Skip to main content Accessibility help

We use cookies to distinguish you from other users and to provide you with a better experience on our websites. Close this message to accept cookies or find out how to manage your cookie settings.

Close cookie message
×
Hostname: page-component-848d4c4894-75dct Total loading time: 0 Render date: 2024-05-02T21:49:07.693Z Has data issue: false hasContentIssue false

12 - Role of Programmed Cell Death in Neurodegenerative Disease

from Part II - Cell Death in Tissues and Organs

Published online by Cambridge University Press:  07 September 2011

Douglas R. Green
By
Dale E. Bredesen
Edited by
John C. Reed
Douglas R. Green
Affiliation:
St. Jude Children's Research Hospital, Memphis, Tennessee
Dale E. Bredesen
Affiliation:
University of California – San Francisco
John C. Reed
Affiliation:
Sanford-Burnham Medical Research Institute, La Jolla, California
Get access

Summary

Introduction: Programmed cell death, cell death signaling, and neurodegenerative disease

Many of the diseases that affect the nervous system feature an abnormality of cell death of one sort or another. For example, developmental and neoplastic disorders of the nervous system feature dysregulation of the intrinsic cellular programs that mediate cell death. Furthermore, there is increasing evidence to suggest that such dysregulation may also occur in neurodegenerative, infectious, traumatic, ischemic, metabolic, and demyelinating disorders. Therefore, targeting the central biochemical controls of cell survival and death may represent a productive therapeutic approach, especially if combined with other therapeutic strategies. Furthermore, recent results from stem cell studies suggest that the fate of neural stem cells may also play an important role in disease outcomes, and therefore, cell death apparently plays a central role in many neurological diseases and potentially in their prevention and treatment.

Type
Chapter
Information
Apoptosis
Physiology and Pathology
 , pp. 135 - 144
Publisher: Cambridge University Press
Print publication year: 2011

