Selective Neuronal Vulnerability in Neurodegenerative Diseases: From Stressor Thresholds to Degeneration

Neuron

Volumn 71, Issue 1, 2011, Pages 35-48

  Saxena, Smita ^a,b   Caroni, Pico ^a  

^a FRIEDRICH MIESCHER INSTITUTE FOR BIOMEDICAL RESEARCH   (Switzerland)

^b UNIVERSITY OF BERN   (Switzerland)

Author keywords

[No Author keywords available]

Indexed keywords

BETA SECRETASE 1; PEROXISOME PROLIFERATOR ACTIVATED RECEPTOR GAMMA COACTIVATOR 1ALPHA; SOMATOMEDIN C; TAU PROTEIN;

AGE; ALZHEIMER DISEASE; AMYOTROPHIC LATERAL SCLEROSIS; BIOACCUMULATION; BIOLOGICAL MODEL; CELL STRESS; CELL SUBPOPULATION; COMORBIDITY; DISEASE ACTIVITY; DISEASE COURSE; DISEASE PREDISPOSITION; DOPAMINERGIC NERVE CELL; ENVIRONMENTAL STRESS; FAMILIAL DISEASE; GENETIC PREDISPOSITION; GLIA CELL; HOMEOSTASIS; HUMAN; HUNTINGTON CHOREA; IMMUNOPATHOLOGY; MOTONEURON; NERVE CELL; NERVE CELL EXCITABILITY; NERVE CELL LESION; NONHUMAN; PARKINSON DISEASE; PATHOPHYSIOLOGY; PRIORITY JOURNAL; PROTEIN DEFECT; PROTEIN EXPRESSION; PROTEIN FOLDING; PROTEIN FUNCTION; REVIEW; STRESSOR THRESHOLD MODEL; TAUOPATHY; VASCULAR DISEASE;

ANIMALS; HUMANS; MODELS, BIOLOGICAL; NERVE DEGENERATION; NEURODEGENERATIVE DISEASES; PROTEOSTASIS DEFICIENCIES; SIGNAL TRANSDUCTION; STRESS, PHYSIOLOGICAL;

EID: 79960167259     PISSN: 08966273     EISSN: 10974199     Source Type: Journal    
DOI: 10.1016/j.neuron.2011.06.031     Document Type: Review

References (168)

1. Aguzzi A., Calella A.M. Prions: protein aggregation and infectious diseases. Physiol. Rev. 2009, 89:1105-1152.
2. Appel S.H., Beers D.R., Henkel J.S. T cell-microglial dialogue in Parkinson's disease and amyotrophic lateral sclerosis: are we listening?. Trends Immunol. 2010, 31:7-17.
3. Arrasate M., Mitra S., Schweitzer E.S., Segal M.R., Finkbeiner S. Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death. Nature 2004, 431:805-810.
4. Atkin J.D., Farg M.A., Walker A.K., McLean C., Tomas D., Horne M.K. Endoplasmic reticulum stress and induction of the unfolded protein response in human sporadic amyotrophic lateral sclerosis. Neurobiol. Dis. 2008, 30:400-407.
5. Autere J., Moilanen J.S., Finnilä S., Soininen H., Mannermaa A., Hartikainen P., Hallikainen M., Majamaa K. Mitochondrial DNA polymorphisms as risk factors for Parkinson's disease and Parkinson's disease dementia. Hum. Genet. 2004, 115:29-35.
6. Balch W.E., Morimoto R.I., Dillin A., Kelly J.W. Adapting proteostasis for disease intervention. Science 2008, 319:916-919.
7. Ballatore C., Lee V.M., Trojanowski J.Q. Tau-mediated neurodegeneration in Alzheimer's disease and related disorders. Nat. Rev. Neurosci. 2007, 8:663-672.
8. Bamford N.S., Zhang H., Schmitz Y., Wu N.P., Cepeda C., Levine M.S., Schmauss C., Zakharenko S.S., Zablow L., Sulzer D. Heterosynaptic dopamine neurotransmission selects sets of corticostriatal terminals. Neuron 2004, 42:653-663.
9. Banerjee R., Starkov A.A., Beal M.F., Thomas B. Mitochondrial dysfunction in the limelight of Parkinson's disease pathogenesis. Biochim. Biophys. Acta 2009, 1792:651-663.
10. Bell R.D., Deane R., Chow N., Long X., Sagare A., Singh I., Streb J.W., Guo H., Rubio A., Van Nostrand W., et al. SRF and myocardin regulate LRP-mediated amyloid-beta clearance in brain vascular cells. Nat. Cell Biol. 2009, 11:143-153.
11. Bernales S., Papa F.R., Walter P. Intracellular signaling by the unfolded protein response. Annu. Rev. Cell Dev. Biol. 2006, 22:487-508.
12. Bezprozvanny I., Mattson M.P. Neuronal calcium mishandling and the pathogenesis of Alzheimer's disease. Trends Neurosci. 2008, 31:454-463.
13. Biskup S., Moore D.J. Detrimental deletions: mitochondria, aging and Parkinson's disease. Bioessays 2006, 28:963-967.
14. Björkqvist M., Wild E.J., Thiele J., Silvestroni A., Andre R., Lahiri N., Raibon E., Lee R.V., Benn C.L., Soulet D., et al. A novel pathogenic pathway of immune activation detectable before clinical onset in Huntington's disease. J. Exp. Med. 2008, 205:1869-1877.
