Paradoxical aggregation versus oligomerisation properties of mutant and wild-type huntingtin fragments

Experimental Neurology

Volumn 199, Issue 2, 2006, Pages 243-244

  Rubinsztein, David C ^a   Huntington, James A ^a  

^a ADDENBROOKE S HOSPITAL   (United Kingdom)

Author keywords

[No Author keywords available]

Indexed keywords

HUNTINGTIN; MUTANT PROTEIN;

ARTICLE; CLINICAL FEATURE; EXON; GENE MUTATION; GENETIC ANALYSIS; HUMAN; HUNTINGTON CHOREA; MUTANT; NEUROPROTECTION; OLIGOMERIZATION; PRIORITY JOURNAL; PROTEIN DEGRADATION; WILD TYPE;

EID: 33745263202     PISSN: 00144886     EISSN: 10902430     Source Type: Journal    
DOI: 10.1016/j.expneurol.2006.03.006     Document Type: Note

References (12)

1. Arrasate M., Mitra S., Schweitzer E.S., Segal M.R., and Finkbeiner S. Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death. Nature 431 (2004) 805-810
2. Cong S.Y., Pepers B.A., Roos R.A.C., van Ommen G.-J.B., and Dorsman J.C. Small N-terminal mutant huntingtin fragments, but not- wild-type, are mainly present in monomeric form: implications for pathogenesis. Exp. Neurol. (2006) 10.1016/j.expneurol.2005.11.008 (this issue)
3. Goehler H., Lalowski M., Stelzl U., Waelter S., Stroedicke M., Worm U., Droege A., Lindenberg K.S., Knoblich M., Haenig C., Herbst M., Suopanki J., Scherzinger E., Abraham C., Bauer B., Hasenbank R., Fritzsche A., Ludewig A.H., Buessow K., Coleman S.H., Gutekunst C.-A., Landwehrmeyer B.G., Lehrach H., and Wanker E.E. A protein interaction network links GIT1, an enhancer of huntingtin aggregation, to Huntington's disease. Mol. Cell 15 (2004) 853-865
4. Gunawardena S., and Goldstein L.S. Polyglutamine diseases and transport problems: deadly traffic jams on neuronal highways. Arch. Neurol. 62 (2005) 46-51
5. Humbert S., Bryson E.A., Cordelières F.P., Connors N.C., Datta S.R., Finkbeiner S., Greenberg M.E., and Saudou F. The IGF-1/Akt pathway is neuroprotective in Huntington's disease and involves Huntingtin phosphorylation by Akt. Dev. Cell 2 (2002) 831-837
6. Ko J., Ou S., and Patterson P.H. New anti-huntingtin monoclonal antibodies: implications for huntingtin conformation and its binding proteins. Brain Res. Bull. 56 (2001) 319-329
7. Luo S., Vacher C., Davies J.E., and Rubinsztein D.C. Cdk5 phosphorylation of huntingtin reduces its cleavage by caspases: implications for mutant huntingtin toxicity. J. Cell Biol. 169 (2005) 647-656
8. Ravikumar B., Vacher C., Berger Z., Davies J.E., Luo S., Oroz L.G., Scaravilli F., Easton D.F., Duden R., O'Kane C.J., and Rubinsztein D.C. mTOR inhibition induces autophagy and reduces toxicity of the Huntington's disease mutation in Drosophila and mouse models. Nat. Genet. 36 (2004) 585-595
9. Rubinsztein D.C. The molecular pathology of Huntington's disease. Curr. Med. Chem. Immunol. Endocr. Metab. Agents 3 (2003) 329-340
10. Soupanki J., Gotz C., Lutsch G., Schiller J., Harjes P., Herrmann A., and Wanker E.E. Interaction of huntingtin fragments with brain membranes-Clues to early dysfunction in Huntington's disease. J. Neurochem. 96 (2006) 870-884
11. Steffan J., Agrawal S., Pallos N., Rockabrand J., Trotman E., Slepko L.C., Illes M., Lukacsovich K., Zhu T., Cattaneo Y.-Z., Pandolfi E., Thompson P.P., and Marsh L.M. SUMO modification of Huntingtin and Huntington's disease pathology. Science 304 (2004) 100-104
12. Wyttenbach A., Swartz J., Kita H., Thykjaer T., Carmichael J., Bradley J., Brown R., Maxwell M., Schapira A., Orntoft T.F., Kato K., and Rubinsztein D.C. Polyglutamine expansions cause decreased CRE-mediated transcription and early gene expression changes prior to cell death in an inducible cell model of Huntington's disease. Hum. Mol. Genet. 10 (2001) 1829-1845