Potential of cystamine and cysteamine in the treatment of neurodegenerative diseases

Progress in Neuro-Psychopharmacology and Biological Psychiatry

Volumn 35, Issue 2, 2011, Pages 380-389

  Gibrat, C ^a   Cicchetti, F ^a,b  

^a LAVAL UNIVERSITY   (Canada)

^b UNIVERSITÉ LAVAL   (Canada)

Author keywords

Huntington's disease; Neuroprotection; Neurorescue; Neurorestoration; Parkinson's disease

Indexed keywords

CYSTAMINE; HEAT SHOCK PROTEIN; MERCAPTAMINE; NEUROTROPHIC FACTOR; PROTEIN GLUTAMINE GAMMA GLUTAMYLTRANSFERASE;

APOPTOSIS; ARTICLE; BLOOD BRAIN BARRIER; DISORDERS OF MITOCHONDRIAL FUNCTIONS; DRUG DOSE COMPARISON; DRUG DOSE INCREASE; DRUG EFFICACY; DRUG MECHANISM; DRUG PENETRATION; DRUG RESEARCH; DRUG TARGETING; DRUG TOLERABILITY; ENZYME INHIBITION; EXPERIMENTAL MODEL; HUMAN; HUNTINGTON CHOREA; LOW DRUG DOSE; MOTOR DYSFUNCTION; NAUSEA; NERVE CELL NECROSIS; NERVE REGENERATION; NERVOUS SYSTEM INFLAMMATION; NEUROPATHOLOGY; NEUROPROTECTION; NONHUMAN; OXIDATIVE STRESS; PARKINSON DISEASE; PROTEIN AGGREGATION; PROTEIN EXPRESSION;

ANIMALS; CYSTAMINE; CYSTEAMINE; DISEASE MODELS, ANIMAL; ENZYME INHIBITORS; HUMANS; HUNTINGTON DISEASE; MICE; MICE, TRANSGENIC; MOLECULAR TARGETED THERAPY; NEURODEGENERATIVE DISEASES; NEUROPROTECTIVE AGENTS; PARKINSON DISEASE; RADIATION-PROTECTIVE AGENTS;

EID: 79952898732     PISSN: 02785846     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.pnpbp.2010.11.023     Document Type: Article

References (158)

1. Aoi M., Date I., Tomita S., Ohmoto T. GDNF induces recovery of the nigrostriatal dopaminergic system in the rat brain following intracerebroventricular or intraparenchymal administration. Acta Neurochir 2000, 142:805-810.
2. Aoi M., Date I., Tomita S., Ohmoto T. Single or continuous injection of glial cell line-derived neurotrophic factor in the striatum induces recovery of the nigrostriatal dopaminergic system. Neurol Res 2000, 22:832-836.
3. Apostol B.L., Kazantsev A., Raffioni S., Illes K., Pallos J., Bodai L., et al. A cell-based assay for aggregation inhibitors as therapeutics of polyglutamine-repeat disease and validation in Drosophila. Proc Natl Acad Sci USA 2003, 100(10):5950-5955.
4. Arrasate M., Mitra S., Schweitzer E.S., Segal M.R., Finkbeiner S. Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death. Nature 2004, 431:805-810.
5. Auluck P.K., Bonini N.M. Pharmacological prevention of Parkinson disease in Drosophila. Nat Med 2002, 8:1185-1186.
6. Bacq Z.M., Beaumariage M.L., Van Caneghem P. Importance for radioprotective effect in mammals of pharmacological and biochemical actions of cysteamine and related substances. Ann Ist Super Sanità 1965, 1:639-645.
7. Bailey C.D., Johnson G.V. Tissue transglutaminase contributes to disease progression in the R6/2 Huntington's disease mouse model via aggregate-independent mechanisms. J Neurochem 2005, 92:83-92.
8. Bailey C.D., Johnson G.V. The protective effects of cystamine in the R6/2 Huntington's disease mouse involve mechanisms other than the inhibition of tissue transglutaminase. Neurobiol Aging 2006, 27:871-879.
9. Baquet Z.C., Bickford P.C., Jones K.R. Brain-derived neurotrophic factor is required for the establishment of the proper number of dopaminergic neurons in the substantia nigra pars compacta. J Neurosci 2005, 25:6251-6259.
10. Beal M.F. Bioenergetic approaches for neuroprotection in Parkinson's disease. Ann Neurol 2003, 53(Suppl 3):S39-S47. discussion S-8.
11. Bensinger S.J., Tontonoz P. A Nurr1 pathway for neuroprotection. Cell 2009, 137:26-28.
12. Berruyer C., Martin F.M., Castellano R., Macone A., Malergue F., Garrido-Urbani S., et al. Vanin-1-/- mice exhibit a glutathione-mediated tissue resistance to oxidative stress. Mol Cell Biol 2004, 24:7214-7224.