15. Björkqvist M., Wild E.J., Tabrizi S.J. Harnessing immune alterations in neurodegenerative diseases. Neuron 2009, 64:21-24.
16. Blasko I., Beer R., Bigl M., Apelt J., Franz G., Rudzki D., Ransmayr G., Kampfl A., Schliebs R. Experimental traumatic brain injury in rats stimulates the expression, production and activity of Alzheimer's disease beta-secretase (BACE-1). J. Neural Transm. 2004, 111:523-536.
17. Bogaerts V., Theuns J., van Broeckhoven C. Genetic findings in Parkinson's disease and translation into treatment: a leading role for mitochondria?. Genes Brain Behav. 2008, 7:129-151.
18. Boillée S., Yamanaka K., Lobsiger C.S., Copeland N.G., Jenkins N.A., Kassiotis G., Kollias G., Cleveland D.W. Onset and progression in inherited ALS determined by motor neurons and microglia. Science 2006, 312:1389-1392.
19. Boillée S., Vande Velde C., Cleveland D.W. ALS: a disease of motor neurons and their nonneuronal neighbors. Neuron 2006, 52:39-59.
20. Bolmont T., Clavaguera F., Meyer-Luehmann M., Herzig M.C., Radde R., Staufenbiel M., Lewis J., Hutton M., Tolnay M., Jucker M. Induction of tau pathology by intracerebral infusion of amyloid-beta -containing brain extract and by amyloid-beta deposition in APP x Tau transgenic mice. Am. J. Pathol. 2007, 171:2012-2020.
21. Bories C., Amendola J., Lamotte d'Incamps B., Durand J. Early electrophysiological abnormalities in lumbar motoneurons in a transgenic mouse model of amyotrophic lateral sclerosis. Eur. J. Neurosci. 2007, 25:451-459.
22. Brignull H.R., Moore F.E., Tang S.J., Morimoto R.I. Polyglutamine proteins at the pathogenic threshold display neuron-specific aggregation in a pan-neuronal Caenorhabditis elegans model. J. Neurosci. 2006, 26:7597-7606.
23. Browne S.E., Yang L., DiMauro J.P., Fuller S.W., Licata S.C., Beal M.F. Bioenergetic abnormalities in discrete cerebral motor pathways presage spinal cord pathology in the G93A SOD1 mouse model of ALS. Neurobiol. Dis. 2006, 22:599-610.
24. Brundin P., Li J.Y., Holton J.L., Lindvall O., Revesz T. Research in motion: the enigma of Parkinson's disease pathology spread. Nat. Rev. Neurosci. 2008, 9:741-745.
25. Burré J., Sharma M., Tsetsenis T., Buchman V., Etherton M.R., Südhof T.C. Alpha-synuclein promotes SNARE-complex assembly in vivo and in vitro. Science 2010, 329:1663-1667.
26. Busche M.A., Eichhoff G., Adelsberger H., Abramowski D., Wiederhold K.H., Haass C., Staufenbiel M., Konnerth A., Garaschuk O. Clusters of hyperactive neurons near amyloid plaques in a mouse model of Alzheimer's disease. Science 2008, 321:1686-1689.
27. Cepeda C., Wu N., André V.M., Cummings D.M., Levine M.S. The corticostriatal pathway in Huntington's disease. Prog. Neurobiol. 2007, 81:253-271.
28. Chan C.S., Guzman J.N., Ilijic E., Mercer J.N., Rick C., Tkatch T., Meredith G.E., Surmeier D.J. 'Rejuvenation' protects neurons in mouse models of Parkinson's disease. Nature 2007, 447:1081-1086.
29. Chaturvedi R.K., Adhihetty P., Shukla S., Hennessy T., Calingasan N., Yang L., Starkov A., Kiaei M., Cannella M., Sassone J., et al. Impaired PGC-1α function in muscle in Huntington's disease. Hum. Mol. Genet. 2009, 18:3048-3065.
30. Cheung K.H., Shineman D., Müller M., Cárdenas C., Mei L., Yang J., Tomita T., Iwatsubo T., Lee V.M., Foskett J.K. Mechanism of Ca2+ disruption in Alzheimer's disease by presenilin regulation of InsP3 receptor channel gating. Neuron 2008, 58:871-883.
31. Cho D.H., Nakamura T., Fang J., Cieplak P., Godzik A., Gu Z., Lipton S.A. S-nitrosylation of Drp1 mediates beta-amyloid-related mitochondrial fission and neuronal injury. Science 2009, 324:102-105.
32. Choi W.S., Kruse S.E., Palmiter R.D., Xia Z. Mitochondrial complex I inhibition is not required for dopaminergic neuron death induced by rotenone, MPP+, or paraquat. Proc. Natl. Acad. Sci. USA 2008, 105:15136-15141.
33. Clavaguera F., Bolmont T., Crowther R.A., Abramowski D., Frank S., Probst A., Fraser G., Stalder A.K., Beibel M., Staufenbiel M., et al. Transmission and spreading of tauopathy in transgenic mouse brain. Nat. Cell Biol. 2009, 11:909-913.
34. Clement A.M., Nguyen M.D., Roberts E.A., Garcia M.L., Boillée S., Rule M., McMahon A.P., Doucette W., Siwek D., Ferrante R.J., et al. Wild-type nonneuronal cells extend survival of SOD1 mutant motor neurons in ALS mice. Science 2003, 302:113-117.