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Banwait, S, Galvan, V, Zhang, J, et al. C-terminal cleavage of the amyloid-beta protein precursor at Asp664: a switch associated with Alzheimer's disease. J Alzheimers Dis. Feb 2008;13(1):1–16.
Barrett, GL, Bartlett, PF. The p75 nerve growth factor receptor mediates survival or death depending on the stage of sensory neuron development. Proc Natl Acad Sci U S A. 1994;91(14):6501–5.
Barrett, GL, Georgiou, A. The low-affinity nerve growth factor receptor p75NGFR mediates death of PC12 cells after nerve growth factor withdrawal. J Neurosci Res. 1996;45:117–28.
Beher, D, Hesse, L, Masters, CL, Multhaup, G. Regulation of amyloid protein precursor (APP) binding to collagen and mapping of the binding sites on APP and collagen type I. J Biol Chem. Jan 19 1996;271(3):1613–20.
Bianchi, L, Gerstbrein, B, Frokjaer-Jensen, C, et al. The neurotoxic MEC-4(d) DEG/ENaC sodium channel conducts calcium: implications for necrosis initiation. Nat Neurosci. Dec 2004;7(12):1337–44.
Bordeaux, MC, Forcet, C, Granger, L, et al. The RET proto-oncogene induces apoptosis: a novel mechanism for Hirschsprung disease. EMBO J. 2000;19(15):4056–4063.
Boyce, M, Bryant, KF, Jousse, C, et al. A selective inhibitor of eIF2alpha dephosphorylation protects cells from ER stress. Science. Feb 11 2005;307(5711):935–9.
Breckenridge, DG, Stojanovic, M, Marcellus, RC, Shore, GC. Caspase cleavage product of BAP31 induces mitochondrial fission through endoplasmic reticulum calcium signals, enhancing cytochrome c release to the cytosol. J Cell Biol. Mar 31 2003;160(7):1115–27.
Bredesen, DE, Mehlen, P, Rabizadeh, S. Apoptosis and dependence receptors: a molecular basis for cellular addiction. Physiol Rev. Apr 2004;84(2):411–30.
Bredesen, DE, Rabizadeh, S. p75NTR and apoptosis: Trk-dependent and Trk-independent effects. Trends Neurosci. 1997;20(7):287–90.
Bredesen, DE, Rao, RV, Mehlen, P. Cell death in the nervous system. Nature. Oct 19 2006;443(7113):796–802.
Bredesen, DE, Ye, X, Tasinato, A, et al. p75NTR and the concept of cellular dependence: seeing how the other half die [see comments]. Cell Death Differ. 1998;5(5):365–71.
Bredesen, DE. Keeping neurons alive: the molecular control of apoptosis (part I). Neuroscientist. 1996;2:181–90.
Butterfield, DA, Bush, AI. Alzheimer's amyloid beta-peptide (1–42): involvement of methionine residue 35 in the oxidative stress and neurotoxicity properties of this peptide. Neurobiol Aging. May-Jun 2004;25(5):563–8.
Caceres, J, Brandan, E. Interaction between Alzheimer's disease beta A4 precursor protein (APP) and the extracellular matrix: evidence for the participation of heparan sulfate proteoglycans. J Cell Biochem. May 1997;65(2):145–58.
Calfon, M, Zeng, H, Urano, F, et al. IRE1 couples endoplasmic reticulum load to secretory capacity by processing the XBP-1 mRNA. Nature. Jan 3 2002;415(6867):92–6.
Chae, HJ, Kim, HR, Xu, C, et al. BI-1 regulates an apoptosis pathway linked to endoplasmic reticulum stress. Mol Cell. Aug 13 2004;15(3):355–66.
Clarke, PG. Developmental cell death: morphological diversity and multiple mechanisms. Anat Embryol. 1990;181(3):195–13.
Dal Canto, MC, Gurney, ME. Development of central nervous system pathology in a murine transgenic model of human amyotrophic lateral sclerosis. Am J Pathol. 1994;145(6):1271–9.
Dobson, CM, Ellis, RJ. Protein folding and misfolding inside and outside the cell. EMBO J. Sep 15 1998;17(18):5251–4.
Dobson, CM. Protein folding and misfolding. Nature. Dec 18 2003;426(6968):884–90.
Ellerby, LM, Andrusiak, RL, Wellington, CL, et al. Cleavage of atrophin-1 at caspase site aspartic acid 109 modulates cytotoxicity. J Biol Chem. 1999;274(13):8730–6.
