Mitochondrial dynamics in cell death and neurodegeneration (original) (raw)

References

  1. Liesa M, Palacín M, Zorzano A (2009) Mitochondrial dynamics in mammalian health and disease. Physiol Rev 89:799–845
    Article CAS PubMed Google Scholar
  2. Chan DC (2006) Mitochondria: dynamic organelles in disease, aging, and development. Cell 125:1241–1252
    Article CAS PubMed Google Scholar
  3. Chan DC (2006) Mitochondrial fusion and fission in mammals. Annu Rev Cell Dev Biol 22:79–99
    Article CAS PubMed Google Scholar
  4. Hales KG, Fuller MT (1997) Developmentally regulated mitochondrial fusion mediated by a conserved, novel, predicted GTPase. Cell 90:121–129
    Article CAS PubMed Google Scholar
  5. Shin HW, Shinotsuka C, Torii S, Murakami K, Nakayama K (1997) Identification and subcellular localization of a novel mammalian dynamin-related protein homologous to yeast Vps1p and Dnm1p. J Biochem 122:525–530
    CAS PubMed Google Scholar
  6. Smirnova E, Shurland DL, Ryazantsev SN, van der Bliek AM (1998) A human dynamin-related protein controls the distribution of mitochondria. J Cell Biol 143:351–358
    Article CAS PubMed Google Scholar
  7. Alexander C, Votruba M, Pesch UE, Thiselton DL, Mayer S, Moore A, Rodriguez M, Kellner U, Leo-Kottler B, Auburger G, Bhattacharya SS, Wissinger B (2000) OPA1, encoding a dynamin-related GTPase, is mutated in autosomal dominant optic atrophy linked to chromosome 3q28. Nat Genet 26:211–215
    Article CAS PubMed Google Scholar
  8. Delettre C, Lenaers G, Griffoin JM, Gigarel N, Lorenzo C, Belenguer P, Pelloquin L, Grosgeorge J, Turc-Carel C, Perret E, Astarie-Dequeker C, Lasquellec L, Arnaud B, Ducommun B, Kaplan J, Hamel CP (2000) Nuclear gene OPA1, encoding a mitochondrial dynamin-related protein, is mutated in dominant optic atrophy. Nat Genet 26:207–210
    Article CAS PubMed Google Scholar
  9. Santel A, Fuller MT (2001) Control of mitochondrial morphology by a human mitofusin. J Cell Sci 114:867–874
    CAS PubMed Google Scholar
  10. Praefcke GJ, McMahon HT (2004) The dynamin superfamily: universal membrane tubulation and fission molecules? Nat Rev Mol Cell Biol 5:133–147
    Article CAS PubMed Google Scholar
  11. Smirnova E, Griparic L, Shurland DL, van der Bliek AM (2001) Dynamin-related protein Drp1 is required for mitochondrial division in mammalian cells. Mol Biol Cell 12:2245–2256
    CAS PubMed Google Scholar
  12. Frank S, Gaume B, Bergmann-Leitner ES, Leitner WW, Robert EG, Catez F, Smith CL, Youle RJ (2001) The role of dynamin-related protein 1, a mediator of mitochondrial fission, in apoptosis. Dev Cell 1:515–525
    Article CAS PubMed Google Scholar
  13. Yoon Y, Pitts KR, McNiven MA (2001) Mammalian dynamin-like protein DLP1 tubulates membranes. Mol Biol Cell 12:2894–2905
    CAS PubMed Google Scholar
  14. Hoppins S, Nunnari J (2009) The molecular mechanism of mitochondrial fusion. Biochim Biophys Acta 1793:20–26
    Article CAS PubMed Google Scholar
  15. Olichon A, Baricault L, Gas N, Guillou E, Valette A, Belenguer P, Lenaers G (2003) Loss of OPA1 perturbates the mitochondrial inner membrane structure and integrity, leading to cytochrome c release and apoptosis. J Biol Chem 278:7743–7746
    Article CAS PubMed Google Scholar
  16. Suen DF, Norris KL, Youle RJ (2008) Mitochondrial dynamics and apoptosis. Genes Dev 22:1577–1590
    Article CAS PubMed Google Scholar
  17. Olichon A, Guillou E, Delettre C, Landes T, Arnauné-Pelloquin L, Emorine LJ, Mils V, Daloyau M, Hamel C, Amati-Bonneau P, Bonneau D, Reynier P, Lenaers G, Belenguer P (2006) Mitochondrial dynamics and disease, OPA1. Biochim Biophys Acta 1763:500–509