35. Cohen E., Dillin A. The insulin paradox: aging, proteotoxicity and neurodegeneration. Nat. Rev. Neurosci. 2008, 9:759-767.
36. Cohen E., Paulsson J.F., Blinder P., Burstyn-Cohen T., Du D., Estepa G., Adame A., Pham H.M., Holzenberger M., Kelly J.W., et al. Reduced IGF-1 signaling delays age-associated proteotoxicity in mice. Cell 2009, 139:1157-1169.
37. Cole S.L., Vassar R. Linking vascular disorders and Alzheimer's disease: potential involvement of BACE1. Neurobiol. Aging 2009, 30:1535-1544.
38. Cui L., Jeong H., Borovecki F., Parkhurst C.N., Tanese N., Krainc D. Transcriptional repression of PGC-1alpha by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration. Cell 2006, 127:59-69.
39. Darios F., Muriel M.P., Khondiker M.E., Brice A., Ruberg M. Neurotoxic calcium transfer from endoplasmic reticulum to mitochondria is regulated by cyclin-dependent kinase 5-dependent phosphorylation of tau. J. Neurosci. 2005, 25:4159-4168.
40. David G., Nguyen K., Barrett E.F. Early vulnerability to ischemia/reperfusion injury in motor terminals innervating fast muscles of SOD1-G93A mice. Exp. Neurol. 2007, 204:411-420.
41. Decarli C. Vascular factors in dementia: an overview. J. Neurol. Sci. 2004, 226:19-23.
42. Desplats P., Lee H.J., Bae E.J., Patrick C., Rockenstein E., Crews L., Spencer B., Masliah E., Lee S.J. Inclusion formation and neuronal cell death through neuron-to-neuron transmission of alpha-synuclein. Proc. Natl. Acad. Sci. USA 2009, 106:13010-13015.
43. 2+ homeostasis, Abeta levels, and Alzheimer's disease risk. Cell 2008, 133:1149-1161.
44. Eisele Y.S., Obermüller U., Heilbronner G., Baumann F., Kaeser S.A., Wolburg H., Walker L.C., Staufenbiel M., Heikenwalder M., Jucker M. Peripherally applied Abeta-containing inoculates induce cerebral beta-amyloidosis. Science 2010, 330:980-982.
45. Ferri A., Cozzolino M., Crosio C., Nencini M., Casciati A., Gralla E.B., Rotilio G., Valentine J.S., Carrì M.T. Familial ALS-superoxide dismutases associate with mitochondria and shift their redox potentials. Proc. Natl. Acad. Sci. USA 2006, 103:13860-13865.
46. Finkbeiner S., Cuervo A.M., Morimoto R.I., Muchowski P.J. Disease-modifying pathways in neurodegeneration. J. Neurosci. 2006, 26:10349-10357.
47. Frey D., Schneider C., Xu L., Borg J., Spooren W., Caroni P. Early and selective loss of neuromuscular synapse subtypes with low sprouting competence in motoneuron diseases. J. Neurosci. 2000, 20:2534-2542.
48. Frost B., Diamond M.I. Prion-like mechanisms in neurodegenerative diseases. Nat. Rev. Neurosci. 2010, 11:155-159.
49. Frost B., Jacks R.L., Diamond M.I. Propagation of tau misfolding from the outside to the inside of a cell. J. Biol. Chem. 2009, 284:12845-12852.
50. Fukui H., Moraes C.T. The mitochondrial impairment, oxidative stress and neurodegeneration connection: reality or just an attractive hypothesis?. Trends Neurosci. 2008, 31:251-256.
51. Gan L., Mucke L. Paths of convergence: sirtuins in aging and neurodegeneration. Neuron 2008, 58:10-14.
52. Garcia S.M., Casanueva M.O., Silva M.C., Amaral M.D., Morimoto R.I. Neuronal signaling modulates protein homeostasis in Caenorhabditis elegans post-synaptic muscle cells. Genes Dev. 2007, 21:3006-3016.
53. Gidalevitz T., Ben-Zvi A., Ho K.H., Brignull H.R., Morimoto R.I. Progressive disruption of cellular protein folding in models of polyglutamine diseases. Science 2006, 311:1471-1474.
54. Gitcho M.A., Strider J., Carter D., Taylor-Reinwald L., Forman M.S., Goate A.M., Cairns N.J. VCP mutations causing frontotemporal lobar degeneration disrupt localization of TDP-43 and induce cell death. J. Biol. Chem. 2009, 284:12384-12398.
55. Glass C.K., Saijo K., Winner B., Marchetto M.C., Gage F.H. Mechanisms underlying inflammation in neurodegeneration. Cell 2010, 140:918-934.
56. Gleichmann M., Mattson M.P. Alzheimer's disease and neuronal network activity. Neuromol. Med. 2010, 12:44-47.
57. Golde T.E., Miller V.M. Proteinopathy-induced neuronal senescence: a hypothesis for brain failure in Alzheimer's and other neurodegenerative diseases. Alzheimers Res. Ther. 2009, 1:5-17.
58. Gowing G., Lalancette-Hébert M., Audet J.N., Dequen F., Julien J.P. Macrophage colony stimulating factor (M-CSF) exacerbates ALS disease in a mouse model through altered responses of microglia expressing mutant superoxide dismutase. Exp. Neurol. 2009, 220:267-275.