Ellerby, LM, Hackam, AS, Propp, SS, et al. Kennedy's disease: caspase cleavage of the androgen receptor is a crucial event in cytotoxicity. J Neurochem. 1999;72(1):185–95.
Fombonne, J, Rabizadeh, S, Banwait, S, Mehlen, P, Bredesen, DE. Selective vulnerability in Alzheimer's disease: amyloid precursor protein and p75NTR interaction. Ann Neurol. Mar 2009;65(3):294–303.
Forcet, C, Ye, X, Granger, L, et al. The dependence receptor DCC (deleted in colorectal cancer) defines an alternative mechanism for caspase activation. Proc Natl Acad Sci U S A. 2001;98(6):3416–21.
Friedlander, RM, Brown, RH, Gagliardini, V, Wang, J, Yuan, J. Inhibition of ICE slows ALS in mice [letter] [published erratum appears in Nature 1998 Apr 9;392(6676):560]. Nature. 1997;388(6637):31.
Galvan, V, Gorostiza, OF, Banwait, S, et al. Reversal of Alzheimer's-like pathology and behavior in human APP transgenic mice by mutation of Asp664. Proc Natl Acad Sci U S A. May 2 2006;103(18):7130–5.
Gervais, FG, Xu, D, Robertson, GS, et al. Involvement of caspases in proteolytic cleavage of Alzheimer's amyloid-beta precursor protein and amyloidogenic A beta peptide formation. Cell. 1999;97(3):395–406.
Gilloteaux, J, Jamison, JM, Arnold, D, et al. Cancer cell necrosis by autoschizis: synergism of antitumor activity of vitamin C: vitamin K3 on human bladder carcinoma T24 cells. Scanning. Nov 1998;20(8):564–75.
Goldberg, AL. Protein degradation and protection against misfolded or damaged proteins. Nature. Dec 18 2003;426(6968):895–9.
Goldberg, YP, Nicholson, DW, Rasper, DM, et al. Cleavage of huntingtin by apopain, a proapoptotic cysteine protease, is modulated by the polyglutamine tract. Nature Genet. 1996;13:442–9.
Graham, RK, Deng, Y, Slow, EJ, et al. Cleavage at the caspase-6 site is required for neuronal dysfunction and degeneration due to mutant huntingtin. Cell. Jun 16 2006;125(6):1179–91.
Hegde, RS, Mastrianni, JA, Scott, MR, et al. A transmembrane form of the prion protein in neurodegenerative disease. Science. 1998;279(5352):827–34.
Hermel, E, Gafni, J, Propp, SS, et al. Specific caspase interactions and amplification are involved in selective neuronal vulnerability in Huntington's disease. Cell Death Differ. Apr 2004;11(4):424–38.
Hirabayashi, M, Inoue, K, Tanaka, K, et al. VCP/p97 in abnormal protein aggregates, cytoplasmic vacuoles, and cell death, phenotypes relevant to neurodegeneration. Cell Death Differ. Oct 2001;8(10):977–84.
Ho, A, Sudhof, TC. Binding of F-spondin to amyloid-beta precursor protein: a candidate amyloid-beta precursor protein ligand that modulates amyloid-beta precursor protein cleavage. Proc Natl Acad Sci U S A. Feb 24 2004;101(8):2548–53.
Kaufman, RJ. Stress signaling from the lumen of the endoplasmic reticulum: coordination of gene transcriptional and translational controls. Genes Dev. May 15 1999;13(10):1211–33.
Kaushik, S, Cuervo, AM. Chaperone-mediated autophagy. Methods Mol Biol. 2008;445:227–44.
Kawahara, T, Yanagi, H, Yura, T, Mori, K. Endoplasmic reticulum stress-induced mRNA splicing permits synthesis of transcription factor Hac1p/Ern4p that activates the unfolded protein response. Mol Biol Cell. Oct 1997;8(10):1845–62.
Klein, WL. Abeta toxicity in Alzheimer's disease: globular oligomers (ADDLs) as new vaccine and drug targets. Neurochem Int. Nov 2002;41(5):345–52.
Kobayashi, T, Tanaka, K, Inoue, K, Kakizuka, A. Functional ATPase activity of p97/valosin-containing protein (VCP) is required for the quality control of endoplasmic reticulum in neuronally differentiated mammalian PC12 cells. J Biol Chem. Dec 6 2002;277(49):47358–65.
Kopito, RR, Ron, D. Conformational disease. Nat Cell Biol. 2000;2(11):E207–9.
