Cystamine increases L-cysteine levels in Huntington's disease transgenic mouse brain and in a PC12 model of polyglutamine aggregation

Journal of Neurochemistry

Volumn 91, Issue 2, 2004, Pages 413-422

  Fox, Jonathan H ^a   Barber, David S ^b   Singh, Bhupinder ^a   Zucker, Birgit ^a   Swindell, Mary K ^a   Norflus, Fran ^a   Buzescu, Rodica ^a   Chopra, Raman ^a   Ferrante, Robert J ^c,d   Kazantsev, Aleksey ^a   Hersch, Steven M ^a,e  

^a MASSACHUSETTS GENERAL HOSPITAL   (United States)

^b UNIVERSITY OF FLORIDA   (United States)

^c BOSTON UNIVERSITY SCHOOL OF MEDICINE   (United States)

^d VETERANS AFFAIRS MEDICAL CENTER   (United States)

^e MassGeneral Inst for Neurodeg Dis   (United States)

Author keywords

Cystamine; Glutathione; Huntington's disease; L cysteine; Neuroprotection; Oxidative stress

Indexed keywords

3 NITROPROPIONIC ACID; CYSTAMINE; CYSTEINE; GLUTATHIONE; HUNTINGTIN; POLYGLUTAMINE; PROTEIN GLUTAMINE GAMMA GLUTAMYLTRANSFERASE;

ANIMAL EXPERIMENT; ANIMAL MODEL; ANTIOXIDANT ACTIVITY; ARTICLE; BRAIN INJURY; BRAIN TISSUE; CONTROLLED STUDY; CROSS LINKING; ENZYME INHIBITION; HUNTINGTON CHOREA; MOUSE; NERVE DEGENERATION; NEUROPROTECTION; NONHUMAN; OXIDATIVE STRESS; PLEIOTROPY; POLYMERASE CHAIN REACTION; PRIORITY JOURNAL; PROTEIN AGGREGATION; STRIATE CORTEX; TRANSGENIC MOUSE;

ANIMALS; ANTIOXIDANTS; BRAIN; BUTHIONINE SULFOXIMINE; CYSTAMINE; CYSTEINE; CYTOPROTECTION; DISEASE MODELS, ANIMAL; DISEASE PROGRESSION; ENZYME INHIBITORS; FEMALE; GENE EXPRESSION REGULATION; GLUTATHIONE; HUMANS; HUNTINGTON DISEASE; MALE; MICE; MICE, TRANSGENIC; NEURONS; NEUROPROTECTIVE AGENTS; OXIDATIVE STRESS; PC12 CELLS; PEPTIDES; RATS;

ANIMALIA; MUS MUSCULUS;

EID: 5444229092     PISSN: 00223042     EISSN: None     Source Type: Journal    
DOI: 10.1111/j.1471-4159.2004.02726.x     Document Type: Article

References (49)