    Article CAS PubMed Google Scholar
  18. Knott AB, Perkins G, Schwarzenbacher R, Bossy-Wetzel E (2008) Mitochondrial fragmentation in neurodegeneration. Nat Rev Neurosci 9:505–518
    Article CAS PubMed Google Scholar
  19. Zorzano A, Liesa M, Palacín M (2009) Mitochondrial dynamics as a bridge between mitochondrial dysfunction and insulin resistance. Arch Physiol Biochem 115:1–12
    Article CAS PubMed Google Scholar
  20. Su B, Wang X, Zheng L, Perry G, Smith MA, Zhu X (2010) Abnormal mitochondrial dynamics and neurodegenerative diseases. Biochim Biophys Acta 1802:135–142
    CAS PubMed Google Scholar
  21. Züchner S, Mersiyanova IV, Muglia M, Bissar-Tadmouri N, Rochelle J, Dadali EL, Zappia M, Nelis E, Patitucci A, Senderek J, Parman Y, Evgrafov O, Jonghe PD, Takahashi Y, Tsuji S, Pericak-Vance MA, Quattrone A, Battaloglu E, Polyakov AV, Timmerman V, Schröder JM, Vance JM (2004) Mutations in the mitochondrial GTPase mitofusin 2 cause Charcot-Marie-Tooth neuropathy type 2A. Nat Genet 36:449–451
    Article PubMed CAS Google Scholar
  22. Lawson VH, Graham BV, Flanigan KM (2005) Clinical and electrophysiologic features of CMT2A with mutations in the mitofusin 2 gene. Neurology 65:197–204
    Article CAS PubMed Google Scholar
  23. Cartoni R, Martinou JC (2009) Role of mitofusin 2 mutations in the physiopathology of Charcot-Marie-Tooth disease type 2A. Exp Neurol 218:268–273
    Article CAS PubMed Google Scholar
  24. Harder Z, Zunino R, McBride H (2004) Sumo1 conjugates mitochondrial substrates and participates in mitochondrial fission. Curr Biol 14:340–345
    CAS PubMed Google Scholar
  25. Ishihara N, Fujita Y, Oka T, Mihara K (2006) Regulation of mitochondrial morphology through proteolytic cleavage of OPA1. EMBO J 25:2966–2977
    Article CAS PubMed Google Scholar
  26. Nakamura N, Kimura Y, Tokuda M, Honda S, Hirose S (2006) MARCH-V is a novel mitofusin 2- and Drp1-binding protein able to change mitochondrial morphology. EMBO Rep 7:1019–1022
    Article CAS PubMed Google Scholar
  27. Chang CR, Blac Kstone C (2007) Cyclic AMP-dependent protein kinase phosphorylation of Drp1 regulates its GTPase activity and mitochondrial morphology. J Biol Chem 282:21583–21587
    Article CAS PubMed Google Scholar
  28. Hajek P, Chomyn A, Attardi G (2007) Identification of a novel mitochondrial complex containing mitofusin 2 and stomatin-like protein 2. J Biol Chem 282:5670–5681
    Article CAS PubMed Google Scholar
  29. Taguchi N, Ishihara N, Jofuku A, Oka T, Mihara K (2007) Mitotic phosphorylation of dynamin-related GTPase Drp1 participates in mitochondrial fission. J Biol Chem 282:11521–11529
    Article CAS PubMed Google Scholar
  30. Wasiak S, Zunino R, McBride HM (2007) Bax/Bak promote sumoylation of DRP1 and its stable association with mitochondria during apoptotic cell death. J Cell Biol 177:439–450
    Article CAS PubMed Google Scholar
  31. Zunino R, Schauss A, Rippstein P, Andrade-Navarro M, McBride HM (2007) The SUMO protease SENP5 is required to maintain mitochondrial morphology and function. J Cell Sci 120:1178–1188
    Article CAS PubMed Google Scholar
  32. Han XJ, Lu YF, Li SA, Kaitsuka T, Sato Y, Tomizawa K, Nairn AC, Takei K, Matsui H, Matsushita M (2008) CaM kinase I alpha-induced phosphorylation of Drp1 regulates mitochondrial morphology. J Cell Biol 182:573–585
    Article CAS PubMed Google Scholar
  33. Cho DH, Nakamura T, Fang J, Cieplak P, Godzik A, Gu Z, Lipton SA (2009) _S_-Nitrosylation of Drp1 mediates b-amyloid-related mitochondrial fission and neuronal injury. Science 324:102–105
    Article CAS PubMed Google Scholar