Kopito, RR. Aggresomes, inclusion bodies and protein aggregation. Trends Cell Biol. 2000;10(12):524–30.
Lee, K, Tirasophon, W, Shen, X, et al. IRE1-mediated unconventional mRNA splicing and S2P-mediated ATF6 cleavage merge to regulate XBP1 in signaling the unfolded protein response. Genes Dev. Feb 15 2002;16(4):452–66.
Li, J, Lee, B, Lee, AS. Endoplasmic reticulum stress-induced apoptosis: multiple pathways and activation of p53-up-regulated modulator of apoptosis (PUMA) and NOXA by p53. J Biol Chem. Mar 17 2006;281(11):7260–70.
Liu, X, Van Vleet, T, Schnellmann, RG. The role of calpain in oncotic cell death. Annu Rev Pharmacol Toxicol. 2004;44:349–70.
Llambi, F, Causeret, F, Bloch-Gallego, E, Mehlen, P. Netrin-1 acts as a survival factor via its receptors UNC5H and DCC. EMBO J. 2001;20(11):2715–22.
Lourenco, FC, Galvan, V, Fombonne, J, et al. Netrin-1 interacts with amyloid precursor protein and regulates amyloid-beta production. Cell Death Differ. May 2009;16(5):655–63.
Lu, DC, Rabizadeh, S, Chandra, S, et al. A second cytotoxic proteolytic peptide derived from amyloid β-protein precursor [see comments]. Nature Med. 2000;6(4):397–404.
Lu, DC, Shaked, GM, Masliah, E, Bredesen, DE, Koo, EH. Amyloid beta protein toxicity mediated by the formation of amyloid-beta protein precursor complexes. Ann Neurol. Dec 2003;54(6):781–9.
Lu, DC, Soriano, S, Bredesen, DE, Koo, EH. Caspase cleavage of the amyloid precursor protein modulates amyloid beta-protein toxicity. J Neurochem. Nov 2003;87(3):733–41.
Mah, SP, Zhong, LT, Liu, Y, Roghani, A, Edwards, RH, Bredesen, DE. The protooncogene bcl-2 inhibits apoptosis in PC12 cells. J Neurochem. 1993;60(3):1183–6.
Mahul-Mellier, AL, Hemming, FJ, Blot, B, Fraboulet, S, Sadoul, R. Alix, making a link between apoptosis-linked gene-2, the endosomal sorting complexes required for transport, and neuronal death in vivo. J Neurosci. Jan 11 2006;26(2):542–9.
Mathai, JP, Germain, M, Shore, GC. BH3-only BIK regulates BAX,BAK-dependent release of Ca2+ from endoplasmic reticulum stores and mitochondrial apoptosis during stress-induced cell death. J Biol Chem. Jun 24 2005;280(25):23829–36.
Mehlen, P, Rabizadeh, S, Snipas, SJ, Assa-Munt, N, Salvesen, GS, Bredesen, DE. The DCC gene product induces apoptosis by a mechanism requiring receptor proteolysis. Nature. 1998;395(6704):801–4.
Mucke, L, Masliah, E, Yu, GQ, et al. High-level neuronal expression of abeta 1–42 in wild-type human amyloid protein precursor transgenic mice: synaptotoxicity without plaque formation. J Neurosci. Jun 1 2000;20(11):4050–8.
Ng, FW, Nguyen, M, Kwan, T, et al. p28 Bap31, a Bcl-2/Bcl-XL- and procaspase-8-associated protein in the endoplasmic reticulum. J Cell Biol. Oct 20 1997;139(2):327–38.
Nguyen, TV, Galvan, V, Huang, W, et al. Signal transduction in Alzheimer disease: p21-activated kinase signaling requires C-terminal cleavage of APP at Asp664. J Neurochem. Feb 2008;104(4):1065–80.
Nikolaev, VO, Lohse, MJ. Novel techniques for real-time monitoring of cGMP in living cells. Handb Exp Pharmacol. 2009(191):229–43.
Nishimoto, I. A new paradigm for neurotoxicity by FAD mutants of betaAPP: a signaling abnormality. Neurobiol Aging. Jan-Feb 1998;19(1 Suppl):S33–8.
Ona, VO, Li, M, Vonsattel, JPG, et al. Inhibition of caspase-1 slows disease progression in a mouse model of Huntington's disease. Nature. 1999;399:263–7.
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. Jun 2001;13(3):349–55.
Rabizadeh, S, Bredesen, DE. Is p75NGFR involved in developmental neural cell death? Dev Neurosci. 1994;16(3–4):207–11.
Rabizadeh, S, Oh, J, Zhong, LT, et al. Induction of apoptosis by the low-affinity NGF receptor. Science. 1993;261(5119):345–8.