1. Apostol B. L., Kazantsev A., Raffioni S. et al. (2003) A cell-based assay for aggregation inhibitors as therapeutics of polyglutamine-repeat disease and validation in Drosophila. Proc. Natl Acad. Sci. USA 100, 5950-5955.
2. Baker M. A., Cerniglia G. J. and Zaman A. (1990) Microtiter plate assay for the measurement of glutathione and glutathione disulfide in large numbers of biological samples. Anal. Biochem. 190, 360-365.
3. Balijepalli S., Boyd M. R. and Ravindranath V. (2000) Human brain thioltransferase: constitutive expression and localization by fluorescence in situ hybridization. Brain Res. Mol. Brain Res. 85, 123-132.
4. Beal M. F. (2000) Energetics in the pathogenesis of neurodegenerative diseases. Trends Neurosci. 23, 298-304.
5. Beal M. F. (2002) Coenzyme Q10 as a possible treatment for neurodegenerative diseases. Free Radic. Res. 36, 455-460.
6. Beal M. F. and Ferrante R. J. (2004) Experimental therapeutics in transgenic mouse models of Huntington's disease. Nat. Rev. Neurosci. 5, 373-384.
7. Bogdanov M. B., Andreassen O. A., Dedeoglu A., Ferrante R. J. and Beal M. F. (2001) Increased oxidative damage to DNA in a transgenic mouse model of Huntington's disease. J. Neurochem. 79, 1246-1249.
8. Browne S. E., Bowling A. C., MacGarvey U., Baik M. J., Berger S. C., Muqit M. M., Bird E. D. and Beal M. F. (1997) Oxidative damage and metabolic dysfunction in Huntington's disease: selective vulnerability of the basal ganglia. Ann. Neurol. 41, 646-653.
9. Browne S. E., Ferrante R. J. and Beal M. F. (1999) Oxidative stress in Huntington's disease. Brain Pathol. 9, 147-163.
10. Cha J. H. (2000) Transcriptional dysregulation in Huntington's disease. Trends Neurosci. 23, 387-392.
11. Dedeoglu A., Kubilus J. K., Jeitner T. M., et al. (2002) Therapeutic effects of cystamine in a murine model of Huntington's disease. J. Neurosci. 22, 8942-8950.
12. Dunah A. W., Jeong H., Griffin A., Kim Y. M., Standaert D. G., Hersch S. M., Mouradian M. M., Young A. B., Tanese N. and Krainc D. (2002) Sp1 and TAFII130 transcriptional activity disrupted in early Huntington's disease. Science 296, 2238-2243.
13. Fariss M. W. and Reed D. J. (1987) High-performance liquid chromatography of thiols and disulfides: dinitrophenol derivatives. Methods Enzymol. 143, 101-109.
14. Fink S. L., Ho D. Y. and Sapolsky R. M. (1996) Energy and glutamate dependency of 3-nitropropionic acid neurotoxicity in culture. Exp. Neurol. 138, 298-304.
15. Fontaine M. A., Geddes J. W., Banks A. and Butterfield D. A. (2000) Effect of exogenous and endogenous antioxidants on 3-nitropionic acid-induced in vivo oxidative stress and striatal lesions: insights into Huntington's disease. J. Neurochem. 75, 1709-1715.
16. Gines S., Seong I. S., Fossale E., Ivanova E., Trettel F., Gusella J. F., Wheeler V. C., Persichetti F. and MacDonald M. E. (2003) Specific progressive cAMP reduction implicates energy deficit in presymptomatic Huntington's disease knock-in mice. Hum. Mol. Genet. 12, 497-508.
17. Hersch S. and Ferrante R. (2003) Neuropathology and pathophysiology of Huntington's disease, in Movement Disorders (Watts, R. and Koller, W., eds), pp. 503-518. McGraw-Hill, New York.
18. Mickey M. A. and Morton A. J. (2000) Mice transgenic for the Huntington's disease mutation are resistant to chronic 3-nitropropionic acid-induced striatal toxicity. J. Neurochem. 75, 2163-2171.
19. Huang C. S., Moore W. R. and Meister A. (1988) On the active site thiol of gamma-glutamylcysteine synthetase: relationships to catalysis, inhibition, and regulation. Proc. Natl Acad. Sci. USA 85, 2464-2468.
20. Jeitner T. M., Bogdanov M. B., Matson W. R. et al. (2001) N(epsilon)-(gamma-L-Glutamyl)-L-lysine (GGEL) is increased in cerebrospinal fluid of patients with Huntington's disease. J. Neurochem. 79, 1109-1112.
21. Jokay I., Kelemenics K., Gyuris A. and Minarovits J. (1998) 5-Methylthio-cysteine and cystamine are potent stimulators of thiol production and glutathione synthesis. Life Sci. 62, PL27-33.
22. Kahlem P., Green H. and Djian P. (1998) Transglutaminase action imitates Huntington's disease: selective polymerization of Huntingtin containing expanded polyglutamine. Mol. Cell 1, 595-601.
23. Karpuj M. V., Becher M. W. and Steinman L. (2002a) Evidence for a role for transglutaminase in Huntington's disease and the potential therapeutic implications. Neurochem. Int. 40, 31-36.
24. Karpuj M. V., Becher M. W., Springer J. E., Chabas D., Youssef S., Pedotti R., Mitchell D. and Steinman L. (2002b) Prolonged survival and decreased abnormal movements in transgenic model of Huntington disease, with administration of the transglutaminase inhibitor cystamine. Nat. Med. 8, 143-149.
25. Kenchappa R. S. and Ravindranath V. (2003) Glutaredoxin is essential for maintenance of brain mitochondrial complex I: studies with MPTP. FASEB J. 17, 717-719.