13. Bharath S., Hsu M., Kaur D., Rajagopalan S., Andersen J.K. Glutathione, iron and Parkinson's disease. Biochem Pharmacol 2002, 64:1037-1048.
14. Bibel M., Barde Y.A. Neurotrophins: key regulators of cell fate and cell shape in the vertebrate nervous system. Genes Dev 2000, 14:2919-2937.
15. Biziagos E., Crance J.M., Passagot J., Deloince R. Effect of antiviral substances on hepatitis A virus replication in vitro. J Med Virol 1987, 22:57-66.
16. Bjorkqvist M., Wild E.J., Thiele J., Silvestroni A., Andre R., Lahiri N., et al. A novel pathogenic pathway of immune activation detectable before clinical onset in Huntington's disease. J Exp Med 2008, 205:1869-1877.
17. Boka G., Anglade P., Wallach D., Javoy-Agid F., Agid Y., Hirsch E.C. Immunocytochemical analysis of tumor necrosis factor and its receptors in Parkinson's disease. Neurosci Lett 1994, 172:151-154.
18. Borrell-Pages M., Canals J.M., Cordelieres F.P., Parker J.A., Pineda J.R., Grange G., et al. Cystamine and cysteamine increase brain levels of BDNF in Huntington disease via HSJ1b and transglutaminase. J Clin Invest 2006, 116:1410-1424.
19. Bousquet M., Gibrat C., Ouellet M., Rouillard C., Calon F., Cicchetti F. Cystamine metabolism and brain transport properties: clinical implications for neurodegenerative diseases. J Neurochem 2010, 114:1651-1658.
20. Caccamo D., Curro M., Condello S., Ferlazzo N., Ientile R. Critical role of transglutaminase and other stress proteins during neurodegenerative processes. Amino Acids 2010, 38:653-658.
21. Calkins M.J., Townsend J.A., Johnson D.A., Johnson J.A. Cystamine protects from 3-nitropropionic acid lesioning via induction of nf-e2 related factor 2 mediated transcription. Exp Neurol 2010, 224:307-317.
22. Cardoso F. Huntington disease and other choreas. Neurol Clin 2009, 27:719-736. vi.
23. Chai Y., Koppenhafer S.L., Shoesmith S.J., Perez M.K., Paulson H.L. Evidence for proteasome involvement in polyglutamine disease: localization to nuclear inclusions in SCA3/MJD and suppression of polyglutamine aggregation in vitro. Hum Mol Genet 1999, 8:673-682.
24. Chang D.T., Rintoul G.L., Pandipati S., Reynolds I.J. Mutant huntingtin aggregates impair mitochondrial movement and trafficking in cortical neurons. Neurobiol Dis 2006, 22:388-400.
25. Chang M.L., Klaidman L.K., Adams J.D. The effects of oxidative stress on in vivo brain GSH turnover in young and mature mice. Mol Chem Neuropathol 1997, 30:187-197.
26. Checkoway H., Farin F.M., Costa-Mallen P., Kirchner S.C., Costa L.G. Genetic polymorphisms in Parkinson's disease. Neurotoxicology 1998, 19:635-643.
27. Checkoway H., Nelson L.M. Epidemiologic approaches to the study of Parkinson's disease etiology. Epidemiology 1999, 10:327-336.
28. Coloso R.M., Hirschberger L.L., Dominy J.E., Lee J.I., Stipanuk M.H. Cysteamine dioxygenase: evidence for the physiological conversion of cysteamine to hypotaurine in rat and mouse tissues. Adv Exp Med Biol 2006, 583:25-36.
29. Cooper J.K., Schilling G., Peters M.F., Herring W.J., Sharp A.H., Kaminsky Z., et al. Truncated N-terminal fragments of huntingtin with expanded glutamine repeats form nuclear and cytoplasmic aggregates in cell culture. Hum Mol Genet 1998, 7:783-790.
30. Dalrymple A., Wild E.J., Joubert R., Sathasivam K., Bjorkqvist M., Petersen A., et al. Proteomic profiling of plasma in Huntington's disease reveals neuroinflammatory activation and biomarker candidates. J Proteome Res 2007, 6:2833-2840.
31. Danzer K.M., Ruf W.P., Putcha P., Joyner D., Hashimoto T., Glabe C., et al. Heat-shock protein 70 modulates toxic extracellular {alpha}-synuclein oligomers and rescues trans-synaptic toxicity. Faseb J 2010, [Epub ahead of print].
