Autophagy activators rescue and alleviate pathogenesis of a mouse model with proteinopathies of the TAR DNA-binding protein 43

Proceedings of the National Academy of Sciences of the United States of America

Volumn 109, Issue 37, 2012, Pages 15024-15029

  Wang, I Fang ^a,b,c   Guo, Bo Shen ^b   Liu, Yu Chih ^c   Wu, Cheng Chun ^c   Yang, Chun Hung ^b   Tsai, Kuen Jer ^c   Shen, Che Kun James ^a,b  

^a NATIONAL DEFENSE MEDICAL CENTER   (Taiwan)

^b INSTITUTE OF MOLECULAR BIOLOGY   (Taiwan)

^c NATIONAL CHENG KUNG UNIVERSITY   (Taiwan)

Author keywords

Neuronal apoptosis; Protein aggregation

Indexed keywords

CARBAMAZEPINE; CASPASE 3; PROTEIN P62; RAPAMYCIN; SPERMIDINE; TAMOXIFEN; TAR DNA BINDING PROTEIN;

ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ARTICLE; AUTOPHAGY; CELL SURVIVAL; CONTROLLED STUDY; DEGENERATIVE DISEASE; FEMALE; FOREBRAIN; LEARNING DISORDER; MEMORY DISORDER; MOTOR PERFORMANCE; MOUSE; NERVE CELL; NONHUMAN; PRIORITY JOURNAL; PROTEIN METABOLISM; TDP 43 PROTEINOPATHY;

ANALYSIS OF VARIANCE; ANIMALS; AUTOPHAGY; BLOTTING, WESTERN; CARBAMAZEPINE; CASPASE 3; FLUOROMETRY; FRONTOTEMPORAL DEMENTIA; IN SITU NICK-END LABELING; MAZE LEARNING; MEMORY DISORDERS; MICE; PSYCHOMOTOR PERFORMANCE; ROTAROD PERFORMANCE TEST; SIROLIMUS; SPERMIDINE; TAMOXIFEN; TDP-43 PROTEINOPATHIES; TOR SERINE-THREONINE KINASES;

ANIMALIA; MAMMALIA; MUS;

EID: 84866289381     PISSN: 00278424     EISSN: 10916490     Source Type: Journal    
DOI: 10.1073/pnas.1206362109     Document Type: Article

References (37)