  34. Koch A, Thiemann M, Grabenbauer M, Yoon Y, McNiven MA, Schrader M (2003) Dynamin-like protein 1 is involved in peroxisomal fission. J Biol Chem 278:8597–8605
    Article CAS PubMed Google Scholar
  35. Pitts KR, McNiven MA, Yoon Y (2004) Mitochondria-specific function of the dynamin family protein DLP1 is mediated by its C-terminal domains. J Biol Chem 279:50286–55094
    Article CAS PubMed Google Scholar
  36. Bossy-Wetzel E, Barsoum MJ, Godzik A, Schwarzenbacher R, Lipton SA (2003) Mitochondrial fission in apoptosis, neurodegeneration and aging. Curr Opin Cell Biol 15:706–716
    Article CAS PubMed Google Scholar
  37. Zhang P, Hinshaw JE (2001) Three-dimensional reconstruction of dynamin in the constricted state. Nat Cell Biol 3:922–926
    Article CAS PubMed Google Scholar
  38. Zhu PP, Patterson A, Stadler J, Seeburg DP, Sheng M, Blackstone C (2004) Intra- and intermolecular domain interactions of the C-terminal GTPase effector domain of the multimeric dynamin-like GTPase Drp1. J Biol Chem 279:35967–35974
    Article CAS PubMed Google Scholar
  39. Damke H, Binns DD, Ueda H, Schmid SL, Baba T (2001) Dynamin GTPase domain mutants block endocytic vesicle formation at morphologically distinct stages. Mol Biol Cell 12:2578–2589
    CAS PubMed Google Scholar
  40. Ingerman E, Perkins EM, Marino M, Mears JA, McCaffery JM, Hinshaw JE, Nunnari JJ (2005) Dnm1 forms spirals that are structurally tailored to fit mitochondria. Cell Biol 170:1021–1027
    Article CAS Google Scholar
  41. Ramachandran R, Surka M, Chappie JS, Fowler DM, Foss TR, Song BD, Schmid SL (2007) The dynamin middle domain is critical for tetramerization and higher-order self-assembly. EMBO J 26:559–566
    Article CAS PubMed Google Scholar
  42. Lackner LL, Horner JS, Nunnari J (2009) Mechanistic analysis of a dynamin effector. Science 325:874–877
    Article CAS PubMed Google Scholar
  43. James DI, Parone PA, Mattenberger Y, Martinou JC (2003) hFis1, a novel component of the mammalian mitochondrial fission machinery. J Biol Chem 278:36373–36379
    Article CAS PubMed Google Scholar
  44. Yoon Y, Krueger EW, Oswald BJ, McNiven MA (2003) The mitochondrial protein hFis1 regulates mitochondrial fission in mammalian cells through an interaction with the dynamin-like protein DLP1. Mol Cell Biol 23:5409–5420
    Article CAS PubMed Google Scholar
  45. Lee YJ, Jeong SY, Karbowski M, Smith CL, Youle RJ (2004) Roles of the mammalian mitochondrial fission and fusion mediators Fis1, Drp1, and Opa1 in apoptosis. Mol Biol Cell 15:5001–5011
    Article CAS PubMed Google Scholar
  46. Cribbs JT, Strack S (2007) Reversible phosphorylation of Drp1 by cyclic AMP-dependent protein kinase and calcineurin regulates mitochondrial fission and cell death. EMBO Rep 8:939–944
    Article CAS PubMed Google Scholar
  47. Cereghetti GM, Stangherlin A, Martins, de Brito O, Chang CR, Blackstone C, Bernardi P, Scorrano L (2008) Dephosphorylation by calcineurin regulates translocation of Drp1 to mitochondria. Proc Natl Acad Sci USA 105:15803–15808
  48. Meuer K, Suppanz IE, Lingor P, Planchamp V, Göricke B, Fichtner L, Braus GH, Dietz GP, Jakobs S, Bähr M, Weishaupt JH (2007) Cyclin-dependent kinase 5 is an upstream regulator of mitochondrial fission during neuronal apoptosis. Cell Death Differ 14:651–661
    Article CAS PubMed Google Scholar
  49. Wang X, Su B, Lee HG, Li X, Perry G, Smith MA, Zhu X (2009) Impaired balance of mitochondrial fission and fusion in Alzheimer’s disease. J Neurosci 29:9090–9103
    Article CAS PubMed Google Scholar