32. Dawbarn D., Allen S.J. Neurotrophins and neurodegeneration. Neuropathol Appl Neurobiol 2003, 29:211-230.
33. Dedeoglu A., Kubilus J.K., Jeitner T.M., Matson S.A., Bogdanov M., Kowall N.W., et al. Therapeutic effects of cystamine in a murine model of Huntington's disease. J Neurosci 2002, 22:8942-8950.
34. Demarco C., Bombardieri G., Riva F., Dupre S., Cavallini D. Degradation of cystaldimine, the product of oxidative deamination of cystamine. Biochim Biophys Acta 1965, 100:89-97.
35. Di Leandro L., Maras B., Schinina M.E., Dupre S., Koutris I., Martin F.M., et al. Cystamine restores GSTA3 levels in Vanin-1 null mice. Free Radic Biol Med 2008, 44:1088-1096.
36. Dong Z., Wolfer D.P., Lipp H.P., Bueler H. Hsp70 gene transfer by adeno-associated virus inhibits MPTP-induced nigrostriatal degeneration in the mouse model of Parkinson disease. Mol Ther 2005, 11:80-88.
37. Dubinsky R., Gray C. CYTE-I-HD: phase I dose finding and tolerability study of cysteamine (Cystagon) in Huntington's disease. Mov Disord 2006, 21:530-533.
38. Dubow J.S. Autonomic dysfunction in Parkinson's disease. Dis Mon 2007, 53:265-274.
39. Emerich D.F., Winn S.R., Christenson L., Palmatier M.A., Gentile F.T., Sanberg P.R. A novel approach to neural transplantation in Parkinson's disease: use of polymer-encapsulated cell therapy. Neurosci Biobehav Rev 1992, 16:437-447.
40. Fitzmaurice P.S., Ang L., Guttman M., Rajput A.H., Furukawa Y., Kish S.J. Nigral glutathione deficiency is not specific for idiopathic Parkinson's disease. Mov Disord 2003, 18:969-976.
41. Fox J.H., Barber D.S., Singh B., Zucker B., Swindell M.K., Norflus F., et al. Cystamine increases L-cysteine levels in Huntington's disease transgenic mouse brain and in a PC12 model of polyglutamine aggregation. J Neurochem 2004, 91:413-422.
42. Fumagalli F., Racagni G., Riva M.A. Shedding light into the role of BDNF in the pharmacotherapy of Parkinson's disease. Pharmacogenomics J 2006, 6:95-104.
43. Galpern W.R., Frim D.M., Tatter S.B., Altar C.A., Beal M.F., Isacson O. Cell-mediated delivery of brain-derived neurotrophic factor enhances dopamine levels in an MPP+ rat model of substantia nigra degeneration. Cell Transplant 1996, 5:225-232.
44. Gibrat C., Bousquet M., Saint-Pierre M., Levesque D., Calon F., Rouillard C., et al. Cystamine prevents MPTP-induced toxicity in young adult mice via the up-regulation of the brain-derived neurotrophic factor. Prog Neuropsychopharmacol Biol Psychiatry 2010, 34:193-203.
45. Gorman A.M., Szegezdi E., Quigney D.J., Samali A. Hsp27 inhibits 6-hydroxydopamine-induced cytochrome c release and apoptosis in PC12 cells. Biochem Biophys Res Commun 2005, 327:801-810.
46. Griffin M., Casadio R., Bergamini C.M. Transglutaminases: nature's biological glues. Biochem J 2002, 368:377-396.
47. Grinberg L.T., Rueb U., Alho A.T., Heinsen H. Brainstem pathology and non-motor symptoms in PD. J Neurol Sci 2010, 289:81-88.
48. Halliday G.M., McCann H. The progression of pathology in Parkinson's disease. Ann NY Acad Sci 2010, 1184:188-195.
49. Hamzehei M., Ledinko N. Inhibition of influenza A virus replication by rifampicin and selenocystamine. J Med Virol 1980, 6:169-174.
50. Harris M.K., Shneyder N., Borazanci A., Korniychuk E., Kelley R.E., Minagar A. Movement disorders. Med Clin North Am 2009, 93:371-388. viii.
51. Hazeki N., Nakamura K., Goto J., Kanazawa I. Rapid aggregate formation of the huntingtin N-terminal fragment carrying an expanded polyglutamine tract. Biochem Biophys Res Commun 1999, 256:361-366.
52. Henchcliffe C., Beal M.F. Mitochondrial biology and oxidative stress in Parkinson disease pathogenesis. Nat Clin Pract Neurol 2008, 4:600-609.
53. Hirsch E.C., Breidert T., Rousselet E., Hunot S., Hartmann A., Michel P.P. The role of glial reaction and inflammation in Parkinson's disease. Ann NY Acad Sci 2003, 991:214-228.