59. Grathwohl S.A., Kälin R.E., Bolmont T., Prokop S., Winkelmann G., Kaeser S.A., Odenthal J., Radde R., Eldh T., Gandy S., et al. Formation and maintenance of Alzheimer's disease beta-amyloid plaques in the absence of microglia. Nat. Neurosci. 2009, 12:1361-1363.
60. Gu M., Gash M.T., Cooper J.M., Wenning G.K., Daniel S.E., Quinn N.P., Marsden C.D., Schapira A.H. Mitochondrial respiratory chain function in multiple system atrophy. Mov. Disord. 1997, 12:418-422.
61. Gurney M.E., Pu H., Chiu A.Y., Dal Canto M.C., Polchow C.Y., Alexander D.D., Caliendo J., Hentati A., Kwon Y.W., Deng H.X., et al. Motor neuron degeneration in mice that express a human Cu, Zn superoxide dismutase mutation. Science 1994, 264:1772-1775.
62. Guzman J.N., Sanchez-Padilla J., Wokosin D., Kondapalli J., Ilijic E., Schumacker P.T., Surmeier D.J. Oxidant stress evoked by pacemaking in dopaminergic neurons is attenuated by DJ-1. Nature 2010, 468:696-700.
63. Haass C., Selkoe D.J. Soluble protein oligomers in neurodegeneration: lessons from the Alzheimer's amyloid beta-peptide. Nat. Rev. Mol. Cell Biol. 2007, 8:101-112.
64. Hara T., Nakamura K., Matsui M., Yamamoto A., Nakahara Y., Suzuki-Migishima R., Yokoyama M., Mishima K., Saito I., Okano H., Mizushima N. Suppression of basal autophagy in neural cells causes neurodegenerative disease in mice. Nature 2006, 441:885-889.
65. Harraz M.M., Marden J.J., Zhou W., Zhang Y., Williams A., Sharov V.S., Nelson K., Luo M., Paulson H., Schöneich C., Engelhardt J.F. SOD1 mutations disrupt redox-sensitive Rac regulation of NADPH oxidase in a familial ALS model. J. Clin. Invest. 2008, 118:659-670.
66. Hawkes C.H., Del Tredici K., Braak H. Parkinson's disease: a dual-hit hypothesis. Neuropathol. Appl. Neurobiol. 2007, 33:599-614.
67. Hedden T., Van Dijk K.R., Becker J.A., Mehta A., Sperling R.A., Johnson K.A., Buckner R.L. Disruption of functional connectivity in clinically normal older adults harboring amyloid burden. J. Neurosci. 2009, 29:12686-12694.
68. Hof P.R., Morrison J.H. The aging brain: morphomolecular senescence of cortical circuits. Trends Neurosci. 2004, 27:607-613.
69. Hotamisligil G.S. Endoplasmic reticulum stress and the inflammatory basis of metabolic disease. Cell 2010, 140:900-917.
70. Hsu A.L., Murphy C.T., Kenyon C. Regulation of aging and age-related disease by DAF-16 and heat-shock factor. Science 2003, 300:1142-1145.
71. Jellinger K.A. Lewy body-related alpha-synucleinopathy in the aged human brain. J. Neural Transm. 2004, 111:1219-1235.
72. Johnson J.O., Mandrioli J., Benatar M., Abramzon Y., Van Deerlin V.M., Trojanowski J.Q., Gibbs J.R., Brunetti M., Gronka S., Wuu J., et al. Exome sequencing reveals VCP mutations as a cause of familial ALS. Neuron 2010, 68:857-864. ITALSGEN Consortium.
73. Kanekura K., Suzuki H., Aiso S., Matsuoka M. ER stress and unfolded protein response in amyotrophic lateral sclerosis. Mol. Neurobiol. 2009, 39:81-89.
74. Kang J., Rivest S. MyD88-deficient bone marrow cells accelerate onset and reduce survival in a mouse model of amyotrophic lateral sclerosis. J. Cell Biol. 2007, 179:1219-1230.
75. Keeney P.M., Xie J., Capaldi R.A., Bennett J.P. Parkinson's disease brain mitochondrial complex I has oxidatively damaged subunits and is functionally impaired and misassembled. J. Neurosci. 2006, 26:5256-5264.
76. Kern A., Behl C. The unsolved relationship of brain aging and late-onset Alzheimer disease. Biochim. Biophys. Acta 2009, 1790:1124-1132.
77. Kieran D., Woods I., Villunger A., Strasser A., Prehn J.H. Deletion of the BH3-only protein puma protects motoneurons from ER stress-induced apoptosis and delays motoneuron loss in ALS mice. Proc. Natl. Acad. Sci. USA 2007, 104:20606-20611.
78. Kim D.Y., Carey B.W., Wang H., Ingano L.A., Binshtok A.M., Wertz M.H., Pettingell W.H., He P., Lee V.M., Woolf C.J., Kovacs D.M. BACE1 regulates voltage-gated sodium channels and neuronal activity. Nat. Cell Biol. 2007, 9:755-764.
79. Kimata Y., Kohno K. Endoplasmic reticulum stress-sensing mechanisms in yeast and mammalian cells. Curr. Opin. Cell Biol. 2011, 23:135-142.
80. Knott A.B., Perkins G., Schwarzenbacher R., Bossy-Wetzel E. Mitochondrial fragmentation in neurodegeneration. Nat. Rev. Neurosci. 2008, 9:505-518.
