Impaired ATF6α processing, decreased Rheb and neuronal cell cycle re-entry in Huntington's disease

Neurobiology of Disease

Volumn 41, Issue 1, 2011, Pages 23-32

  Fernandez Fernandez, Maria Rosario ^a   Ferrer, Isidro ^b   Lucas, Jose J ^a  

^a CENTRO DE BIOLOGÍA MOLECULAR SEVERO OCHOA   (Spain)

^b HOSPITAL UNIVERSITARI DE BELLVITGE   (Spain)

Author keywords

ATF6 ; Cell cycle re entry; Cyclin E; Huntington's disease; Interneurons; Medium size spiny neurons; Polyglutamine diseases; Rheb; Small GTPases; Striatal physiology

Indexed keywords

ACTIVATING TRANSCRIPTION FACTOR 6; ACTIVATING TRANSCRIPTION FACTOR 6 ALPHA; RHEB PROTEIN; UNCLASSIFIED DRUG;

ADULT; AGED; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; BRAIN TISSUE; CELL CYCLE PROGRESSION; CELL SURVIVAL; CLINICAL ARTICLE; CONTROLLED STUDY; ENDOPLASMIC RETICULUM STRESS; FEMALE; HUMAN; HUMAN TISSUE; HUNTINGTON CHOREA; MALE; MITOSIS; MOUSE; NERVE CELL NECROSIS; NEUROPATHOLOGY; NONHUMAN; PATHOPHYSIOLOGY; PRIORITY JOURNAL; PROTEIN AGGREGATION; PROTEIN FUNCTION; PROTEIN PROCESSING; SIGNAL TRANSDUCTION;

ACTIVATING TRANSCRIPTION FACTOR 6; AGED; AGED, 80 AND OVER; ANIMALS; CELL CYCLE; CELL CYCLE PROTEINS; FEMALE; HUMANS; HUNTINGTON DISEASE; MALE; MICE; MIDDLE AGED; MONOMERIC GTP-BINDING PROTEINS; NEURONS; NEUROPEPTIDES;

EID: 78349312360     PISSN: 09699961     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.nbd.2010.08.014     Document Type: Article

References (29)

1. Arrasate M., et al. Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death. Nature 2004, 431:805-810.
2. Brown M.S., et al. Regulated intramembrane proteolysis: a control mechanism conserved from bacteria to humans. Cell 2000, 100:391-398.
3. Buerger C., et al. Localization of Rheb to the endomembrane is critical for its signaling function. Biochem. Biophys. Res. Commun. 2006, 344:869-880.
4. Carnemolla A., et al. Rrs1 is involved in endoplasmic reticulum stress response in Huntington disease. J. Biol. Chem. 2009, 284:18167-18173.
5. Davies S.W., et al. Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation. Cell 1997, 90:537-548.
6. Diaz-Hernandez M., et al. Neuronal induction of the immunoproteasome in Huntington's disease. J. Neurosci. 2003, 23:11653-11661.
7. Duennwald M.L., Lindquist S. Impaired ERAD and ER stress are early and specific events in polyglutamine toxicity. Genes Dev. 2008, 22:3308-3319.
8. Ferrer I., et al. Parvalbumin-immunoreactive neurons in the cerebral cortex and striatum in Huntington's disease. Neurodegeneration 1994, 3:169-173.
9. Gomez-Sintes R., Lucas J.J. NFAT/Fas signaling mediates the neuronal apoptosis and motor side effects of GSK-3 inhibition in a mouse model of lithium therapy. J. Clin. Invest. 2010, 120:2432-2445.
10. Harrington K.M., Kowall N.W. Parvalbumin immunoreactive neurons resist degeneration in Huntington's disease striatum. J. Neuropathol. Exp. Neurol. 1991, 50:309.
11. Haze K., et al. Mammalian transcription factor ATF6 is synthesized as a transmembrane protein and activated by proteolysis in response to endoplasmic reticulum stress. Mol. Biol. Cell 1999, 10:3787-3799.
12. Herrup K., Yang Y. Cell cycle regulation in the postmitotic neuron: oxymoron or new biology?. Nat. Rev. Neurosci. 2007, 8:368-378.
13. Karbowniczek M., et al. Rheb inhibits C-raf activity and B-raf/C-raf heterodimerization. J. Biol. Chem. 2006, 281:25447-25456.
14. Mangiarini L., 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.
15. Manning B.D., Cantley L.C. Rheb fills a GAP between TSC and TOR. Trends Biochem. Sci. 2003, 28:573-576.
16. Matamales M., et al. Striatal medium-sized spiny neurons: identification by nuclear staining and study of neuronal subpopulations in BAC transgenic mice. PLoS ONE 2009, 4:e4770.
17. Matus S., et al. The stress rheostat: an interplay between the unfolded protein response (UPR) and autophagy in neurodegeneration. Curr. Mol. Med. 2008, 8:157-172.
18. Nakayama H., et al. ER stress is the initial response to polyglutamine toxicity in PC12 cells. Biochem. Biophys. Res. Commun. 2008, 377:550-555.
19. Paschen W., Mengesdorf T. Endoplasmic reticulum stress response and neurodegeneration. Cell Calcium 2005, 38:409-415.
20. Patil C., Walter P. Intracellular signaling from the endoplasmic reticulum to the nucleus: the unfolded protein response in yeast and mammals. Curr. Opin. Cell Biol. 2001, 13:349-355.
21. Pelegri C., et al. Cell cycle activation in striatal neurons from Huntington's disease patients and rats treated with 3-nitropropionic acid. Int. J. Dev. Neurosci. 2008, 26:665-671.
22. Ravikumar B., et al. Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease. Nat. Genet. 2004, 36:585-595.
23. Reijonen S., et al. Inhibition of endoplasmic reticulum stress counteracts neuronal cell death and protein aggregation caused by N-terminal mutant huntingtin proteins. Exp. Cell Res. 2008, 314:950-960.
24. Schewe D.M., Aguirre-Ghiso J.A. ATF6alpha-Rheb-mTOR signaling promotes survival of dormant tumor cells in vivo. Proc. Natl Acad. Sci. USA 2008, 105:10519-10524.
25. Subramaniam S., et al. Rhes, a striatal specific protein, mediates mutant-huntingtin cytotoxicity. Science 2009, 324:1327-1330.
26. A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. Cell 1993, 72:971-983. The Huntington's Disease Collaborative Research Group.
27. Vonsattel J.P., DiFiglia M. Huntington disease. J. Neuropathol. Exp. Neurol. 1998, 57:369-384.
28. Yang Y., Herrup K. Cell division in the CNS: protective response or lethal event in post-mitotic neurons?. Biochim. Biophys. Acta 2007, 1772:457-466.
29. Ye J., et al. ER stress induces cleavage of membrane-bound ATF6 by the same proteases that process SREBPs. Mol. Cell 2000, 6:1355-1364.