54. Hirsch E.C., Hunot S. Neuroinflammation in Parkinson's disease: a target for neuroprotection?. Lancet Neurol 2009, 8:382-397.
55. Hodges A., Strand A.D., Aragaki A.K., Kuhn A., Sengstag T., Hughes G., et al. Regional and cellular gene expression changes in human Huntington's disease brain. Hum Mol Genet 2006, 15:965-977.
56. Hsu T.C., Chen Y.C., Lai W.X., Chiang S.Y., Huang C.Y., Tzang B.S. Beneficial effects of treatment with cystamine on brain in NZB/WF1 mice. Eur J Pharmacol 2008, 591:307-314.
57. Huxtable R.J. Physiological actions of taurine. Physiol Rev 1992, 72:101-163.
58. Jain A., Martensson J., Stole E., Auld P.A., Meister A. Glutathione deficiency leads to mitochondrial damage in brain. Proc Natl Acad Sci USA 1991, 88:1913-1917.
59. Jeitner T.M., Delikatny E.J., Ahlqvist J., Capper H., Cooper A.J. Mechanism for the inhibition of transglutaminase 2 by cystamine. Biochem Pharmacol 2005, 69:961-970.
60. Junn E., Ronchetti R.D., Quezado M.M., Kim S.Y., Mouradian M.M. Tissue transglutaminase-induced aggregation of alpha-synuclein: implications for Lewy body formation in Parkinson's disease and dementia with Lewy bodies. Proc Natl Acad Sci USA 2003, 100:2047-2052.
61. Karpuj M.V., Becher M.W., Springer J.E., Chabas D., Youssef S., Pedotti R., et al. Prolonged survival and decreased abnormal movements in transgenic model of Huntington disease, with administration of the transglutaminase inhibitor cystamine. Nat Med 2002, 8:143-149.
62. Kaufmann H., Nahm K., Purohit D., Wolfe D. Autonomic failure as the initial presentation of Parkinson disease and dementia with Lewy bodies. Neurology 2004, 63:1093-1095.
63. Kirik D., Georgievska B., Rosenblad C., Bjorklund A. Delayed infusion of GDNF promotes recovery of motor function in the partial lesion model of Parkinson's disease. Eur J Neurosci 2001, 13:1589-1599.
64. Klegeris A., McGeer E.G., McGeer P.L. Therapeutic approaches to inflammation in neurodegenerative disease. Curr Opin Neurol 2007, 20:351-357.
65. Klepac N., Relja M., Klepac R., Hecimovic S., Babic T., Trkulja V. Oxidative stress parameters in plasma of Huntington's disease patients, asymptomatic Huntington's disease gene carriers and healthy subjects: a cross-sectional study. J Neurol 2007, 254:1676-1683.
66. Kleta R., Gahl W.A. Pharmacological treatment of nephropathic cystinosis with cysteamine. Expert Opin Pharmacother 2004, 5:2255-2262.
67. Klucken J., Shin Y., Masliah E., Hyman B.T., McLean P.J. Hsp70 reduces alpha-synuclein aggregation and toxicity. J Biol Chem 2004, 279:25497-25502.
68. Knott C., Stern G., Wilkin G.P. Inflammatory regulators in Parkinson's disease: iNOS, lipocortin-1, and cyclooxygenases-1 and -2. Mol Cell Neurosci 2000, 16:724-739.
69. Kollmann G., Castel N., Shapiro B. Further studies on protection of DNA against ionizing radiation. Int J Radiat Biol Relat Stud Phys Chem Med 1970, 18:587-594.
70. Koo H.N., Hong S.H., Kim C.Y., Ahn J.W., Lee Y.G., Kim J.J., et al. Inhibitory effect of apoptosis in human astrocytes CCF-STTG1 cells by lemon oil. Pharmacol Res 2002, 45:469-473.
71. Kordower J.H. In vivo gene delivery of glial cell line--derived neurotrophic factor for Parkinson's disease. Ann Neurol 2003, 53(Suppl 3):S120-S132. discussion S32-4.
72. Kordower J.H., Emborg M.E., Bloch J., Ma S.Y., Chu Y., Leventhal L., et al. Neurodegeneration prevented by lentiviral vector delivery of GDNF in primate models of Parkinson's disease. Science 2000, 290:767-773.
73. Krogh K., Ostergaard K., Sabroe S., Laurberg S. Clinical aspects of bowel symptoms in Parkinson's disease. Acta Neurol Scand 2008, 117:60-64.
74. Kuemmerle S., Gutekunst C.A., Klein A.M., Li X.J., Li S.H., Beal M.F., et al. Huntington aggregates may not predict neuronal death in Huntington's disease. Ann Neurol 1999, 46:842-849.