81. Kok E., Haikonen S., Luoto T., Huhtala H., Goebeler S., Haapasalo H., Karhunen P.J. Apolipoprotein E-dependent accumulation of Alzheimer disease-related lesions begins in middle age. Ann. Neurol. 2009, 65:650-657.
82. Komatsu M., Waguri S., Chiba T., Murata S., Iwata J., Tanida I., Ueno T., Koike M., Uchiyama Y., Kominami E., Tanaka K. Loss of autophagy in the central nervous system causes neurodegeneration in mice. Nature 2006, 441:880-884.
83. Kong J., Xu Z. Massive mitochondrial degeneration in motor neurons triggers the onset of amyotrophic lateral sclerosis in mice expressing a mutant SOD1. J. Neurosci. 1998, 18:3241-3250.
84. Kuo J.J., Siddique T., Fu R., Heckman C.J. Increased persistent Na(+) current and its effect on excitability in motoneurones cultured from mutant SOD1 mice. J. Physiol. 2005, 563:843-854.
85. Kwiatkowski T.J., Bosco D.A., Leclerc A.L., Tamrazian E., Vanderburg C.R., Russ C., Davis A., Gilchrist J., Kasarskis E.J., Munsat T., et al. Mutations in the FUS/TLS gene on chromosome 16 cause familial amyotrophic lateral sclerosis. Science 2009, 323:1205-1208.
86. Lammich S., Schöbel S., Zimmer A.K., Lichtenthaler S.F., Haass C. Expression of the Alzheimer protease BACE1 is suppressed via its 5′-untranslated region. EMBO Rep. 2004, 5:620-625.
87. Lee H.J., Patel S., Lee S.J. Intravesicular localization and exocytosis of alpha-synuclein and its aggregates. J. Neurosci. 2005, 25:6016-6024.
88. Lessing D., Bonini N.M. Maintaining the brain: insight into human neurodegeneration from Drosophila melanogaster mutants. Nat. Rev. Genet. 2009, 10:359-370.
89. Lim J., Crespo-Barreto J., Jafar-Nejad P., Bowman A.B., Richman R., Hill D.E., Orr H.T., Zoghbi H.Y. Opposing effects of polyglutamine expansion on native protein complexes contribute to SCA1. Nature 2008, 452:713-718.
90. Lin M.T., Beal M.F. Mitochondrial dysfunction and oxidative stress in neurodegenerative diseases. Nature 2006, 443:787-795.
91. Lin J.H., Walter P., Yen T.S.B. Endoplasmic reticulum stress in disease pathogenesis. Annu. Rev. Pathol. 2008, 3:399-425.
92. Lino M.M., Schneider C., Caroni P. Accumulation of SOD1 mutants in postnatal motoneurons does not cause motoneuron pathology or motoneuron disease. J. Neurosci. 2002, 22:4825-4832.
93. Liu J., Lillo C., Jonsson P.A., Vande Velde C., Ward C.M., Miller T.M., Subramaniam J.R., Rothstein J.D., Marklund S., Andersen P.M., et al. Toxicity of familial ALS-linked SOD1 mutants from selective recruitment to spinal mitochondria. Neuron 2004, 43:5-17.
94. Lobsiger C.S., Cleveland D.W. Glial cells as intrinsic components of non-cell-autonomous neurodegenerative disease. Nat. Neurosci. 2007, 10:1355-1360.
95. Lorenzo L.E., Barbe A., Portalier P., Fritschy J.M., Bras H. Differential expression of GABAA and glycine receptors in ALS-resistant vs. ALS-vulnerable motoneurons: possible implications for selective vulnerability of motoneurons. Eur. J. Neurosci. 2006, 23:3161-3170.
96. Mackic J.B., Bading J., Ghiso J., Walker L., Wisniewski T., Frangione B., Zlokovic B.V. Circulating amyloid-beta peptide crosses the blood-brain barrier in aged monkeys and contributes to Alzheimer's disease lesions. Vascul. Pharmacol. 2002, 38:303-313.
97. Malhotra J.D., Kaufman R.J. The endoplasmic reticulum and the unfolded protein response. Semin. Cell Dev. Biol. 2007, 18:716-731.
98. Maragakis N.J., Rothstein J.D. Mechanisms of disease: astrocytes in neurodegenerative disease. Nat. Clin. Pract. Neurol. 2006, 2:679-689.
99. Mattson M.P., Magnus T. Ageing and neuronal vulnerability. Nat. Rev. Neurosci. 2006, 7:278-294.
100. Matus S., Glimcher L.H., Hetz C. Protein folding stress in neurodegenerative diseases: a glimpse into the ER. Curr. Opin. Cell Biol. 2011, 23:239-252.
101. McKee A.C., Cantu R.C., Nowinski C.J., Hedley-Whyte E.T., Gavett B.E., Budson A.E., Santini V.E., Lee H.S., Kubilus C.A., Stern R.A. Chronic traumatic encephalopathy in athletes: progressive tauopathy after repetitive head injury. J. Neuropathol. Exp. Neurol. 2009, 68:709-735.
102. Meyer-Luehmann M., Stalder M., Herzig M.C., Kaeser S.A., Kohler E., Pfeifer M., Boncristiano S., Mathews P.M., Mercken M., Abramowski D., et al. Extracellular amyloid formation and associated pathology in neural grafts. Nat. Neurosci. 2003, 6:370-377.
