Huntington's disease - Yet another mad protein?;Huntingtons sjukdom - Ännu ett galet protein?

Lakartidningen

Volumn 98, Issue 50, 2001, Pages 5756-5761

  Petersén, Åsa ^c   Hansson, Oskar ^c   Brundin, Patrik ^a,b  

^a LUND UNIVERSITY   (Sweden)

^b LUND UNIVERSITY   (Sweden)

^c NONE

Author keywords

[No Author keywords available]

Indexed keywords

NERVE PROTEIN;

ANIMAL; BIOLOGICAL MODEL; DISEASE MODEL; GENETICS; HUMAN; HUNTINGTON CHOREA; MOUSE; MUTATION; REVIEW; TRINUCLEOTIDE REPEAT;

ANIMALS; DISEASE MODELS, ANIMAL; HUMANS; HUNTINGTON DISEASE; MICE; MODELS, GENETIC; MUTATION; NERVE TISSUE PROTEINS; TRINUCLEOTIDE REPEATS;

EID: 0035852392     PISSN: 00237205     EISSN: None     Source Type: Journal    
DOI: None     Document Type: Article

References (20)

1. Vonsattel JP, DiFiglia M. Huntington disease. J Neuropathol Exp Neurol 1998;57:369-84.
2. Huntington's Disease Collaborative Research Group. A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. Cell 1993;72:971-83.
3. Sherman MY, Goldberg AL. Cellular defenses against unfolded proteins: a cell biologist thinks about neurodegenerative diseases. Neuron 2001;29:15-32.
4. Roizin L, Stellar S, Liu LC. Neuronal nuclear-cytoplasmic changes in Huntington's chorea: electron microscope investigations. Adv Neurol 1979;23:93-122.
5. Petersén Å, Mani K, Brundin P. Recent advances on the pathogenesis of Huntington's disease. Exp Neurol 1999;157:1-18.
6. Petersén Å, Larsen KE, Behr GG, Romero N, Przedborski S, Brundin P, et al. Expanded CAG repeats in exon 1 of the Huntington's disease gene stimulate dopamine-mediated striatal neuron autophagy and degeneration. Hum Mol Genet 2001;10:1243-54.
7. Dyer RB, McMurray CT. Mutant protein in Huntington disease is resistant to proteolysis in affected brain. Nat Genet. In press.
8. Cattaneo E, Rigamonti D, Goffredo D, Zuccato C, Squitieri F, Sipione S. Loss of normal huntingtin function: new developments in Huntington's disease research. Trends Neurosci 2001;24:182-8.
9. Zeitlin S, Liu JP, Chapman DL, Papaioannou VE, Efstratiadis A. Increased apoptosis and early embryonic lethality in mice nullizygous for the Huntington's disease gene homologue. Nat Genet 1995;11:155-63.
10. Bence NF, Sampat RM, Kopito RR. Impairment of the ubiquitin-proteosome system by protein aggregation. Science 2001:292:1552-5.
11. Cha JH. Transcriptional dysregulation in Huntington's disease. Trends Neurosci 2000;9:387-92.
12. Steffan JS, Kazantsev A, Spasic-Boskovic O, Greenwald M, Zhu YZ, Gohler H, et al. The Huntington's disease protein interacts with P53 and CREB-binding protein and represses transcription. Proc Natl Acad Sci USA 2000;97:6763-8.
13. Nucifora FC Jr, Sasaki M, Peters MF, Huang H, Cooper JK, Yamada M. Interference by huntingtin and atrophin-1 with CBP mediated transcription leading to cellular toxicity. Science 2001;291:2423-8.
14. Hansson O, Petersén Å, Leist M, Nicotera P, Castilho RF, Brundin P. Transgenic mice expressing a Huntington's disease mutation are resistant to quinolinic acid-induced striatal excitotoxicity. Proc Natl Acad Sci USA 1999;96:8727-32.
15. The Huntington Study Group. A randomized, placebo-controlled trial of coenzyme Q10 and remacemide in Huntington's disease. Neurology 2001;57:397-404.
16. Bachoud-Levi AC, Remy P, Nguyen JP, Brugieres P, Lefaucheur JP, Bourdet C, et al. Motor and cognitive improvements in patients with Huntington's disease after neural transplantation. Lancet 2000;356:1975-9.
17. Chen M, Ona VO, Li M, Ferrante RJ, Fink KB, Zhu S, et al. Minocycline inhibits caspase-1 and caspase-3 expression and delays mortality in a transgenic mouse model of Huntington disease. Nat Med 2000;6:797-801.
18. Ferrante RJ, Andreassen OA, Jenkins BG, Dedeoglu A, Kuemmerle S, Kubilus JK, et al. Neuroprotective effects of creatine in a transgenic mouse model of Huntington's disease. J Neurosci 2000;20: 4389-97.
19. Zuccato C, Ciammola A, Rigamonti D, Leavitt BR, Goffredo D, Conti L. Loss of huntingtin-mediated BDNF gene transcription in Huntington's disease. Science 2001;293:493-8.
20. Steffan JS, Bodai L, Pallos J, Poelman M, McCampbell A, Apostol BL, et al. Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila. Nature 2001;413:739-43.