75. Lang A.E. Clinical trials of disease-modifying therapies for neurodegenerative diseases: the challenges and the future. Nat Med 2010, 16:1223-1226.
76. Lee J.I., Londono M., Hirschberger L.L., Stipanuk M.H. Regulation of cysteine dioxygenase and gamma-glutamylcysteine synthetase is associated with hepatic cysteine level. J Nutr Biochem 2004, 15:112-122.
77. Lesort M., Chun W., Johnson G.V., Ferrante R.J. Tissue transglutaminase is increased in Huntington's disease brain. J Neurochem 1999, 73:2018-2027.
78. Lesort M., Lee M., Tucholski J., Johnson G.V. Cystamine inhibits caspase activity. Implications for the treatment of polyglutamine disorders. J Biol Chem 2003, 278:3825-3830.
79. Levivier M., Przedborski S., Bencsics C., Kang U.J. Intrastriatal implantation of fibroblasts genetically engineered to produce brain-derived neurotrophic factor prevents degeneration of dopaminergic neurons in a rat model of Parkinson's disease. J Neurosci 1995, 15:7810-7820.
80. Lewis S.J., Foltynie T., Blackwell A.D., Robbins T.W., Owen A.M., Barker R.A. Heterogeneity of Parkinson's disease in the early clinical stages using a data driven approach. J Neurol Neurosurg Psychiatry 2005, 76:343-348.
81. Lorand L. DRPLA aggregation and transglutaminase, revisited. Nat Genet 1998, 20:231.
82. Lorand L., Conrad S.M. Transglutaminases. Mol Cell Biochem 1984, 58:9-35.
83. Maguire-Zeiss K.A. alpha-Synuclein: a therapeutic target for Parkinson's disease?. Pharmacol Res 2008, 58:271-280.
84. Mandel S., Grunblatt E., Riederer P., Gerlach M., Levites Y., Youdim M.B. Neuroprotective strategies in Parkinson's disease: an update on progress. CNS Drugs 2003, 17:729-762.
85. Mastroberardino P.G., Iannicola C., Nardacci R., Bernassola F., De Laurenzi V., Melino G., et al. Tissue transglutaminase ablation reduces neuronal death and prolongs survival in a mouse model of Huntington's disease. Cell Death Differ 2002, 9:873-880.
86. McGeer P.L., Itagaki S., Boyes B.E., McGeer E.G. Reactive microglia are positive for HLA-DR in the substantia nigra of Parkinson's and Alzheimer's disease brains. Neurology 1988, 38:1285-1291.
87. McGeer P.L., McGeer E.G. Glial reactions in Parkinson's disease. Mov Disord 2008, 23:474-483.
88. McGeer P.L., Yasojima K., McGeer E.G. Inflammation in Parkinson's disease. Adv Neurol 2001, 86:83-89.
89. Meade C.A., Deng Y.P., Fusco F.R., Del Mar N., Hersch S., Goldowitz D., et al. Cellular localization and development of neuronal intranuclear inclusions in striatal and cortical neurons in R6/2 transgenic mice. J Comp Neurol 2002, 449:241-269.
90. Mitra S., Tsvetkov A.S., Finkbeiner S. Single neuron ubiquitin-proteasome dynamics accompanying inclusion body formation in huntington disease. J Biol Chem 2009, 284:4398-4403.
91. Mitsui K., Nakayama H., Akagi T., Nekooki M., Ohtawa K., Takio K., et al. Purification of polyglutamine aggregates and identification of elongation factor-1alpha and heat shock protein 84 as aggregate-interacting proteins. J Neurosci 2002, 22:9267-9277.
92. Mogi M., Harada M., Riederer P., Narabayashi H., Fujita K., Nagatsu T. Tumor necrosis factor-alpha (TNF-alpha) increases both in the brain and in the cerebrospinal fluid from parkinsonian patients. Neurosci Lett 1994, 165:208-210.
93. Muchowski P.J., Wacker J.L. Modulation of neurodegeneration by molecular chaperones. Nat Rev Neurosci 2005, 6:11-22.
94. Muramatsu M., Kakita K., Nakagawa K., Kuriyama K. A modulating role of taurine on release of acetylcholine and norepinephrine from neuronal tissues. Jpn J Pharmacol 1978, 28:259-268.
95. Murer M.G., Yan Q., Raisman-Vozari R. Brain-derived neurotrophic factor in the control human brain, and in Alzheimer's disease and Parkinson's disease. Prog Neurobiol 2001, 63:71-124.
96. Newton G.L., Aguilera J.A., Ward J.F., Fahey R.C. Effect of polyamine-induced compaction and aggregation of DNA on the formation of radiation-induced strand breaks: quantitative models for cellular radiation damage. Radiat Res 1997, 148:272-284.