  50. Figueroa-Romero C, Iñiguez-Lluh JA, Stadler J, Chang CR, Arnoult D, Keller PJ, Hong Y, Blackstone C, Feldman EL (2009) SUMOylation of the mitochondrial fission protein Drp1 occurs at multiple nonconsensus sites within the B domain and is linked to its activity cycle. FASEB J 23:3917–3927
    Article CAS PubMed Google Scholar
  51. Yonashiro R, Ishido S, Kyo S, Fukuda T, Goto E, Matsuki Y, Ohmura-Hoshino M, Sada K, Hotta H, Yamamura H, Inatome R, Yanagi S (2006) A novel mitochondrial ubiquitin ligase plays a critical role in mitochondrial dynamics. EMBO J 25:3618–3626
    Article CAS PubMed Google Scholar
  52. Karbowski M, Neutzner A, Youle RJ (2007) The mitochondrial E3 ubiquitin ligase MARCH5 is required for Drp1 dependent mitochondrial division. J Cell Biol 178:71–84
    Article CAS PubMed Google Scholar
  53. Braschi E, Zunino R, McBride HM (2009) MAPL is a new mitochondrial SUMO E3 ligase that regulates mitochondrial fission. EMBO Rep 10:748–754
    Article CAS PubMed Google Scholar
  54. Rojo M, Legros F, Chateau D, Lombès A (2002) Membrane topology and mitochondrial targeting of mitofusins, ubiquitous mammalian homologs of the transmembrane GTPase Fzo. J Cell Sci 115:1663–1674
    CAS PubMed Google Scholar
  55. Santel A (2006) Get the balance right: mitofusins roles in health and disease. Biochim Biophys Acta 1763:490–499
    Article CAS PubMed Google Scholar
  56. Santel A, Frank S, Gaume B, Herrler M, Youle RJ, Fuller MT (2003) Mitofusin-1 protein is a generally expressed mediator of mitochondrial fusion in mammalian cells. J Cell Sci 116:2763–2774
    Article CAS PubMed Google Scholar
  57. Neuspiel M, Zunino R, Gangaraju S, Rippstein P, McBride H (2005) Activated mitofusin 2 signals mitochondrial fusion, interferes with Bax activation, and reduces susceptibility to radical induced depolarization. J Biol Chem 280:25060–25070
    Article CAS PubMed Google Scholar
  58. Karbowski M, Norris KL, Cleland MM, Jeong SY, Youle RJ (2006) Role of Bax and Bak in mitochondrial morphogenesis. Nature 443:658–662
    Article CAS PubMed Google Scholar
  59. Koshiba T, Detmer SA, Kaiser JT, Chen H, McCaffery JM, Chan DC (2004) Structural basis of mitochondrial tethering by mitofusin complexes. Science 305:858–862
    Article CAS PubMed Google Scholar
  60. Chen H, Detmer SA, Ewald AJ, Griffin EE, Fraser SE, Chan DC (2003) Mitofusins Mfn1 and Mfn2 coordinately regulate mitochondrial fusion and are essential for embryonic development. J Cell Biol 160:189–200
    Article CAS PubMed Google Scholar
  61. Chen H, McCaffery JM, Chan DC (2007) Mitochondrial fusion protects against neurodegeneration in the cerebellum. Cell 130:548–562
    Article CAS PubMed Google Scholar
  62. Bach D, Pich S, Soriano FX, Vega N, Baumgartner B, Oriola J, Daugaard JR, Lloberas J, Camps M, Zierath JR, Rabasa-Lhoret R, Wallberg-Henriksson H, Laville M, Palacín M, Vidal H, Rivera F, Brand M, Zorzano A (2003) Mitofusin-2 determines mitochondrial network architecture and mitochondrial metabolism. A novel regulatory mechanism altered in obesity. J Biol Chem 278:17190–17197
    Article CAS PubMed Google Scholar
  63. Chen H, Chomyn A, Chan DC (2005) Disruption of fusion results in mitochondrial heterogeneity and dysfunction. J Biol Chem 280:26185–26192
    Article CAS PubMed Google Scholar
  64. Cipolat S, Martins de Brito O, Dal Zilio B, Scorrano L (2004) OPA1 requires mitofusin 1 to promote mitochondrial fusion. Proc Natl Acad Sci USA 101:15927–15932
    Article CAS PubMed Google Scholar
  65. Olichon A, Landes T, Arnauné-Pelloquin L, Emorine LJ, Mils V, Guichet A, Delettre C, Hamel C, Amati-Bonneau P, Bonneau D, Reynier P, Lenaers G, Belenguer P (2007) Effects of OPA1 mutations on mitochondrial morphology and apoptosis: relevance to ADOA pathogenesis. J Cell Physiol 211:423–430