Rao, RV, Bredesen, DE. Misfolded proteins, endoplasmic reticulum stress and neurodegeneration. Curr Opin Cell Biol. Dec 2004;16(6):653–62.
Rao, RV, Castro-Obregon, S, Frankowski, H, et al. Coupling endoplasmic reticulum stress to the cell death program. An Apaf-1-independent intrinsic pathway. J Biol Chem. 2002;277(24):21836–42.
Rao, RV, Hermel, E, Castro-Obregon, S, et al. Coupling endoplasmic reticulum stress to the cell death program. Mechanism of caspase activation. J Biol Chem. Sep 7 2001;276(36):33869–74.
Rao, RV, Niazi, K, Mollahan, P, et al. Coupling endoplasmic reticulum stress to the cell-death program: a novel HSP90-independent role for the small chaperone protein p23. Cell Death Differ. Mar 2006;13(3):415–25.
Rao, RV, Peel, A, Logvinova, A, et al. Coupling endoplasmic reticulum stress to the cell death program: role of the ER chaperone GRP78. FEBS Lett. Mar 13 2002;514(2–3):122–8.
Rao, RV, Poksay, KS, Castro-Obregon, S, et al. Molecular components of a cell death pathway activated by endoplasmic reticulum stress. J Biol Chem. Jan 2 2004;279(1):177–87.
Reddy, RK, Mao, C, Baumeister, P, Austin, RC, Kaufman, RJ, Lee, AS. Endoplasmic reticulum chaperone protein GRP78 protects cells from apoptosis induced by topoisomerase inhibitors: role of ATP binding site in suppression of caspase-7 activation. J Biol Chem. Jun 6 2003;278(23):20915–24.
Roberson, ED, Scearce-Levie, K, Palop, JJ, et al. Reducing endogenous tau ameliorates amyloid beta-induced deficits in an Alzheimer's disease mouse model. Science. May 4 2007;316(5825):750–4.
Roth, W, Kermer, P, Krajewska, M, et al. Bifunctional apoptosis inhibitor (BAR) protects neurons from diverse cell death pathways. Cell Death Differ. Oct 2003;10(10):1178–87.
Ruiz-Vela, A, Opferman, JT, Cheng, EH, Korsmeyer, SJ. Proapoptotic BAX and BAK control multiple initiator caspases. EMBO Rep. Apr 2005;6(4):379–85.
Saganich, MJ, Schroeder, BE, Galvan, V, Bredesen, DE, Koo, EH, Heinemann, SF. Deficits in synaptic transmission and learning in amyloid precursor protein (APP) transgenic mice require C-terminal cleavage of APP. J Neurosci. Dec 27 2006;26(52):13428–36.
Salvesen, GS, Dixit, VM. Caspases: intracellular signaling by proteolysis. Cell. 1997;91(4):443–6.
Scorrano, L, Oakes, SA, Opferman, JT, et al. BAX and BAK regulation of endoplasmic reticulum Ca2+: a control point for apoptosis. Science. Apr 4 2003;300(5616):135–9.
Selkoe, DJ. Folding proteins in fatal ways. Nature. Dec 18 2003;426(6968):900–4.
Shaked, GM, Kummer, MP, Lu, DC, Galvan, V, Bredesen, DE, Koo, EH. A{beta} induces cell death by direct interaction with its cognate extracellular domain on APP (APP 597–624). FASEB J. Jun 2006;20(8):1254–6.
Sherman, MY, Goldberg, AL. Cellular defenses against unfolded proteins: a cell biologist thinks about neurodegenerative diseases. Neuron. 2001;29(1):15–32.
Sidrauski, C, Walter, P. The transmembrane kinase Ire1p is a site-specific endonuclease that initiates mRNA splicing in the unfolded protein response. Cell. Sep 19 1997;90(6):1031–9.
Sitia, R, Braakman, I. Quality control in the endoplasmic reticulum protein factory. Nature. Dec 18 2003;426(6968):891–4.
Stupack, DG, Puente, XS, Boutsaboualoy, S, Storgard, CM, Cheresh, DA. Apoptosis of adherent cells by recruitment of caspase-8 to unligated integrins. J Cell Biol. Oct 29 2001;155(3):459–70.
Susin, SA, Zamzami, N, Castedo, M, Hirsch, T, Marchetti, P, Macho, A, Daugas, E, Geuskens, M, Kroemer, G. Bcl-2 inhibits the mitochondrial release of an apoptogenic protease. J Exp Med. 1996; 1;184(4):1331–41.
Syntichaki, P, Xu, K, Driscoll, M, Tavernarakis, N. Specific aspartyl and calpain proteases are required for neurodegeneration in C. elegans. Nature. Oct 31 2002;419(6910):939–44.