97. Nucifora F.C., Sasaki M., Peters M.F., Huang H., Cooper J.K., Yamada M., et al. Interference by huntingtin and atrophin-1 with cbp-mediated transcription leading to cellular toxicity. Science 2001, 291:2423-2428.
98. O'Keeffe G.C., Michell A.W., Barker R.A. Biomarkers in Huntington's and Parkinson's Disease. Ann NY Acad Sci 2009, 1180:97-110.
99. Ogawa O., Zhu X., Perry G., Smith M.A. Mitochondrial abnormalities and oxidative imbalance in neurodegenerative disease. Sci Aging Knowl Environ 2002, 2002:pe16.
100. Olanow C.W., Kieburtz K., Schapira A.H. Why have we failed to achieve neuroprotection in Parkinson's disease?. Ann Neurol 2008, 64(Suppl 2):S101-S110.
101. Park D., Choi S.S., Ha K.S. Transglutaminase 2: a multi-functional protein in multiple subcellular compartments. Amino Acids 2010, 39:619-631.
102. Pasantes-Morales H., Arzate N.E., Cruz C. The role of taurine in nervous tissue: its effects on ionic fluxes. Adv Exp Med Biol 1981, 139:273-292.
103. Pavese N., Gerhard A., Tai Y.F., Ho A.K., Turkheimer F., Barker R.A., et al. Microglial activation correlates with severity in Huntington disease: a clinical and PET study. Neurology 2006, 66:1638-1643.
104. Pearce R.K., Owen A., Daniel S., Jenner P., Marsden C.D. Alterations in the distribution of glutathione in the substantia nigra in Parkinson's disease. J Neural Transm 1997, 104:661-677.
105. Perez M.K., Paulson H.L., Pendse S.J., Saionz S.J., Bonini N.M., Pittman R.N. Recruitment and the role of nuclear localization in polyglutamine-mediated aggregation. J Cell Biol 1998, 143:1457-1470.
106. Perry T.L., Yong V.W. Idiopathic Parkinson's disease, progressive supranuclear palsy and glutathione metabolism in the substantia nigra of patients. Neurosci Lett 1986, 67:269-274.
107. Peterson T.C., Brown I.R. Cysteamine in combination with N-acetylcysteine prevents acetaminophen-induced hepatotoxicity. Can J Physiol Pharmacol 1992, 70:20-28.
108. Pillai A., Veeranan-Karmegam R., Dhandapani K.M., Mahadik S.P. Cystamine prevents haloperidol-induced decrease of BDNF/TrkB signaling in mouse frontal cortex. J Neurochem 2008, 107:941-951.
109. Pinto J.T., Khomenko T., Szabo S., McLaren G.D., Denton T.T., Krasnikov B.F., et al. Measurement of sulfur-containing compounds involved in the metabolism and transport of cysteamine and cystamine. Regional differences in cerebral metabolism. J Chromatogr B Analyt Technol Biomed Life Sci 2009, 877:3434-3441.
110. Pinto J.T., Van Raamsdonk J.M., Leavitt B.R., Hayden M.R., Jeitner T.M., Thaler H.T., et al. Treatment of YAC128 mice and their wild-type littermates with cystamine does not lead to its accumulation in plasma or brain: implications for the treatment of Huntington disease. J Neurochem 2005, 94:1087-1101.
111. Pitari G., Maurizi G., Flati V., Ursini C.L., Spera L., Dupre S., et al. Enzymatic synthesis of S-aminoethyl-L-cysteine from pantetheine. Biochim Biophys Acta 1992, 1116:27-33.
112. Poewe W. Dysautonomia and cognitive dysfunction in Parkinson's disease. Mov Disord 2007, 22(Suppl 17):S374-S378.
113. Porritt M.J., Batchelor P.E., Howells D.W. Inhibiting BDNF expression by antisense oligonucleotide infusion causes loss of nigral dopaminergic neurons. Exp Neurol 2005, 192:226-234.
114. Prescott L.F., Sutherland G.R., Park J., Smith I.J., Proudfoot A.T. Cysteamine, methionine, and penicillamine in the treatment of paracetamol poisoning. Lancet 1976, 2:109-113.
115. Ratan R.R., Murphy T.H., Baraban J.M. Oxidative stress induces apoptosis in embryonic cortical neurons. J Neurochem 1994, 62:376-379.
116. Riederer P., Sian J., Gerlach M. Is there neuroprotection in Parkinson syndrome?. J Neurol 2000, 247(Suppl 4):IV/8-IV/11.
117. Saijo K., Winner B., Carson C.T., Collier J.G., Boyer L., Rosenfeld M.G., et al. A Nurr1/CoREST pathway in microglia and astrocytes protects dopaminergic neurons from inflammation-induced death. Cell 2009, 137:47-59.
