title: Protein quality control system in neurodegeneration: a healing company hard to beat but failure is fatal.
abstract: A common feature in most neurodegenerative diseases and aging is the progressive accumulation of damaged proteins. Proteins are essential for all crucial biological functions. Under some notorious conditions, proteins loss their three dimensional native conformations and are converted into disordered aggregated structures. Such changes rise into pathological conditions and eventually cause serious protein conformation disorders. Protein aggregation and inclusion bodies formation mediated multifactorial proteotoxic stress has been reported in the progression of Parkinson's disease (PD), Huntington's disease (HD), Alzheimer's disease (AD), amyotrophic lateral sclerosis (ALS) and Prion disease. Ongoing studies have been remarkably informative in providing a systematic outlook for better understanding the concept and fundamentals of protein misfolding and aggregations. However, the precise role of protein quality control system and precursors of this mechanism remains elusive. In this review, we highlight recent insights and discuss emerging cytoprotective strategies of cellular protein quality control system implicated in protein deposition diseases. Our current review provides a clear, understandable framework of protein quality control system that may offer the more suitable therapeutic strategies for protein-associated diseases.
authors: Cellular and Molecular Neurobiology Laboratory, Indian Institute of Technology, Jodhpur, Rajasthan, 342011, India.
authors: Deepak
authors: D
authors: Chhangani
authors: Amit
authors: A
authors: Mishra
mesh_terms: D000818:Animals; D006801:Humans; D009410:Nerve Degeneration / Q000473:pathology*; D009416:Nerve Regeneration; D009419:Nerve Tissue Proteins / Q000378:metabolism*; D017510:Protein Folding; D059748:Proteolysis; D013312:Stress, Physiological
chemical_list: D009419:Nerve Tissue Proteins
references: Cell Mol Life Sci. 1999 Aug 15;55(10):1230-54
references: 10487205
references: Am J Physiol Heart Circ Physiol. 2008 Dec;295(6):H2541-50
references: 18978187
references: Nat Cell Biol. 2010 Sep;12(9):836-41
references: 20811356
references: Nat Rev Genet. 2007 Sep;8(9):711-23
references: 17680008
references: FEBS Lett. 2001 Nov 30;509(1):31-5
references: 11734201
references: Essays Biochem. 2005;41:157-71
references: 16250904
references: Nat Med. 2004 Oct;10(10):1055-63
references: 15459709
references: Biol Chem. 2007 Aug;388(8):797-804
references: 17655498
references: J Biol Chem. 2011 Sep 9;286(36):31328-36
references: 21768107
references: Proc Natl Acad Sci U S A. 2012 Jul 31;109(31):12479-84
references: 22802614
references: J Neurosci. 2011 Oct 12;31(41):14508-20
references: 21994367
references: PLoS One. 2010 Apr 01;5(4):e9979
references: 20376313
references: Science. 1998 Aug 28;281(5381):1322-6
references: 9735050
references: Mol Cell. 2009 Feb 27;33(4):505-16
references: 19250911
references: J Biol Chem. 2011 Jan 28;286(4):2785-94
references: 21098017
references: Nat Genet. 1999 Dec;23(4):425-8
references: 10581028
references: Nat Cell Biol. 2005 Aug;7(8):766-72
references: 16056268
references: Biofactors. 2003;17(1-4):249-57
references: 12897446
references: Neuron. 2005 Jul 21;47(2):191-199
references: 16039562
references: Hum Mol Genet. 2009 Nov 15;18(22):4268-81
references: 19661182
references: Biol Chem. 2010 May;391(5):481-9
references: 20302520
references: Curr Opin Cell Biol. 2004 Dec;16(6):653-62
references: 15530777
references: Nature. 2006 Mar 23;440(7083):551-5
references: 16554822
references: Mol Biol Cell. 2005 Apr;16(4):1777-87
references: 15703212
references: Biochem Biophys Res Commun. 2003 Aug 1;307(3):689-95
references: 12893279
references: J Biol Chem. 2006 Oct 6;281(40):30299-304
references: 16901900
references: Cell. 2012 Nov 21;151(5):1042-54
references: 23178123
