Soluble N-terminal fragment of mutant Huntingtin protein impairs mitochondrial axonal transport in cultured hippocampal neurons

Neuroscience Bulletin

Volumn 30, Issue 1, 2014, Pages 74-80

  Tian, Jun ^a   Yan, Ya Ping ^a   Zhou, Rui ^b   Lou, Hui Fang ^b   Rong, Ye ^b   Zhang, Bao Rong ^a  

^a THE SECOND AFFILIATED HOSPITAL OF ZHEJIANG UNIVERSITY SCHOOL OF MEDICINE   (China)

^b ZHEJIANG UNIVERSITY SCHOOL OF MEDICINE   (China)

Author keywords

axonal transport; hippocampal neurons; Huntington; mitochondria

Indexed keywords

HUNTINGTIN; MUTANT PROTEIN;

AMINO TERMINAL SEQUENCE; ANIMAL CELL; ARTICLE; CONTROLLED STUDY; EMBRYO; HIPPOCAMPAL NEURONAL CULTURE; HUNTINGTON CHOREA; MITOCHONDRIAL MEMBRANE; NERVE FIBER TRANSPORT; NONHUMAN; PROTEIN EXPRESSION; RAT; TRANSPORT KINETICS;

ANIMALS; AXONAL TRANSPORT; CELLS, CULTURED; HIPPOCAMPUS; HUMANS; MITOCHONDRIA; MUTATION; NERVE TISSUE PROTEINS; NEURONS; RATS; RATS, SPRAGUE-DAWLEY; SOLUBILITY;

EID: 84895796826     PISSN: 16737067     EISSN: 19958218     Source Type: Journal    
DOI: 10.1007/s12264-013-1393-0     Document Type: Article

References (27)

1. Ha AD, Fung VS. Huntington's disease. Curr Opin Neurol 2012, 25: 491-498.
2. Singer C. Comprehensive treatment of Huntington disease and other choreic disorders. Cleve Clin J Med 2012, 79Suppl 2: S30-34.
3. A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. The Huntington's Disease Collaborative Research Group. Cell 1993, 72: 971-983.
4. Goldberg YP, Nicholson DW, Rasper DM, Kalchman MA, Koide HB, Graham RK, et al. Cleavage of huntingtin by apopain, a proapoptotic cysteine protease, is modulated by the polyglutamine tract. Nat Genet 1996, 13: 442-449.
5. Waldron-Roby E, Ratovitski T, Wang X, Jiang M, Watkin E, Arbez N, et al. Transgenic mouse model expressing the caspase 6 fragment of mutant huntingtin. J Neurosci 2012, 32: 183-193.
6. Wellington CL, Singaraja R, Ellerby L, Savill J, Roy S, Leavitt B, et al. Inhibiting caspase cleavage of huntingtin reduces toxicity and aggregate formation in neuronal and nonneuronal cells. J Biol Chem 2000, 275: 19831-19838.
7. Mangiarini L, Sathasivam K, Seller M, Cozens B, Harper A, Hetherington C, et al. Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice. Cell 1996, 87: 493-506.
8. Gutekunst CA, Li SH, Yi H, Mulroy JS, Kuemmerle S, Jones R, et al. Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology. J Neurosci 1999, 19: 2522-2534.
9. Arrasate M, Finkbeiner S. Protein aggregates in Huntington's disease. Exp Neurol 2012, 238: 1-11.
10. Davies SW, Turmaine M, Cozens BA, DiFiglia M, Sharp AH, Ross CA, et al. Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation. Cell 1997, 90: 537-548.
11. Arrasate M, Mitra S, Schweitzer ES, Segal MR, Finkbeiner S. Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death. Nature 2004, 431: 805-810.
12. Saxton WM, Hollenbeck PJ. The axonal transport of mitochondria. J Cell Sci 2012, 125: 2095-2104.
13. Goldstein LS. Axonal transport and neurodegenerative disease: can we see the elephant? Prog Neurobiol 2012, 99: 186-190.
14. Chang DT, Rintoul GL, Pandipati S, Reynolds IJ. Mutant huntingtin aggregates impair mitochondrial movement and trafficking in cortical neurons. Neurobiol Dis 2006, 22: 388-400.
15. Orr AL, Li S, Wang CE, Li H, Wang J, Rong J, et al. N-terminal mutant huntingtin associates with mitochondria and impairs mitochondrial trafficking. J Neurosci 2008, 28: 2783-2792.
16. Lange KW, Sahakian BJ, Quinn NP, Marsden CD, Robbins TW. Comparison of executive and visuospatial memory function in Huntington's disease and dementia of Alzheimer type matched for degree of dementia. J Neurol Neurosurg Psychiatry 1995, 58: 598-606.
17. Spargo E, Everall IP, Lantos PL. Neuronal loss in the hippocampus in Huntington's disease: a comparison with HIV infection. J Neurol Neurosurg Psychiatry 1993, 56: 487-491.
18. Zhang B, Tian J, Yin X, Luo W, Xia K. Protective effect of sodium butyrate on the cell culture model of Huntington disease. Prog Nat Sci 2007, 17: 784-788.
19. Dotti CG, Sullivan CA, Banker GA. The establishment of polarity by hippocampal neurons in culture. J Neurosci 1988, 8: 1454-1468.
20. Kim C, Choi H, Jung ES, Lee W, Oh S, Jeon NL, et al. HDAC6 inhibitor blocks amyloid beta-induced impairment of mitochondrial transport in hippocampal neurons. PLoS One 2012, 7: e42983.
21. Cichon J, Sun C, Chen B, Jiang M, Chen XA, Sun Y, et al. Cofilin aggregation blocks intracellular trafficking and induces synaptic loss in hippocampal neurons. J Biol Chem 2012, 287: 3919-3929.
22. Bilsland LG, Sahai E, Kelly G, Golding M, Greensmith L, Schiavo G. Deficits in axonal transport precede ALS symptoms in vivo. Proc Natl Acad Sci U S A 2010, 107: 20523-20528.
23. Shirendeb U, Reddy AP, Manczak M, Calkins MJ, Mao P, Tagle DA, et al. Abnormal mitochondrial dynamics, mitochondrial loss and mutant huntingtin oligomers in Huntington's disease: implications for selective neuronal damage. Hum Mol Genet 2011, 20: 1438-1455.
24. Reddy PH, Shirendeb UP. Mutant huntingtin, abnormal mitochondrial dynamics, defective axonal transport of mitochondria, and selective synaptic degeneration in Huntington's disease. Biochim Biophys Acta 2012, 1822: 101-110.
25. Itoh K, Nakamura K, Iijima M, Sesaki H. Mitochondrial dynamics in neurodegeneration. Trends Cell Biol 2013, 23: 64-71.
26. Zuccato C, Valenza M, Cattaneo E. Molecular mechanisms and potential therapeutical targets in Huntington's disease. Physiol Rev 2010, 90: 905-981.
27. Trushina E, Dyer RB, Badger JD, 2nd, Ure D, Eide L, Tran DD, et al. Mutant huntingtin impairs axonal trafficking in mammalian neurons in vivo and in vitro. Mol Cell Biol 2004, 24: 8195-8209.