1. Wang HY, Wang IF, Bose J, Shen CK (2004) Structural diversity and functional implications of the eukaryotic TDP gene family. Genomics 83:130-139.
2. Kuo PH, Doudeva LG, Wang YT, Shen CK, Yuan HS (2009) Structural insights into TDP-43 in nucleic-acid binding and domain interactions. Nucleic Acids Res 37:1799-1808.
3. Buratti E, Baralle FE (2008) Multiple roles of TDP-43 in gene expression, splicing regulation, and human disease. Front Biosci 13:867-878.
4. Wang IF, Wu LS, Shen CK (2008) TDP-43: An emerging new player in neurodegenerative diseases. Trends Mol Med 14:479-485.
5. Lagier-Tourenne C, Polymenidou M, Cleveland DW (2010) TDP-43 and FUS/TLS: Emerging roles in RNA processing and neurodegeneration. Hum Mol Genet 19(R1):R46-R64.
6. Polymenidou M, et al. (2011) Long pre-mRNA depletion and RNA missplicing contribute to neuronal vulnerability from loss of TDP-43. Nat Neurosci 14:459-468.
7. Chen-Plotkin AS, Lee VM, Trojanowski JQ (2010) TAR DNA-binding protein 43 in neurodegenerative disease. Nat Rev Neurol 6:211-220.
8. Arai T, et al. (2006) TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Biochem Biophys Res Commun 351:602-611.
9. Neumann M, et al. (2006) Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science 314:130-133.
10. Neumann M, et al. (2009) Phosphorylation of S409/410 of TDP-43 is a consistent feature in all sporadic and familial forms of TDP-43 proteinopathies. Acta Neuropathol 117:137-149.
11. Da Cruz S, Cleveland DW (2011) Understanding the role of TDP-43 and FUS/TLS in ALS and beyond. Curr Opin Neurobiol 21:904-919.
12. Logroscino G, et al.; EURALS (2008) Descriptive epidemiology of amyotrophic lateral sclerosis: New evidence and unsolved issues. J Neurol Neurosurg Psychiatry 79:6-11.
13. Kwong LK, Neumann M, Sampathu DM, Lee VM, Trojanowski JQ (2007) TDP-43 proteinopathy: The neuropathology underlying major forms of sporadic and familial frontotemporal lobar degeneration and motor neuron disease. Acta Neuropathol 114:63-70.
14. Mizushima N, Klionsky DJ (2007) Protein turnover via autophagy: Implications for metabolism. Annu Rev Nutr 27:19-40.
15. Kundu M, Thompson CB (2008) Autophagy: Basic principles and relevance to disease. Annu Rev Pathol 3:427-455.
16. Weidberg H, Shvets E, Elazar Z (2011) Biogenesis and cargo selectivity of autophagosomes. Annu Rev Biochem 80:125-156.
17. Díaz-Troya S, Pérez-Pérez ME, Florencio FJ, Crespo JL (2008) The role of TOR in autophagy regulation from yeast to plants and mammals. Autophagy 4:851-865.
18. Kim SH, et al. (2009) Potentiation of amyotrophic lateral sclerosis (ALS)-associated TDP-43 aggregation by the proteasome-targeting factor, ubiquilin 1. J Biol Chem 284:8083-8092.
19. Brady OA, Meng P, Zheng Y, Mao Y, Hu F (2011) Regulation of TDP-43 aggregation by phosphorylation and p62/SQSTM1. J Neurochem 116:248-259.
20. Bose JK, Huang CC, Shen CK (2011) Regulation of autophagy by neuropathological protein TDP-43. J Biol Chem 286:44441-44448.
21. Ravikumar B, et al. (2004) Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease. Nat Genet 36:585-595.
22. Caccamo A, Majumder S, Richardson A, Strong R, Oddo S (2010) Molecular interplay between mammalian target of rapamycin (mTOR), amyloid-beta, and Tau: Effects on cognitive impairments. J Biol Chem 285:13107-13120.
23. Spilman P, et al. (2010) Inhibition of mTOR by rapamycin abolishes cognitive deficits and reduces amyloid-beta levels in a mouse model of Alzheimer's disease. PLoS ONE 5:e9979.
24. Crews L, et al. (2010) Selective molecular alterations in the autophagy pathway in patients with Lewy body disease and in models of alpha- synucleinopathy. PLoS ONE 5:e9313.
25. Berger Z, et al. (2006) Rapamycin alleviates toxicity of different aggregate-prone proteins. Hum Mol Genet 15:433-442.
26. Bové J, Martínez-Vicente M, Vila M (2011) Fighting neurodegeneration with rapamycin: Mechanistic insights. Nat Rev Neurosci 12:437-452.
27. Ling DJ, Song HJ, Garza D, Neufeld TP, Salvaterra PM (2009) Abeta42-induced neurodegeneration via an age-dependent autophagic-lysosomal injury in Drosophila. PLoS ONE 4:e4201.
28. Zhang X, et al. (2011) Rapamycin treatment augments motor neuron degeneration in SOD1(G93A) mouse model of amyotrophic lateral sclerosis. Autophagy 7:412-425.
29. Tsai KJ, et al. (2010) Elevated expression of TDP-43 in the forebrain of mice is sufficient to cause neurological and pathological phenotypes mimicking FTLD-U. J Exp Med 207:1661-1673.
30. Tsai KJ, Tsai YC, Shen CK (2007) G-CSF rescues the memory impairment of animal models of Alzheimer's disease. J Exp Med 204:1273-1280.
31. Pankiv S, et al. (2007) p62/SQSTM1 binds directly to Atg8/LC3 to facilitate degradation of ubiquitinated protein aggregates by autophagy. J Biol Chem 282:24131-24145.
32. Webb JL, Ravikumar B, Atkins J, Skepper JN, Rubinsztein DC (2003) Alpha-Synuclein is degraded by both autophagy and the proteasome. J Biol Chem 278:25009-25013.
33. Heiseke A, Aguib Y, Riemer C, Baier M, Schätzl HM (2009) Lithium induces clearance of protease resistant prion protein in prion-infected cells by induction of autophagy. J Neurochem 109:25-34.
34. Zhang SQ, et al. (2010) Rapamycin promotes beta-amyloid production via ADAM-10 inhibition. Biochem Biophys Res Commun 398:337-341.
35. Caccamo A, et al. (2009) Rapamycin rescues TDP-43 mislocalization and the associated low molecular mass neurofilament instability. J Biol Chem 284:27416-27424.
36. Pikkarainen M, Hartikainen P, Alafuzoff I (2008) Neuropathologic features of frontotemporal lobar degeneration with ubiquitin-positive inclusions visualized with ubiquitin-binding protein p62 immunohistochemistry. J Neuropathol Exp Neurol 67:280-298.
37. Bjørkøy G, et al. (2005) p62/SQSTM1 forms protein aggregates degraded by autophagy and has a protective effect on huntingtin-induced cell death. J Cell Biol 171:603-614.