references: FEBS Lett. 1996 Aug 26;392(2):129-36
references: 8772190
references: J Biol Chem. 2006 Oct 6;281(40):30152-65
references: 16847061
references: Science. 1997 Dec 19;278(5346):2123-6
references: 9405348
references: Mol Cell. 1998 Apr;1(5):639-48
references: 9660948
references: Mov Disord. 2012 Sep 1;27(10):1218
references: 23136660
references: Comb Chem High Throughput Screen. 2004 Nov;7(7):689-97
references: 15578931
references: Nature. 2009 Jul 16;460(7253):392-5
references: 19587680
references: Annu Rev Cell Dev Biol. 2011;27:107-32
references: 21801009
references: Mol Cell. 2010 Oct 22;40(2):228-37
references: 20965418
references: J Neurochem. 2009 Sep;110(6):1955-64
references: 19645749
references: Exp Gerontol. 2001 Sep;36(9):1539-50
references: 11525876
references: Adv Exp Med Biol. 2007;594:89-99
references: 17205678
references: Science. 2002 Mar 8;295(5561):1852-8
references: 11884745
references: Traffic. 2005 Jun;6(6):429-41
references: 15882441
references: PLoS One. 2011 Feb 16;6(2):e14695
references: 21358815
references: J Biol Chem. 2009 Apr 17;284(16):10537-45
references: 19233847
references: FASEB J. 1992 May;6(8):2545-52
references: 1592207
references: Mol Neurobiol. 2012 Jun;45(3):571-85
references: 22610945
references: FASEB J. 2009 Oct;23(10):3383-92
references: 19509306
references: Hum Mol Genet. 2008 Feb 15;17(4):469-77
references: 17989065
references: Eur J Pharmacol. 2002 Jan 18;435(1):79-83
references: 11790381
references: Mol Cell. 2009 Apr 24;34(2):212-22
references: 19394298
references: Cell Struct Funct. 2002 Dec;27(6):421-9
references: 12576635
references: J Neurosci. 2003 Oct 1;23(26):8872-80
references: 14523089
references: Science. 1996 Mar 15;271(5255):1519-26
references: 8599107
references: Neuron. 1997 Dec;19(6):1147-50
references: 9427237
references: Curr Opin Cell Biol. 2011 Apr;23(2):190-7
references: 21087849
references: Nature. 2007 Jun 14;447(7146):859-63
references: 17568747
references: Nat Struct Biol. 2002 Oct;9(10):716-8
references: 12352951
references: J Biochem. 2002 Apr;131(4):593-603
references: 11926998
references: Protein Eng. 2002 Feb;15(2):153-60
references: 11917152
references: Cell. 2006 May 5;125(3):443-51
references: 16678092
references: Acta Pharmacol Sin. 2009 Jul;30(7):1046-52
references: 19575007
references: J Cell Biol. 2010 Apr 5;189(1):57-68
references: 20368618
references: Methods Mol Biol. 2010;648:3-23
references: 20700702
references: Cell. 2011 Nov 11;147(4):728-41
references: 22078875
references: Nat Struct Mol Biol. 2011 Mar;18(3):345-51
references: 21278757
references: Nat Rev Mol Cell Biol. 2009 Jul;10(7):458-67
references: 19491929
references: Brain Res Bull. 1998;45(2):163-6
references: 9443834
references: J Cell Sci. 2012 Mar 1;125(Pt 5):1141-51
references: 22302993
references: Cell Stress Chaperones. 2009 Nov;14(6):579-89
references: 19291423
references: J Biol Chem. 2002 Sep 27;277(39):36793-8
references: 12145308
references: Autophagy. 2011 Jul;7(7):795-8
references: 21681022
references: Nature. 2006 Jun 15;441(7095):880-4
references: 16625205
references: Biochem J. 2012 Jun 15;444(3):611-7
references: 22497224
references: Int J Biochem Cell Biol. 2003 May;35(5):572-8
references: 12672450
references: PLoS One. 2008 Aug 06;3(8):e2864
references: 18682744
references: Cell Biochem Funct. 2010 Jan;28(1):1-14
references: 19830685
references: J Biol Chem. 1988 May 15;263(14):6797-805
references: 3360807
references: Neurobiol Aging. 2013 Apr;34(4):1310.e11-23
references: 23040663
references: Oncogene. 1999 Nov 1;18(45):6163-71
references: 10557108
references: Science. 2001 May 25;292(5521):1552-5
references: 11375494
references: J Exp Med. 2004 Aug 16;200(4):425-35
references: 15314073
references: Nature. 2008 Feb 28;451(7182):1069-75
references: 18305538
references: J Neurochem. 2004 Jul;90(1):231-44
references: 15198682