26. Lesort M., Tucholski J., Zhang J. and Johnson G. V. (2000) Impaired mitochondrial function results in increased tissue transglutaminase activity in situ. J. Neurochem. 75, 1951-1961.
27. Lesort M., Chun W., Tucholski J. and Johnson G. V. (2002) Does tissue transglutaminase play a role in Huntington's disease? Neurochem. Int. 40, 37-52.
28. Lesort M., Lee M., Tucholski J. and Johnson G. V. (2003) Cystamine inhibits caspase activity. Implications for the treatment of polyglutamine disorders. J. Biol. Chem. 278, 3825-3830.
29. Livak K. J. and Schmittgen T. D. (2001) Analysis of relative gene expression data using real-time quantitative PCR and the 2(-Delta Delta C(T) Method. Methods 25, 402-408.
30. Lorand L. and Conrad S. M. (1984) Transglutaminases. Mol. Cell Biochem. 58, 9-35.
31. Luthi-Carter R., Strand A., Peters N. L. et al. (2000) Decreased expression of striatal signaling genes in a mouse model of Huntington's disease. Hum. Mol. Genet. 9, 1259-1271.
32. Mangiarini L., Sathasivam K., Mahal A., Mott R., Seller M. and Bates G. P. (1997) Instability of highly expanded CAG repeats in mice transgenic for the Huntington's disease mutation. Nat. Genet. 15, 197-200.
33. Mastroberardino P. G., Iannicola C., Nardacci R. et al. (2002) 'Tissue' transglutaminase ablation reduces neuronal death and prolongs survival in a mouse model of Huntington's disease. Cell Death Differ. 9, 873-880.
34. McBean G. J. and Flynn J. (2001) Molecular mechanisms of cystine transport. Biochem. Soc. Trans. 29, 717-722.
35. Montine T. J., Beal M. F., Robertson D., Cudkowicz M. E., Biaggioni I., O'Donnell H., Zackert W. E., Roberts L. J. and Morrow J. D. (1999) Cerebrospinal fluid F2-isoprostanes are elevated in Huntington's disease. Neurology 52, 1104-1105.
36. Morton A. J. and Leavens W. (2000) Mice transgenic for the human Huntington's disease mutation have reduced sensitivity to kainic acid toxicity. Brain Res. Bull. 52, 51-59.
37. Nucifora F. C. Jr, Sasaki M., Peters M. F. et al. (2001) Interference by huntingtin and atrophin-1 with cbp-mediated transcription leading to cellular toxicity. Science 291, 2423-2428.
38. Nurcombe V., Wreford N. and Bertram J. (2001) Stereological estimation of neuronal number. The optical disector/cavalieri combination, in Methods in Molecular Biology. Neutrophin Protocols, Vol. 169 (Rush, R., ed.), pp. 251-268. Humana Press, Totowa, New Jersey.
39. Panov A. V., Gutekunst C. A., Leavitt B. R., Hayden M. R., Burke J. R., Strittmatter W. J. and Greenamyre J. T. (2002) Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines. Nat. Neurosci. 5, 731-736.
40. Perez-Severiano F., Rios C. and Segovia J. (2000) Striatal oxidative damage parallels the expression of a neurological phenotype in mice transgenic for the mutation of Huntington's disease. Brain Res. 862, 234-237.
41. Perez-Severiano F., Santamaria A., Pedraza-Chaverri J., Medina-Campos O. N., Rios C. and Segovia J. (2004) Increased formation of reactive oxygen species, but no changes in glutathione peroxidase activity, in striata of mice transgenic for the Huntington's disease mutation. Neurochem. Res. 29, 729-733.
42. Petersen A., Hansson O., Puschban Z. et al. (2001) Mice transgenic for exon 1 of the Huntington's disease gene display reduced striatal sensitivity to neurotoxicity induced by dopamine and 6-hydroxydopamine. Eur. J. Neurosci. 14, 1425-1435.
43. Polidori M. C., Mecocci P., Browne S. E., Senin U. and Beal M. F. (1999) Oxidative damage to mitochondrial DNA in Huntington's disease parietal cortex. Neurosci. Lett. 272, 53-56.
44. Ratan R. R., Murphy T. H. and Baraban J. M. (1994) Oxidative stress induces apoptosis in embryonic cortical neurons. J. Neurochem. 62, 376-379.
45. Rivas-Arancibia S., Rodriguez A. I., Zigova T., Willing A. E., Brown W. D., Cahill D. W. and Sanberg P. R. (2001) Taurine increases rat survival and reduces striatal damage caused by 3-nitropropionic acid. Int. J. Neurosci. 108, 55-67.
46. Sato H., Tamba M., Okuno S., Sato K., Keino-Masu K., Masu M. and Bannai S. (2002) Distribution of cystine/glutamate exchange transporter, system x(c)-, in the mouse brain. J. Neurosci. 22, 8028-8033.
47. Schulz J. B., Henshaw D. R., MacGarvey U. and Beal M. F. (1996) Involvement of oxidative stress in 3-nitropropionic acid neurotoxicity. Neurochem. Int. 29, 167-171.
48. Shanker G. and Aschner M. (2001) Identification and characterization of uptake systems for cystine and cysteine in cultured astrocytes and neurons: evidence for methylmercury-targeted disruption of astrocyte transport. J. Neurosci. Res. 66, 998-1002.
49. Trettel F., Rigamonti D., Hilditch-Maguire P., Wheeler V. C., Sharp A. H., Persichetti F., Cattaneo E. and MacDonald M. E. (2000) Dominant phenotypes produced by the HD mutation in STHdh (Q111) striatal cells. Hum. Mol. Genet. 9, 2799-2809.