118. Salam O.M. Modulation of inflammatory paw oedema by cysteamine in the rat. Pharmacol Res 2002, 45:275-284.
119. Sapp E., Kegel K.B., Aronin N., Hashikawa T., Uchiyama Y., Tohyama K., et al. Early and progressive accumulation of reactive microglia in the Huntington disease brain. J Neuropathol Exp Neurol 2001, 60:161-172.
120. Saudou F., Finkbeiner S., Devys D., Greenberg M.E. Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions. Cell 1998, 95:55-66.
121. Schapira A.H. Neuroprotection and dopamine agonists. Neurology 2002, 58:S9-S18.
122. Sechi G., Deledda M.G., Bua G., Satta W.M., Deiana G.A., Pes G.M., et al. Reduced intravenous glutathione in the treatment of early Parkinson's disease. Prog Neuropsychopharmacol Biol Psychiatry 1996, 20:1159-1170.
123. Seroogy K.B., Lundgren K.H., Tran T.M., Guthrie K.M., Isackson P.J., Gall C.M. Dopaminergic neurons in rat ventral midbrain express brain-derived neurotrophic factor and neurotrophin-3 mRNAs. J Comp Neurol 1994, 342:321-334.
124. Sharma R., Kodavanti U.P., Smith L.L., Mehendale H.M. The uptake and metabolism of cystamine and taurine by isolated perfused rat and rabbit lungs. Int J Biochem Cell Biol 1995, 27:655-664.
125. Shieh C.H., Hong C.J., Huang Y.H., Tsai S.J. Potential antidepressant properties of cysteamine on hippocampal BDNF levels and behavioral despair in mice. Prog Neuropsychopharmacol Biol Psychiatry 2008, 32:1590-1594.
126. Shin J.Y., Fang Z.H., Yu Z.X., Wang C.E., Li S.H., Li X.J. Expression of mutant huntingtin in glial cells contributes to neuronal excitotoxicity. J Cell Biol 2005, 171:1001-1012.
127. Shin Y., Klucken J., Patterson C., Hyman B.T., McLean P.J. The co-chaperone carboxyl terminus of Hsp70-interacting protein (CHIP) mediates alpha-synuclein degradation decisions between proteasomal and lysosomal pathways. J Biol Chem 2005, 280:23727-23734.
128. Shults C., Steardo L., Barone P., Mohr E., Juncos J., Serrati C., et al. Huntington's disease: effect of cysteamine, a somatostatin-depleting agent. Neurology 1986, 36:1099-1102.
129. Sian J., Dexter D.T., Lees A.J., Daniel S., Agid Y., Javoy-Agid F., et al. Alterations in glutathione levels in Parkinson's disease and other neurodegenerative disorders affecting basal ganglia. Ann Neurol 1994, 36:348-355.
130. Singhrao S.K., Neal J.W., Morgan B.P., Gasque P. Increased complement biosynthesis by microglia and complement activation on neurons in Huntington's disease. Exp Neurol 1999, 159:362-376.
131. Smoluk G.D., Fahey R.C., Ward J.F. Equilibrium dialysis studies of the binding of radioprotector compounds to DNA. Radiat Res 1986, 107:194-204.
132. Sofic E., Lange K.W., Jellinger K., Riederer P. Reduced and oxidized glutathione in the substantia nigra of patients with Parkinson's disease. Neurosci Lett 1992, 142:128-130.
133. Stack E.C., Ferro J.L., Kim J., Del Signore S.J., Goodrich S., Matson S., et al. Therapeutic attenuation of mitochondrial dysfunction and oxidative stress in neurotoxin models of Parkinson's disease. Biochim Biophys Acta 2008, 1782:151-162.
134. Steffan J.S., Kazantsev A., Spasic-Boskovic O., Greenwald M., Zhu Y.Z., 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, 97:6763-6768.
135. Stuart B.O., Stannard J.N. The radioprotection of Saccharomyces cerevisiae by cystamine as measured by different criteria of cell damage. Radiat Res 1966, 28:609-623.
136. Su X., Federoff H.J., Maguire-Zeiss K.A. Mutant alpha-synuclein overexpression mediates early proinflammatory activity. Neurotox Res 2009, 16:238-254.
137. Su X., Maguire-Zeiss K.A., Giuliano R., Prifti L., Venkatesh K., Federoff H.J. Synuclein activates microglia in a model of Parkinson's disease. Neurobiol Aging 2008, 29:1690-1701.
138. Sugama S., Takenouchi T., Cho B.P., Joh T.H., Hashimoto M., Kitani H. Possible roles of microglial cells for neurotoxicity in clinical neurodegenerative diseases and experimental animal models. Inflamm Allergy Drug Targets 2009, 8:277-284.