103. Meyer-Luehmann M., Coomaraswamy J., Bolmont T., Kaeser S., Schaefer C., Kilger E., Neuenschwander A., Abramowski D., Frey P., Jaton A.L., et al. Exogenous induction of cerebral beta-amyloidogenesis is governed by agent and host. Science 2006, 313:1781-1784.
104. Meyer-Luehmann M., Spires-Jones T.L., Prada C., Garcia-Alloza M., de Calignon A., Rozkalne A., Koenigsknecht-Talboo J., Holtzman D.M., Bacskai B.J., Hyman B.T. Rapid appearance and local toxicity of amyloid-beta plaques in a mouse model of Alzheimer's disease. Nature 2008, 451:720-724.
105. Morimoto R.I. Proteotoxic stress and inducible chaperone networks in neurodegenerative disease and aging. Genes Dev. 2008, 22:1427-1438.
106. Morley J.F., Brignull H.R., Weyers J.J., Morimoto R.I. The threshold for polyglutamine-expansion protein aggregation and cellular toxicity is dynamic and influenced by aging in Caenorhabditis elegans. Proc. Natl. Acad. Sci. USA 2002, 99:10417-10422.
107. Mosconi L., De Santi S., Li J., Tsui W.H., Li Y., Boppana M., Laska E., Rusinek H., de Leon M.J. Hippocampal hypometabolism predicts cognitive decline from normal aging. Neurobiol. Aging 2008, 29:676-692.
108. Mosharov E.V., Larsen K.E., Kanter E., Phillips K.A., Wilson K., Schmitz Y., Krantz D.E., Kobayashi K., Edwards R.H., Sulzer D. Interplay between cytosolic dopamine, calcium, and alpha-synuclein causes selective death of substantia nigra neurons. Neuron 2009, 62:218-229.
109. Naert G., Rivest S. CC chemokine receptor 2 deficiency aggravates cognitive impairments and amyloid pathology in a transgenic mouse model of Alzheimer's disease. J. Neurosci. 2011, 31:6208-6220.
110. Nemani V.M., Lu W., Berge V., Nakamura K., Onoa B., Lee M.K., Chaudhry F.A., Nicoll R.A., Edwards R.H. Increased expression of alpha-synuclein reduces neurotransmitter release by inhibiting synaptic vesicle reclustering after endocytosis. Neuron 2010, 65:66-79.
111. Nikolaev A., McLaughlin T., O'Leary D.D., Tessier-Lavigne M. APP binds DR6 to trigger axon pruning and neuron death via distinct caspases. Nature 2009, 457:981-989.
112. O'Connor T., Sadleir K.R., Maus E., Velliquette R.A., Zhao J., Cole S.L., Eimer W.A., Hitt B., Bembinster L.A., Lammich S., et al. Phosphorylation of the translation initiation factor eIF2α increases BACE1 levels and promotes amyloidogenesis. Neuron 2008, 60:988-1009.
113. Palop J.J., Chin J., Mucke L. A network dysfunction perspective on neurodegenerative diseases. Nature 2006, 443:768-773.
114. Palop J.J., Chin J., Roberson E.D., Wang J., Thwin M.T., Bien-Ly N., Yoo J., Ho K.O., Yu G.Q., Kreitzer A., et al. Aberrant excitatory neuronal activity and compensatory remodeling of inhibitory hippocampal circuits in mouse models of Alzheimer's disease. Neuron 2007, 55:697-711.
115. Pasinelli P., Belford M.E., Lennon N., Bacskai B.J., Hyman B.T., Trotti D., Brown R.H. Amyotrophic lateral sclerosis-associated SOD1 mutant proteins bind and aggregate with Bcl-2 in spinal cord mitochondria. Neuron 2004, 43:19-30.
116. Prahlad V., Morimoto R.I. Integrating the stress response: lessons for neurodegenerative diseases from C. elegans. Trends Cell Biol. 2009, 19:52-61.
117. Pun S., Santos A.F., Saxena S., Xu L., Caroni P. Selective vulnerability and pruning of phasic motoneuron axons in motoneuron disease alleviated by CNTF. Nat. Neurosci. 2006, 9:408-419.
118. Rabinovici G.D., Jagust W.J. Amyloid imaging in aging and dementia: testing the amyloid hypothesis in vivo. Behav. Neurol. 2009, 21:117-128.
119. Reiman E.M., Chen K., Alexander G.E., Caselli R.J., Bandy D., Osborne D., Saunders A.M., Hardy J. Functional brain abnormalities in young adults at genetic risk for late-onset Alzheimer's dementia. Proc. Natl. Acad. Sci. USA 2004, 101:284-289.
120. Ron D., Walter P. Signal integration in the endoplasmic reticulum unfolded protein response. Nat. Rev. Mol. Cell Biol. 2007, 8:519-529.
121. Ron-Harel N., Schwartz M. Immune senescence and brain aging: can rejuvenation of immunity reverse memory loss?. Trends Neurosci. 2009, 32:367-375.
122. Roth D.M., Balch W.E. Modeling general proteostasis: proteome balance in health and disease. Curr. Opin. Cell Biol. 2011, 23:126-134.
123. Rothstein J.D. Excitotoxicity and neurodegeneration in amyotrophic lateral sclerosis. Clin. Neurosci. 1995-1996, 3:348-359.
124. Rutkowski D.T., Hegde R.S. Regulation of basal cellular physiology by the homeostatic unfolded protein response. J. Cell Biol. 2010, 189:783-794.