    Article CAS PubMed Google Scholar
  66. Griparic L, van der Wel NN, Orozco IJ, Peters PJ, van der Bliek AM (2004) Loss of the intermembrane space protein Mgm1/OPA1 induces swelling and localized constrictions along the lengths of mitochondria. J Biol Chem 279:18792–18798
    Article CAS PubMed Google Scholar
  67. Olichon A, Elachouri G, Baricault L, Delettre C, Belenguer P, Lenaers G (2007) OPA1 alternate splicing uncouples an evolutionary conserved function in mitochondrial fusion from a vertebrate restricted function in apoptosis. Cell Death Differ 14:682–692
    Article CAS PubMed Google Scholar
  68. McQuibban GA, Saurya S, Freeman M (2003) Mitochondrial membrane remodelling regulated by a conserved rhomboid protease. Nature 423:537–541
    Article CAS PubMed Google Scholar
  69. Cipolat S, Rudka T, Hartmann D, Costa V, Serneels L, Craessaerts K, Metzger K, Frezza C, Annaert W, D’Adamio L, Derks C, Dejaegere T, Pellegrini L, D’Hooge R, Scorrano L, De Strooper B (2006) Mitochondrial rhomboid PARL regulates cytochrome c release during apoptosis via OPA1-dependent cristae remodeling. Cell 126:163–175
    Article CAS PubMed Google Scholar
  70. Duvezin-Caubet S, Koppen M, Wagener J, Zick M, Israel L, Bernacchia A, Jagasia R, Rugarli EI, Imhof A, Neupert W, Langer T, Reichert AS (2007) OPA1 processing reconstituted in yeast depends on the subunit composition of the m-AAA protease in mitochondria. Mol Biol Cell 18:3582–3590
    Article CAS PubMed Google Scholar
  71. Song Z, Chen H, Fiket M, Alexander C, Chan DC (2007) A1 processing controls mitochondrial fusion and is regulated by mRNA splicing, membrane potential, and Yme1L. J Cell Biol 178:749–755
    Article CAS PubMed Google Scholar
  72. Guillery O, Malka F, Landes T, Guillou E, Blackstone C, Lombès A, Belenguer P, Arnoult D, Rojo M (2008) Metalloprotease-mediated OPA1 processing is modulated by the mitochondrial membrane potential. Biol Cell 100:315–325
    Article CAS PubMed Google Scholar
  73. Satoh M, Hamamoto T, Seo N, Kagawa Y, Endo H (2003) Differential sublocalization of the dynamin-related protein OPA1 isoforms in mitochondria. Biochem Biophys Res Commun 300:482–493
    Article CAS PubMed Google Scholar
  74. Frezza C, Cipolat S, Martins de Brito O, Micaroni M, Beznoussenko GV, Rudka T, Bartoli D, Polishuck RS, Danial NN, De Strooper B, Scorrano L (2006) OPA1 controls apoptotic cristae remodeling independently from mitochondrial fusion. Cell 126:177–189
    Article CAS PubMed Google Scholar
  75. Sesaki H, Jensen RE (2004) Ugo1p links the Fzo1p and Mgm1p GTPases for mitochondrial fusion. J Biol Chem 279:28298–28303
    Article CAS PubMed Google Scholar
  76. Coonrod EM, Karren MA, Shaw JM (2007) Ugo1p is a multipass transmembrane protein with a single carrier domain required for mitochondrial fusion. Traffic 8:500–511
    Article CAS PubMed Google Scholar
  77. Cuddeback SM, Yamaguchi H, Komatsu K, Miyashita T, Yamada M, Wu C, Singh S, Wang HG (2001) Molecular cloning and characterization of Bif-1. A novel Src homology 3 domain-containing protein that associates with Bax. J Biol Chem 276:20559–20565
    Article CAS PubMed Google Scholar
  78. Karbowski M, Jeong SY, Youle RJ (2004) Endophilin B1 is required for the maintenance of mitochondrial morphology. J Cell Biol 166:1027–1039
    Article CAS PubMed Google Scholar
  79. Peter BJ, Kent HM, Mills IG, Vallis Y, Butler PJ, Evans PR, McMahon HT (2004) BAR domains as sensors of membrane curvature: the amphiphysin BAR structure. Science 303:495–499