139. Sun L., Xu S., Zhou M., Wang C., Wu Y., Chan P. Effects of cysteamine on MPTP-induced dopaminergic neurodegeneration in mice. Brain Res 2010, 1335:74-82.
140. Tai Y.F., Pavese N., Gerhard A., Tabrizi S.J., Barker R.A., Brooks D.J., et al. Imaging microglial activation in Huntington's disease. Brain Res Bull 2007, 72:148-151.
141. Taylor J.P., Tanaka F., Robitschek J., Sandoval C.M., Taye A., Markovic-Plese S., et al. Aggresomes protect cells by enhancing the degradation of toxic polyglutamine-containing protein. Hum Mol Genet 2003, 12:749-757.
142. Thiruchelvam M.J., Powers J.M., Cory-Slechta D.A., Richfield E.K. Risk factors for dopaminergic neuron loss in human alpha-synuclein transgenic mice. Eur J Neurosci 2004, 19:845-854.
143. Tolosa E., Compta Y., Gaig C. The premotor phase of Parkinson's disease. Parkinsonism Relat Disord 2007, 13(Suppl):S2-S7.
144. Toohey J.I. Sulfur metabolism in AIDS: cystamine as an anti-HIV agent. AIDS Res Hum Retroviruses 2009, 25:1057-1060.
145. Tremblay M.E., Saint-Pierre M., Bourhis E., Levesque D., Rouillard C., Cicchetti F. Neuroprotective effects of cystamine in aged parkinsonian mice. Neurobiol Aging 2006, 27:862-870.
146. Ueland P.M., Mansoor M.A., Guttormsen A.B., Muller F., Aukrust P., Refsum H., et al. Reduced, oxidized and protein-bound forms of homocysteine and other aminothiols in plasma comprise the redox thiol status-a possible element of the extracellular antioxidant defense system. J Nutr 1996, 126:1281S-1284S.
147. Van Raamsdonk J.M., Pearson J., Bailey C.D., Rogers D.A., Johnson G.V., Hayden M.R., et al. Cystamine treatment is neuroprotective in the YAC128 mouse model of Huntington disease. J Neurochem 2005, 95:210-220.
148. Vasilescu D., Rix-Montel M.A. Interaction of sulfur-containing radioprotectors with DNA: a spectrophotometric study. Physiol Chem Phys 1980, 12:51-55.
149. Volpicelli F., Caiazzo M., Greco D., Consales C., Leone L., Perrone-Capano C., et al. Bdnf gene is a downstream target of Nurr1 transcription factor in rat midbrain neurons in vitro. J Neurochem 2007, 102:441-453.
150. von Bohlen und Halbach O., Krause S., Medina D., Sciarretta C., Minichiello L., Unsicker K. Regional- and age-dependent reduction in trkB receptor expression in the hippocampus is associated with altered spine morphologies. Biol Psychiatry 2006, 59:793-800.
151. Wang X., Sarkar A., Cicchetti F., Yu M., Zhu A., Jokivarsi K., et al. Cerebral PET imaging and histological evidence of transglutaminase inhibitor cystamine induced neuroprotection in transgenic R6/2 mouse model of Huntington's disease. J Neurol Sci 2005, 231:57-66.
152. Whitton P.S. Inflammation as a causative factor in the aetiology of Parkinson's disease. Br J Pharmacol 2007, 150:963-976.
153. Yasuhara T., Shingo T., Muraoka K., Wen Ji.Y., Kameda M., Takeuchi A., et al. The differences between high and low-dose administration of VEGF to dopaminergic neurons of in vitro and in vivo Parkinson's disease model. Brain Res 2005, 1038:1-10.
154. Zhang W., Wang T., Pei Z., Miller D.S., Wu X., Block M.L., et al. Aggregated alpha-synuclein activates microglia: a process leading to disease progression in Parkinson's disease. Faseb J 2005, 19:533-542.
155. Zheng S., Newton G.L., Gonick G., Fahey R.C., Ward J.F. Radioprotection of DNA by thiols: relationship between the net charge on a thiol and its ability to protect DNA. Radiat Res 1988, 114:11-27.
156. Zuccato C., Cattaneo E. Role of brain-derived neurotrophic factor in Huntington's disease. Prog Neurobiol 2007, 81:294-330.
157. Zuccato C., Marullo M., Conforti P., MacDonald M.E., Tartari M., Cattaneo E. Systematic assessment of BDNF and its receptor levels in human cortices affected by Huntington's disease. Brain Pathol 2008, 18:225-238.
158. Zuchner T., Brundin P. Mutant huntingtin can paradoxically protect neurons from death. Cell Death Differ 2008, 15:435-442.