Tanaka, Y, Igarashi, S, Nakamura, M, et al. Progressive phenotype and nuclear accumulation of an amino-terminal cleavage fragment in a transgenic mouse model with inducible expression of full-length mutant huntingtin. Neurobiol Dis. Feb 2006;21(2):381–91.
Taylor, JP, Hardy, J, Fischbeck, KH. Toxic proteins in neurodegenerative disease. Science. 2002;296(5575):1991–5.
Thibert, C, Teillet, MA, Lapointe, F, Mazelin, L, Le Douarin, NM, Mehlen, P. Inhibition of neuroepithelial patched-induced apoptosis by Sonic hedgehog. Science. Aug 8 2003;301(5634):843–6.
Turmaine, M, Raza, A, Mahal, A, Mangiarini, L, Bates, GP, Davies, SW. Nonapoptotic neurodegeneration in a transgenic mouse model of Huntington's disease. Proc Natl Acad Sci U S A. 2000;97(14):8093–7.
Urano, F, Wang, X, Bertolotti, A, et al. Coupling of stress in the ER to activation of JNK protein kinases by transmembrane protein kinase IRE1. Science. Jan 28 2000;287(5453):664–6.
Watts, GD, Wymer, J, Kovach, MJ, et al. Inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia is caused by mutant valosin-containing protein. Nat Genet. Apr 2004;36(4):377–81.
Wellington, CL, Singaraja, R, Ellerby, L, et al. Inhibiting caspase cleavage of huntingtin reduces toxicity and aggregate formation in neuronal and nonneuronal cells. J Biol Chem. Jun 30 2000;275(26):19831–8.
Williamson, TL, Marszalek, JR, Vechio, JD, et al. Neurofilaments, radial growth of axons, and mechanisms of motor neuron disease. Cold Spring Harb Symp Quant Biol. 1996;61:709–23. Neuron. 2001 Sep 27;31(6):957–71.
Xu, K, Tavernarakis, N, Driscoll, M. Necrotic cell death in C. elegans requires the function of calreticulin and regulators of Ca(2+) release from the endoplasmic reticulum. Neuron. 2001 Sep 27;31(6):957–71.
Yang, F, Sun, X, Beech, W, et al. Antibody to caspase-cleaved actin detects apoptosis in differentiated neuroblastoma and plaque-associated neurons and microglia in Alzheimer's disease. Am J Pathol. Feb 1998;152(2):379–89.
Yao, R, Cooper, GM. Requirement for phosphatiylinositol-3 kinase in the prevention of apoptosis by nerve growth factor. Science. 1995;267:2003–6.
Yoshida, H, Matsui, T, Yamamoto, A, Okada, T, Mori, K. XBP1 mRNA is induced by ATF6 and spliced by IRE1 in response to ER stress to produce a highly active transcription factor. Cell. Dec 28 2001;107(7):881–91.
Yu, SW, Wang, H, Poitras, MF, et al. Mediation of poly(ADP-ribose) polymerase-1-dependent cell death by apoptosis-inducing factor. Science. Jul 12 2002;297(5579):259–63.
Zhang, H, Xu, Q, Krajewski, S, et al. BAR: An apoptosis regulator at the intersection of caspases and Bcl-2 family proteins. Proc Natl Acad Sci U S A. Mar 14 2000;97(6):2597–2602.

Save book to Kindle

To save this book to your Kindle, first ensure coreplatform@cambridge.org is added to your Approved Personal Document E-mail List under your Personal Document Settings on the Manage Your Content and Devices page of your Amazon account. Then enter the ‘name’ part of your Kindle email address below. Find out more about saving to your Kindle.

Note you can select to save to either the @free.kindle.com or @kindle.com variations. ‘@free.kindle.com’ emails are free but can only be saved to your device when it is connected to wi-fi. ‘@kindle.com’ emails can be delivered even when you are not connected to wi-fi, but note that service fees apply.

Find out more about the Kindle Personal Document Service.

Available formats
×

Save book to Dropbox

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Dropbox.

Available formats
×

Save book to Google Drive

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Google Drive.

Available formats
×