references: Proc Natl Acad Sci U S A. 2005 Sep 13;102(37):13135-40
references: 16141322
references: Proc Natl Acad Sci U S A. 2004 Mar 23;101(12):4100-5
references: 15016915
references: Science. 1987 Jul 3;237(4810):77-80
references: 3299701
references: Nature. 2006 Jun 15;441(7095):885-9
references: 16625204
references: Annu Rev Biochem. 2001;70:603-47
references: 11395418
references: Biochim Biophys Acta. 2008 Dec;1782(12):691-9
references: 18930136
references: Antioxid Redox Signal. 2007 Dec;9(12):2277-93
references: 17979528
references: Mol Cell. 2000 Nov;6(5):1099-108
references: 11106749
references: J Bacteriol. 1980 Sep;143(3):1384-94
references: 6997270
references: Asian J Androl. 2009 Jan;11(1):119-26
references: 19050678
references: Nature. 2008 Aug 28;454(7208):1088-95
references: 18756251
references: Curr Biol. 2010 Jan 26;20(2):143-8
references: 20060297
references: Cell. 2006 Sep 8;126(5):995-1004
references: 16959577
references: Int J Cell Biol. 2010;2010:214074
references: 20182529
references: Cochrane Database Syst Rev. 2007 Jan 24;(1):CD001447
references: 17253460
references: Nature. 2003 Dec 18;426(6968):884-90
references: 14685248
references: Nat Rev Drug Discov. 2006 Jul;5(7):596-613
references: 16816840
references: Physiol Rev. 2002 Apr;82(2):373-428
references: 11917093
references: Autophagy. 2007 Jul-Aug;3(4):295-9
references: 17404494
references: FEBS J. 2007 Apr;274(7):1715-24
references: 17331188
references: Nature. 1994 Jul 14;370(6485):111-7
references: 8022479
references: J Clin Invest. 2008 Jun;118(6):2190-9
references: 18497889
references: Science. 2004 Oct 29;306(5697):865-9
references: 15514157
references: EMBO Rep. 2011 Feb;12(2):149-56
references: 21252941
references: Nature. 2010 Jan 28;463(7280):549-53
references: 20111001
references: FEBS Lett. 1997 Jun 30;410(2-3):493-8
references: 9237690
references: Curr Biol. 2008 Nov 11;18(21):R1019-21
references: 19000801
references: Science. 1993 Mar 5;259(5100):1409-10
references: 8451637
references: Shock. 2010 Dec;34(6):643-8
references: 20823696
references: Am J Pathol. 2007 Aug;171(2):513-24
references: 17620365
references: Nat Med. 2004 Feb;10(2):148-54
references: 14730359
references: Neuron. 2002 Jul 3;35(1):9-12
references: 12123602
references: J Cell Biol. 2005 Nov 21;171(4):603-14
references: 16286508
references: Nature. 1993 Jul 15;364(6434):252-5
references: 8321322
references: J Biol Chem. 2007 Aug 17;282(33):24131-45
references: 17580304
references: Proc Natl Acad Sci U S A. 2001 Jul 17;98(15):8554-9
references: 11438690
references: Annu Rev Biochem. 1999;68:1015-68
references: 10872471
references: Mol Cell. 2005 Feb 4;17(3):351-65
references: 15694337
references: EMBO J. 2003 Oct 15;22(20):5446-58
references: 14532117
references: Genes Dev. 2008 Jun 1;22(11):1451-64
references: 18519638
references: Cell. 2012 Dec 21;151(7):1501-1512
references: 23219485
references: J Biol Chem. 2006 Dec 1;281(48):37017-24
references: 17028181
references: Mol Cell. 2000 Sep;6(3):751-6
references: 11030355
references: Science. 1996 Jul 26;273(5274):501-3
references: 8662539
references: Neurobiol Dis. 2012 Jun;46(3):732-44
references: 22426402
references: Science. 1989 Oct 20;246(4928):382-5
references: 2799391
references: J Biol Chem. 2003 Jun 13;278(24):22044-55
references: 12676955
references: EMBO J. 2003 Mar 3;22(5):1180-7
references: 12606582
references: Brain. 2010 Jan;133(Pt 1):93-104
references: 20007218
references: Trends Neurosci. 2003 Aug;26(8):407-10
references: 12900170
references: Methods Enzymol. 2005;399:833-47
references: 16338398
references: Nature. 2002 Apr 4;416(6880):507-11
references: 11932737
references: Cell Mol Life Sci. 2008 Feb;65(4):656-66
references: 18193166
references: Cell. 1989 Dec 1;59(5):797-806
references: 2590940
references: Genes Dev. 1998 Dec 15;12(24):3788-96
references: 9869631
references: Neurobiol Dis. 2002 Jul;10(2):88-99
references: 12127147
