Cleavage of huntingtin by apopain, a proapoptotic cysteine protease, is modulated by the polyglutamine tract

Nature Genetics

Volumn 13, Issue 4, 1996, Pages 442-449

  Goldberg, Y P ^a   Nicholson, D W ^b   Rasper, D M ^b   Kalchman, M A ^a   Koide, H B ^a   Graham, R K ^a   Bromm, M ^a   Kazemi Esfarjani, P ^a   Thornberry, N A ^c   Vaillancourt, J P ^b   Hayden, M R ^a,d  

^a UNIVERSITY OF BRITISH COLUMBIA   (Canada)

^b MERCK FROSST CENTRE FOR THERAPEUTIC RESEARCH   (Canada)

^c MERCK RESEARCH LABORATORIES   (United States)

^d BC CHILDREN S HOSPITAL   (Canada)

Author keywords

[No Author keywords available]

Indexed keywords

CYSTEINE PROTEINASE; GENE PRODUCT; POLYGLUTAMIC ACID;

AMINO TERMINAL SEQUENCE; ANIMAL CELL; APOPTOSIS; ARTICLE; CELL DEATH; HUMAN; HUNTINGTON CHOREA; MAMMAL CELL; PRIORITY JOURNAL; PROTEIN DEGRADATION; SEQUENCE ANALYSIS;

ANIMALS; APOPTOSIS; CASPASE 3; CASPASES; CELL LINE; CERCOPITHECUS AETHIOPS; CYSTEINE ENDOPEPTIDASES; HUMANS; HUNTINGTON DISEASE; KINETICS; NERVE TISSUE PROTEINS; NUCLEAR PROTEINS; PEPTIDES; RECOMBINANT PROTEINS; STRUCTURE-ACTIVITY RELATIONSHIP; SUBSTRATE SPECIFICITY; TRANSFECTION; TRINUCLEOTIDE REPEATS;

ANIMALIA; MAMMALIA;

EID: 9344227302     PISSN: 10614036     EISSN: None     Source Type: Journal    
DOI: 10.1038/ng0896-442     Document Type: Article

References (37)