125. Rutkowski D.T., Kaufman R.J. That which does not kill me makes me stronger: adapting to chronic ER stress. Trends Biochem. Sci. 2007, 32:469-476.
126. Saxena S., Cabuy E., Caroni P. A role for motoneuron subtype-selective ER stress in disease manifestations of FALS mice. Nat. Neurosci. 2009, 12:627-636.
127. Selkoe D.J. Soluble oligomers of the amyloid beta-protein impair synaptic plasticity and behavior. Behav. Brain Res. 2008, 192:106-113.
128. Sheng D., Qu D., Kwok K.H., Ng S.S., Lim A.Y., Aw S.S., Lee C.W., Sung W.K., Tan E.K., Lufkin T., et al. Deletion of the WD40 domain of LRRK2 in Zebrafish causes Parkinsonism-like loss of neurons and locomotive defect. PLoS Genet. 2010, 6:e1000914. 10.1371/journal.pgen.1000914.
129. Sherer T.B., Betarbet R., Testa C.M., Seo B.B., Richardson J.R., Kim J.H., Miller G.W., Yagi T., Matsuno-Yagi A., Greenamyre J.T. Mechanism of toxicity in rotenone models of Parkinson's disease. J. Neurosci. 2003, 23:10756-10764.
130. Siklós L., Engelhardt J.I., Alexianu M.E., Gurney M.E., Siddique T., Appel S.H. Intracellular calcium parallels motoneuron degeneration in SOD-1 mutant mice. J. Neuropathol. Exp. Neurol. 1998, 57:571-587.
131. Simard A.R., Soulet D., Gowing G., Julien J.P., Rivest S. Bone marrow-derived microglia play a critical role in restricting senile plaque formation in Alzheimer's disease. Neuron 2006, 49:489-502.
132. Spät A., Szanda G., Csordás G., Hajnóczky G. High- and low-calcium-dependent mechanisms of mitochondrial calcium signalling. Cell Calcium 2008, 44:51-63.
133. Sperling R.A., Laviolette P.S., O'Keefe K., O'Brien J., Rentz D.M., Pihlajamaki M., Marshall G., Hyman B.T., Selkoe D.J., Hedden T., et al. Amyloid deposition is associated with impaired default network function in older persons without dementia. Neuron 2009, 63:178-188.
134. Sreedharan J., Blair I.P., Tripathi V.B., Hu X., Vance C., Rogelj B., Ackerley S., Durnall J.C., Williams K.L., Buratti E., et al. TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis. Science 2008, 319:1668-1672.
135. St-Pierre J., Drori S., Uldry M., Silvaggi J.M., Rhee J., Jäger S., Handschin C., Zheng K., Lin J., Yang W., et al. Suppression of reactive oxygen species and neurodegeneration by the PGC-1 transcriptional coactivators. Cell 2006, 127:397-408.
136. Sulzer D. Multiple hit hypotheses for dopamine neuron loss in Parkinson's disease. Trends Neurosci. 2007, 30:244-250.
137. Surmeier D.J., Ding J., Day M., Wang Z., Shen W. D1 and D2 dopamine-receptor modulation of striatal glutamatergic signaling in striatal medium spiny neurons. Trends Neurosci. 2007, 30:228-235.
138. Szczygielski J., Mautes A., Steudel W.I., Falkai P., Bayer T.A., Wirths O.J. Traumatic brain injury: cause or risk of Alzheimer's disease? A review of experimental studies. J. Neural Transm. 2005, 112:1547-1564.
139. Tansey M.G., McCoy M.K., Frank-Cannon T.C. Neuroinflammatory mechanisms in Parkinson's disease: potential environmental triggers, pathways, and targets for early therapeutic intervention. Exp. Neurol. 2007, 208:1-25.
140. Teuling E., Ahmed S., Haasdijk E., Demmers J., Steinmetz M.O., Akhmanova A., Jaarsma D., Hoogenraad C.C. Motor neuron disease-associated mutant vesicle-associated membrane protein-associated protein (VAP) B recruits wild-type VAPs into endoplasmic reticulum-derived tubular aggregates. J. Neurosci. 2007, 27:9801-9815.
141. 2+ leak channels, a function disrupted by familial Alzheimer's disease-linked mutations. Cell 2006, 126:981-993.
142. Urushitani M., Ezzi S.A., Matsuo A., Tooyama I., Julien J.P. The endoplasmic reticulum-Golgi pathway is a target for translocation and aggregation of mutant superoxide dismutase linked to ALS. FASEB J. 2008, 22:2476-2487.
143. van Zundert B., Peuscher M.H., Hynynen M., Chen A., Neve R.L., Brown R.H., Constantine-Paton M., Bellingham M.C. Neonatal neuronal circuitry shows hyperexcitable disturbance in a mouse model of the adult-onset neurodegenerative disease amyotrophic lateral sclerosis. J. Neurosci. 2008, 28:10864-10874.
144. Vande Velde C., Miller T.M., Cashman N.R., Cleveland D.W. Selective association of misfolded ALS-linked mutant SOD1 with the cytoplasmic face of mitochondria. Proc. Natl. Acad. Sci. USA 2008, 105:4022-4027.
145. Vanselow B.K., Keller B.U. Calcium dynamics and buffering in oculomotor neurones from mouse that are particularly resistant during amyotrophic lateral sclerosis (ALS)-related motoneurone disease. J. Physiol. 2000, 525:433-445.
