Attenuation of Rhes Activity Significantly Delays the Appearance of Behavioral Symptoms in a Mouse Model of Huntington's Disease

PLoS ONE

Volumn 8, Issue 1, 2013, Pages

  Baiamonte, Brandon A ^a   Lee, Franklin A ^a   Brewer, Steve T ^a   Spano, Daniela ^b,c   LaHoste, Gerald J ^a  

^a UNIVERSITY OF NEW ORLEANS   (United States)

^b UNIVERSITY OF NAPLES FEDERICO II   (Italy)

^c CEINGE BIOTECNOLOGIE AVANZATE   (Italy)

Author keywords

[No Author keywords available]

Indexed keywords

ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ARTICLE; CONTROLLED STUDY; FEMALE; GENE; GENE ACTIVITY; GENE DOSAGE; GENE EXPRESSION; GENE FUNCTION; GENETIC REGULATION; GENOTYPE; HUNTINGTON CHOREA; IN VIVO STUDY; KNOCKOUT MOUSE; MALE; MOUSE; NONHUMAN; RHES GENE; SYMPTOMATOLOGY; TRANSGENIC MOUSE;

ANIMALS; BEHAVIOR, ANIMAL; BODY WEIGHT; BRAIN; DISEASE MODELS, ANIMAL; DYSTONIA; FEMALE; GENE KNOCKOUT TECHNIQUES; GTP-BINDING PROTEINS; HUMANS; HUNTINGTON DISEASE; MALE; MICE; MICE, TRANSGENIC; MOTOR ACTIVITY; NERVE TISSUE PROTEINS;

MUS; MUS MUSCULUS;

EID: 84872674123     PISSN: None     EISSN: 19326203     Source Type: Journal    
DOI: 10.1371/journal.pone.0053606     Document Type: Article

References (29)

1. Huntington G, (1872) On chorea. The Medical and Surgical Reporter 26: 320-321.
2. Huntington's Disease Collaborative Research Group (1993) A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. Cell 72: 971-983.
3. Li SH, Li XJ, (2004) Huntingtin-protein interactions and the pathogenesis of Huntington's disease. Trends Genet 20: 146-154.
4. Ross CA, (2002) Polyglutamine pathogenesis. Neuron 35: 819-822.
5. Zoghbi HY, Orr HT, (2000) Glutamine repeats and neurodegeneration. Annu Rev Neurosci 23: 217-247.
6. Sathasivam K, Amaechi I, Mangiarini L, Bates GP, (1997) Identification of an HD patient with a (CAG)180 repeat expansion and the propagation of highly expanded CAG repeats in lamda phage. Hum Genet 99: 692-695.
7. Rubinsztein DC, Leggo J, Coles R, Almqvist E, Biancalana V, et al. (1996) Phenotypic characterization of individuals with 30-40 CAG repeats in the Huntington's disease (HD) gene reveals HD cases with 36 repeats and apparently normal elderly individuals with 36-39 repeats. Am J Hum Genet 59: 16-22.
8. Andrew SE, Goldberg YP, Kremer B, Telenius H, Theilmann J, et al. (1993) The relationship between trinucleotide (CAG) repeat length and clinical features of Huntington's. Nat Genet 4: 398-403.
9. Landles C, Bates GP, (2004) Huntingtin and the molecular pathogenesis of Huntington's disease. Fourth in molecular medicine review series. EMBO Reports 5: 958-963.
10. Gil JM, Rego AC, (2008) Mechanisms of neurodegeneration in Huntington's disease. Eur J Neurosci 27: 2803-2820.
11. Vonsattel JP, Difiglia M, (1998) Huntington disease. J Neuropathol Exp Neuro 57: 369-384.
12. Pineda JR, Canals JM, Bosch M, Adell A, Mengod G, et al. (2005) Brain-derived neurotrophic factor modulates dopaminergic deficits in a transgenic mouse model of Huntington's disease. J Neurochem 93: 1057-1068.
13. Falk JD, Vargiu P, Foye PE, Usui H, Perez J, et al. (1999) Rhes: A striatal-specific Ras homolog related to Dexras1. J Neurosci Res 57: 782-788.
14. Harrison LM, LaHoste GJ, (2006) Rhes, the Ras homolog enriched in striatum, is reduced under conditions of dopamine supersensitivity. Neuroscience 137: 483-492.
15. Subramaniam S, Sixt KM, Barrow R, Snyder SH, (2009) Rhes, a striatal specific protein, mediates mutant-Huntingtin cytotoxicity. Science 324: 1327-1330.
16. Kummerle S, Gutekunst CA, Klein AM, Li XJ, Li SH, et al. (1999) Huntingtin aggregates may not predict neuronal death in Huntington's disease. Ann Neurol 46: 842-849.
17. Arrasate M, Mitra S, Schweitzer ES, Segal MR, Finkbeiner S, (2004) Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death. Nature 431: 805-810.
18. Steffan JS, Agrawal N, Pallos J, Rockabrand E, Trotman LC, et al. (2004) SUMO modification of Huntingtin and Huntington's disease pathology. Science 304: 100-104.
19. Spano D, Branchi I, Rosica A, Pirro MT, Riccio A, et al. (2004) Rhes is involved in striatal function. Mol Cell Biol 24: 5788-5796.
20. Stack EC, Kubilus JK, Smith K, Cormier K, Del Signore SJ, et al. (2005) Chronology of behavioral symptoms and neuropathological sequel in R6/2 Huntington's disease transgenic mice. J Comp Neurol 490: 354-370.
21. Spalding JF, Thomas RG, Tietjen GL (1982) Life span of C57 mice as influenced by radiation dose, dose rate, and age at exposure. Energy Citations Database. Available: http://www.osti.gov/energycitations/product.biblio.jsp?osti_id=6866926. Accessed 28 August 2012.
22. Mangiarini L, Sathasivam K, Seller M, Cozens BA, Harper A, et al. (1996) Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice. Cell 87: 493-506.
23. Rubinsztein DC, (2002) Lessons from animal models of Huntington's disease. Trends Genet 18: 202-209.
24. Subramaniam S, Mealer RG, Sixt KM, Barrow RK, Usiello A, et al. (2010) Rhes, a physiological regulator of sumoylation, enhances cross-sumoylation between the basic sumoylation enzymes E1 and Ubc9. J Biol Chem 285: 20428-20432.
25. Desterro JM, Rodriguez MS, Hay RT, (1998) SUMO-1 modification of IκBα inhibits NF-κB activation. Mol Cell 2: 233-239.
26. Hoege C, Pfander B, Moldovan GL, Pyrowolakis G, Jentsch S, (2002) RAD6-dependent DNA repair is linked to modification of PCNA by ubiquitin and SUMO. Nature 419: 135-141.
27. Lin X, Liang M, Liang YY, Brunicardi FC, Feng XH, (2003) SUMO-1/Ubc9 promotes nuclear accumulation and metabolic stability of tumor suppressor SMAD4. J Biol Chem 278: 31043-31048.
28. Steffan JS, (2010) Does Huntingtin play a role in selective macroautophagy? Cell Cycle 9: 3401-3413.
29. Klionsky DJ, Abdalla FC, Abeliovich H, Abraham RT, Acevedo-Arozena A, et al. (2012) Guidelines for the use and interpretation of assays for monitoring autophagy. Autophagy 8: 445-544.