references: Mol Neurobiol. 2013 Feb;47(1):302-12
references: 23001884
references: Neurobiol Dis. 2009 Oct;36(1):26-34
references: 19591933
references: Autophagy. 2008 Jul;4(5):563-6
references: 18326941
references: Eur J Biochem. 2002 Jun;269(12):3076-85
references: 12071973
references: Mol Biol Cell. 2009 Nov;20(21):4524-30
references: 19741096
references: Neuroreport. 1998 Jun 22;9(9):2029-33
references: 9674588
references: Nat Neurosci. 2010 Jul;13(7):805-11
references: 20581817
references: J Mol Med (Berl). 2003 Nov;81(11):678-99
references: 12942175
references: EMBO Mol Med. 2013 Feb;5(2):221-34
references: 23255347
references: Autophagy. 2011 Jul;7(7):673-82
references: 21646866
references: Cell Stress Chaperones. 2011 Jan;16(1):15-31
references: 20694844
references: Annu Rev Biochem. 2006;75:333-66
references: 16756495
references: Exp Neurol. 2012 Nov;238(1):22-8
references: 21095248
references: Biochim Biophys Acta. 2012 Nov;1822(11):1741-51
references: 22867901
references: J Pharmacol Exp Ther. 2010 May;333(2):454-64
references: 20179157
references: Cell Death Differ. 2006 Mar;13(3):385-92
references: 16397584
references: Mol Cell. 2009 May 15;34(3):259-69
references: 19450525
references: Prog Neurobiol. 2012 Feb;96(2):190-207
references: 22270043
references: Mol Vis. 2006 Dec 05;12:1526-35
references: 17167411
references: Proc Natl Acad Sci U S A. 2006 Apr 11;103(15):6025-30
references: 16595634
references: Mol Cell. 2002 Jul;10(1):55-67
references: 12150907
references: Genes Dev. 2008 Jun 1;22(11):1427-38
references: 18519635
references: J Biol Chem. 2008 Mar 21;283(12):7648-56
references: 18201976
references: Biochim Biophys Acta. 2013 Jan;1830(1):2105-17
references: 23041501
references: J Biol Chem. 2005 Mar 25;280(12):11635-40
references: 15664989
references: J Biol Chem. 2013 Feb 1;288(5):3641-54
references: 23235148
references: Nat Med. 2004 Jul;10 Suppl:S10-7
references: 15272267
references: J Neurochem. 1999 Feb;72(2):693-9
references: 9930742
references: J Biol Chem. 2000 Nov 17;275(46):35661-4
references: 10973942
references: Cold Spring Harb Perspect Biol. 2011 Aug 01;3(8):a004374
references: 21746797
references: Nature. 1997 Oct 30;389(6654):971-4
references: 9353120
references: EMBO Rep. 2001 Dec;2(12):1133-8
references: 11743028
references: J Biol Chem. 1996 Jan 12;271(2):635-8
references: 8557666
references: Nature. 2003 Dec 18;426(6968):895-9
references: 14685250
references: Mol Genet Metab. 2012 Nov;107(3):490-5
references: 23041259
references: Trends Neurosci. 2006 Sep;29(9):528-35
references: 16859759
references: J Biol Chem. 2004 Mar 19;279(12):11327-35
references: 14684739
references: Mol Cell Biol. 2006 Dec;26(24):9220-31
references: 17030611
references: Cold Spring Harb Perspect Biol. 2010 Dec;2(12):a006734
references: 21068151
references: FEBS Lett. 2009 Feb 18;583(4):621-6
references: 19166840
references: Cell. 2009 Jun 12;137(6):1136-47
references: 19524515
references: J Neurosci. 2008 Aug 13;28(33):8189-98
references: 18701681
references: Cell Death Differ. 2008 Jan;15(1):171-82
references: 17917680
references: PLoS One. 2012;7(10):e46834
references: 23071649
references: J Neurosci. 2003 Apr 15;23(8):3316-24
references: 12716939
references: Curr Mol Med. 2006 Feb;6(1):87-97
references: 16472116
references: J Biochem. 2009 Nov;146(5):609-16
references: 19737776
references: Eur J Pharmacol. 2002 Jan 18;435(1):73-7
references: 11790380
references: Nat Med. 1997 Oct;3(10):1150-4
references: 9334730
references: Nature. 2003 Dec 18;426(6968):900-4
references: 14685251
references: Mol Cell Biol. 1996 Aug;16(8):4231-9
references: 8754823
references: Hum Mol Genet. 2009 Feb 15;18(4):688-700
references: 19036738
references: J Biol Chem. 2001 Nov 23;276(47):43723-33
references: 11559696
references: Nature. 1988 Apr 28;332(6167):805-10
references: 3282179
references: Neurodegener Dis. 2004;1(2-3):71-87
references: 16908979