1. Martin, S.J. & Green, D.R. Protease Activation during Apoptosis: Death by a Thousand Cuts? Cell 82, 349-352 (1995).
2. Ellis, H.M. & Horvitz, H.R. Genetic control of programmed cell death in the nematode Caenorhabditis elegans. Cell 44, 817-829 (1986).
3. Yuan, J.-Y. et al. The C. elegans cell death gene ced-3 encodes a protein similar to mammalian interleukin-1β-converting enzyme. Cell 75, 641-652 (1993).
4. Nicholson, D.W. et al. Identification and inhibition of the ICE/CED-3 protease necessary for mammalian apoptosis. Nature 376, 37-43 (1995).
5. Tewari, M. et al. Yama/CPP32β, a Mammalian Homolog of CED-3, Is a CmA-Inhibitable Protease That Cleaves the Death Substrate Poly(ADP-Ribose) Polymerase. Cell 81, 801-809 (1995).
6. Fernandes-Alnemri, T., Litwack, G. & Alnemri, E.S. CPP32, a novel human apoptotic protein with homology to Caenorhabditis elegans cell death protein Ced-3 and mammalian interleukin-1β converting enzyme. J. Biol. Chem. 270, 15870-15976 (1994).
7. Casciola-Rosen, L.A., Grant J.A. & Rosen A. DNA-dependent protein kinase is one of a subset of autoantigens specifically cleaved early during apoptosis. J. Exp. Med. 182, 1625-1634 (1995).
8. Wang, X. et al. Cleavage of sterol regulatory element binding proteins (SREBPs) by CPP32 during apoptosis. EMBO J. 15, 1012-1020 (1996).
9. Hayden, M.R. Huntington's Chorea (London: Springer-Verlag, 1981).
10. Harper, P.S. Huntington's Disease (London: W.B. Saunders, 1991).
11. Huntington's Disease Collaborative Research Group. A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. Cell 72, 971-983 (1993).
12. Kremer, H.P.H. et al. Worldwide Study of the Huntington's disease mutation: the sensitivity and specificity of repeated CAG sequences. N. Engl. J. Med. 330, 1401-1406 (1994).
13. Sharp, A. et al. Widespread expression of Huntington's disease gene (IT-15) protein product. Neuron 14, 1065-1074 (1995).
14. DiFiglia, M. et al. Huntingtin is a cytoplasmic protein associated with vesicles in human and rat brain neurons. Neuron 14, 1075-1081 (1995).
15. Trottier, Y. et al. Polyglutamine expansion as a common pathological epitope detected in Huntingdon's disease, in spinocerebellar ataxia 1 and 3 and two additional autosomal dominant cerebellar ataxias. Nature 378, 403-406 (1995).
16. Gutekunst, C.-A. et al Identification and localization of huntingtin in brain and human lymphoblastoid cell lines with anti-fusion protein antibodies. Proc. NaH. Acad. Sci. USA 92, 8710-8714 (1995).
17. Burke, J.R. et al. Huntingtin and DRPLA proteins selectively interact with the enzyme GAPDH. Nature Med. 2, 347-350 (1996).
18. Jou, Y.S & Myers, R.M. Evidence from antibody studies that the CAG repeat in the Huntington disease gene is expressed in the protein. Hum. Mol. Genet. 4, 465-469 (1995).
19. Thomas, L.B. et al. DNA End Labeling (TUNEL) in Huntington's Disease and Other Neuropathological Conditions. Exp. Neurol. 133, 265-272 (1995).
20. Dragunow, M. et al. In situ evidence for DNA fragmentation in Huntington's disease striatum and Alzheimer's disease temporal lobes. Clin. Neuroci. Neuropath. 6, 1053-1057 (1995).
21. Protera-Cailliau, C., Hedreen, J.C., Price, D.L. & Koliatsos, V.E. Evidence for Apoptotic Cell Death in Huntington Disease and Excitotoxic Animal Models. J. Neurosci. 15, 3775-3787 (1995).
22. Nasir, J. et al. Targeted disruption of the Huntington's disease gene results in embryonic lethality and behavioral and morphological changes in heterozygotes. Cell 81, 811-823 (1995).
23. Duyao, M.P. et al. Inactivation of the mouse Huntington's disease gene homolog Hdh. Science 269, 407-410 (1995).
24. Zeitlin, S., Liu, J.-P., Chapman, D.L., Papaioannou, V.E. & Efstratiadis, A. Increased apoptosis and early embryonic lethality in mice nullizygous for the Huntington's disease gene homologue. Nature Genet. 11, 155-162 (1995).
25. Goldberg, Y.P. et al. Absence of disease phenotype and intergenerational stability of the CAG repeat in transgenic mice expressing the Huntington disease transcript. Hum. Mol. Genet. 5, 177-185 (1996).
26. Lazebnik, Y.A., Kaufmann, S.H., Desnoyers, S., Poirier, G.G. & Earnshaw, W.C. Cleavage of poly(ADP-ribose) polymerase by a proteinase with properties like ICE. Nature 371, 346-347 (1994).
27. Burright, E.N. et al. SCA1 Transgenic Mice: A Model for Neurodegeneration Caused by an Expanded CAG Trinucleotide Repeat. Cell. 82, 937-948 (1995).
28. Thornberry, N.A. et al. A novel heterodimeric cysteine protease is required for interleukin-1β processing in monocytes. Nature 356, 768-774 (1992).
29. Casciola-Rosen, L.A., Miller, D.K., Anhalt, G.J. & Rosen, A. Specific cleavage of the 70-kD protein component of the U1 small nuclear ribonucleoprotein is a characteristic biochemical feature of apoptotic cell death. J. Biol. Chem. 269, 30757-30760 (1994).
30. Nicholson, D.W. ICE/CED3-like proteases as therapeutic targets for the control of inappropriate apoptosis. Nature Biotech. 14, 297-301 (1996).
31. Fernandes-Alnemri, T. et al. Mch3, a novel human apoptotic cysteine protease highly related to CPP32 Cancer Res. 55, 6045-6052 (1995).
32. Telenius, H. et al. Molecular analysis of juvenile Huntington disease: the major influence on (CAG)n repeat length is the sex of the affected parent. Hum. Mol. Genet. 2, 1535-1540 (1993).
33. Bump, N.J. et al. Inhibition of ICE Family Proteases by Baculovirus Antiapoptotic Protein p35. Science 269, 1885-1888 (1995).
34. Xue, D. & Horvitz, H.R. Inhibition of the Caenorhabditis elegans cell-death protease CED-3 by a CED-3 cleavage site in baculovirus p35 protein. Nature 377, 248-251 (1995).
35. Thornberry, N.A. Interleukin-1 beta converting enzyme. Meth. Enzymol. 244, 615-631 (1994).
36. Towbin, H., Staehelin, T. & Gordon, J. Electrophoretic transfer of proteins form polyacrylamide gels to nitrocellulose sheets: Procedure and some applications. Proc. Natl. Acad. Sci. USA. 76, 4350-4354 (1979).
37. Kaufmann, S.H., Desnoyers, S., Ottaviano, Y., Davidson, N.E., Poirier, G.G. Specific Proteolytic Cleavage of Poly (ADP-ribose) Polymerase: An Early Marker of Chemotherapy-induced Apoptosis. Cancer Res. 53, 3976-3985 (1993).