146. Vassar R., Kovacs D.M., Yan R., Wong P.C. The beta-secretase enzyme BACE in health and Alzheimer's disease: regulation, cell biology, function, and therapeutic potential. J. Neurosci. 2009, 29:12787-12794.
147. Velliquette R.A., O'Connor T., Vassar R. Energy inhibition elevates beta-secretase levels and activity and is potentially amyloidogenic in APP transgenic mice: possible early events in Alzheimer's disease pathogenesis. J. Neurosci. 2005, 25:10874-10883.
148. Verkhratsky A. Physiology and pathophysiology of the calcium store in the endoplasmic reticulum of neurons. Physiol. Rev. 2005, 85:201-279.
149. Vermeer S.E., Prins N.D., den Heijer T., Hofman A., Koudstaal P.J., Breteler M.M. Silent brain infarcts and the risk of dementia and cognitive decline. N. Engl. J. Med. 2003, 348:1215-1222.
150. Vives-Bauza C., Zhou C., Huang Y., Cui M., de Vries R.L., Kim J., May J., Tocilescu M.A., Liu W., Ko H.S., et al. PINK1-dependent recruitment of Parkin to mitochondria in mitophagy. Proc. Natl. Acad. Sci. USA 2010, 107:378-383.
151. 2+, mitochondria and selective motoneuron vulnerability: implications for ALS. Trends Neurosci. 2005, 28:494-500.
152. Vucic S., Nicholson G.A., Kiernan M.C. Cortical hyperexcitability may precede the onset of familial amyotrophic lateral sclerosis. Brain 2008, 131:1540-1550.
153. Wallace D.C. A mitochondrial paradigm of metabolic and degenerative diseases, aging, and cancer: a dawn for evolutionary medicine. Annu. Rev. Genet. 2005, 39:359-407.
154. Wen Y., Yu W.H., Maloney B., Bailey J., Ma J., Marié I., Maurin T., Wang L., Figueroa H., Herman M., et al. Transcriptional regulation of beta-secretase by p25/cdk5 leads to enhanced amyloidogenic processing. Neuron 2008, 57:680-690.
155. Weydt P., Pineda V.V., Torrence A.E., Libby R.T., Satterfield T.F., Lazarowski E.R., Gilbert M.L., Morton G.J., Bammler T.K., Strand A.D., et al. Thermoregulatory and metabolic defects in Huntington's disease transgenic mice implicate PGC-1alpha in Huntington's disease neurodegeneration. Cell Metab. 2006, 4:349-362.
156. Williams A.J., Paulson H.L. Polyglutamine neurodegeneration: protein misfolding revisited. Trends Neurosci. 2008, 31:521-528.
157. Winklhofer K.F., Tatzelt J., Haass C. The two faces of protein misfolding: gain- and loss-of-function in neurodegenerative diseases. EMBO J. 2008, 27:336-349.
158. Wong P.C., Pardo C.A., Borchelt D.R., Lee M.K., Copeland N.G., Jenkins N.A., Sisodia S.S., Cleveland D.W., Price D.L. An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria. Neuron 1995, 14:1105-1116.
159. Xia W. Exploring Alzheimer's disease in zebrafish. J. Alzheimers Dis. 2010, 20:981-990.
160. Yang L.B., Lindholm K., Yan R., Citron M., Xia W., Yang X.L., Beach T., Sue L., Wong P., Price D., et al. Elevated beta-secretase expression and enzymatic activity detected in sporadic Alzheimer disease. Nat. Med. 2003, 9:3-4.
161. Yoshiyama Y., Higuchi M., Zhang B., Huang S.M., Iwata N., Saido T.C., Maeda J., Suhara T., Trojanowski J.Q., Lee V.M. Synapse loss and microglial activation precede tangles in a P301S tauopathy mouse model. Neuron 2007, 53:337-351.
162. Zacchigna S., Lambrechts D., Carmeliet P. Neurovascular signalling defects in neurodegeneration. Nat. Rev. Neurosci. 2008, 9:169-181.
163. Zeron M.M., Hansson O., Chen N., Wellington C.L., Leavitt B.R., Brundin P., Hayden M.R., Raymond L.A. Increased sensitivity to N-methyl-D-aspartate receptor-mediated excitotoxicity in a mouse model of Huntington's disease. Neuron 2002, 33:849-860.
164. Zhang K., Kaufman R.J. From endoplasmic-reticulum stress to the inflammatory response. Nature 2008, 454:455-462.
165. Zhao W., Beers D.R., Henkel J.S., Zhang W., Urushitani M., Julien J.P., Appel S.H. Extracellular mutant SOD1 induces microglial-mediated motoneuron injury. Glia 2010, 58:231-243.
166. Zhong Z., Deane R., Ali Z., Parisi M., Shapovalov Y., O'Banion M.K., Stojanovic K., Sagare A., Boillee S., Cleveland D.W., Zlokovic B.V. ALS-causing SOD1 mutants generate vascular changes prior to motor neuron degeneration. Nat. Neurosci. 2008, 11:420-422.
167. Zlokovic B.V. Neurovascular mechanisms of Alzheimer's neurodegeneration. Trends Neurosci. 2005, 28:202-208.
168. Zuccato C., Ciammola A., Rigamonti D., Leavitt B.R., Goffredo D., Conti L., MacDonald M.E., Friedlander R.M., Silani V., Hayden M.R., et al. Loss of huntingtin-mediated BDNF gene transcription in Huntington's disease. Science 2001, 293:493-498.