERACTION; TISSUE SPECIFICITY; TRANSGENIC MOUSE;

ANIMALS; HUMANS; HUNTINGTON DISEASE; MICE; MICE, KNOCKOUT; MICE, TRANSGENIC; MUTATION; NERVE TISSUE PROTEINS; NUCLEAR PROTEINS; TRINUCLEOTIDE REPEATS;

ANIMALIA; MUS MUSCULUS;

EID: 0030866538     PISSN: 09646906     EISSN: None     Source Type: Journal    
DOI: 10.1093/hmg/6.10.1633     Document Type: Article

References (13)

1. HDCRG (1993) A novel gene containing a trinucleotide repeat that is unstable on Huntington's disease chromosomes. Cell 72, 971-983.
2. La Spada, A.R. et al. (1991) Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy. Nature 352,77-79.
3. Koide, R. et al. (1994) Unstable expansion of CAG repeat in hereditary dentatorubral-pallidoluysian atrophy (DRPLA). Nature Genet. 6, 9-13.
4. Nagafuchi, S. et al. (1994) Dentatorubral and pallidoluysian atrophy expansion of an unstable CAG trinucleotide on chromosome 12p. Nature Genet. 6, 14-18.
5. Orr, H.T. et al. (1993) Expansion of an unstable trinucleotide CAG repeat in spinocerebellar ataxia type 1. Nature Genet. 4, 221-226.
6. Imbert, G. et al. (1996) Cloning of the gene for spinocerebellar ataxia 2 reveals a locus with high sensitivity to expanded CAG/glutamine repeats. Nature Genet. 14, 285-291.
7. Sampei, K. et al. (1996) Identification of the spinocerebellar ataxia type 2 gene using a direct identification of repeat expansion and cloning technique, DIRECT. Nature Genet. 14, 277-284.
8. Pulst, S.-M. et al. (1996) Moderate expansion of a normally biallelic trinucleotide repeat in spinocerebellar ataxia type 2. Nature Genet. 14, 269-276.
9. Kawaguchi, Y. et al. (1994) CAG expansions in a novel gene for Machado-Joseph disease at chromosome 14q32.1. Nature Genet. 8, 221-228.
10. Zhuchenko, O. et al. (1997) Autosomal dominant cerebellar ataxia (SCA6) associated with small polyglutamine expansions in the alpha 1A-voItage dependent calcium channel. Nature Genet. 15, 62-69.
11. Ross, C.A. (1995) When more is less: pathogenesis of glutamine repeat neurodegenerative diseases. Neuron 15, 493-496.
12. Perutz, M.F. (1996) Glutamine repeats and inherited neurodegenerative diseases: molecular aspects. Curr. Opin. Struct. Biol 6, 848-858.
13. Trottier, Y. et al. (1995) Polyglutamine expansion as a pathological epitope in Huntington's disease and four dominant cerebellar ataxias. Nature 378, 403-406.