    Article CAS PubMed Google Scholar
  80. Arnoult D, Rismanchi N, Grodet A, Roberts RG, Seeburg DP, Estaquier J, Sheng M, Blackstone C (2005) Bax/Bak-dependent release of DDP/TIMM8a promotes Drp1-mediated mitochondrial fission and mitoptosis during programmed cell death. Curr Biol 15:2112–2118
    Article CAS PubMed Google Scholar
  81. Niemann A, Ruegg M, La Padula V, Schenone A, Suter U (2005) Ganglioside-induced differentiation associated protein 1 is a regulator of the mitochondrial network: new implications for Charcot-Marie-Tooth disease. J Cell Biol 170:1067–1078
    Article CAS PubMed Google Scholar
  82. Wagner KM, Rüegg M, Niemann A, Suter U (2009) Targeting and function of the mitochondrial fission factor GDAP1 are dependent on its tail-anchor. PLoS One 4:e5160
    Article PubMed CAS Google Scholar
  83. Baxter RV, Ben Othmane K, Rochelle JM, Stajich JE, Hulette C, Dew-Knight S, Hentati F, Ben Hamida M, Bel S, Stenger JE, Gilbert JR, Pericak-Vance MA, Vance JM (2002) Ganglioside-induced differentiation-associated protein-1 is mutant in Charcot-Marie-Tooth disease type 4A/8q21. Nat Genet 30:21–22
    Article CAS PubMed Google Scholar
  84. Li W, Bengtson MH, Ulbrich A, Matsuda A, Reddy VA, Orth A, Chanda SK, Batalov S, Joazeiro CA (2008) Genome-wide and functional annotation of human E3 ubiquitin ligases identifies MULAN, a mitochondrial E3 that regulates the organelle’s dynamics and signaling. PLoS One 3:e1487
    Article PubMed CAS Google Scholar
  85. Gandre-Babbe S, van der Bliek AM (2008) The novel tail-anchored membrane protein Mff controls mitochondrial and peroxisomal fission in mammalian cells. Mol Biol Cell 19:2402–2412
    Article CAS PubMed Google Scholar
  86. Aihara T, Nakamura N, Honda S, Hirose S (2009) A novel potential role for gametogenetin-binding protein 1 (GGNBP1) in mitochondrial morphogenesis during spermatogenesis in mice. Biol Reprod 80:762–770
    Article CAS PubMed Google Scholar
  87. Parcellier A, Tintignac LA, Zhuravleva E, Dummler B, Brazil DP, Hynx D, Cron P, Schenk S, Olivieri V, Hemmings BA (2009) The carboxy-terminal modulator protein (CTMP) regulates mitochondrial dynamics. PLoS One 4:e5471
    Article PubMed CAS Google Scholar
  88. Choi SY, Huang P, Jenkins GM, Chan DC, Schiller J, Frohman MA (2006) Common lipid links Mfn-mediated mitochondrial fusion and SNARE-regulated exocytosis. Nat Cell Biol 8:1255–1262
    Article CAS PubMed Google Scholar
  89. Huang H, Frohman MA (2009) Lipid signaling on the mitochondrial surface. Biochim Biophys Acta 1791:839–844
    CAS PubMed Google Scholar
  90. Eura Y, Ishihara N, Oka T, Mihara K (2006) Identification of a novel protein that regulates mitochondrial fusion by modulating mitofusin (Mfn) protein function. J Cell Sci 119:4913–4925
    Article CAS PubMed Google Scholar
  91. Liesa M, Borda-d’Agua B, Medina-Gómez G, Lelliott CJ, Paz JC, Rojo M, Palacín M, Vidal-Puig A, Zorzano A (2008) Mitochondrial fusion is increased by the nuclear coactivator PGC-1beta. PLoS One 3:e3613
    Article PubMed CAS Google Scholar
  92. Tondera D, Grandemange S, Jourdain A, Karbowski M, Mattenberger Y, Herzig S, Da Cruz S, Clerc P, Raschke I, Merkwirth C, Ehses S, Krause F, Chan DC, Alexander C, Bauer C, Youle R, Langer T, Martinou JC (2009) SLP-2 is required for stress-induced mitochondrial hyperfusion. EMBO J 28:1589–1600
    Article CAS PubMed Google Scholar
  93. Zamzami N, Kroemer G (2001) The mitochondrion in apoptosis: how Pandora’s box opens. Nat Rev Mol Cell Biol 2:67–71
    Article CAS PubMed Google Scholar
  94. Ow YP, Green DR, Hao Z, Mak TW (2008) Cytochrome c: functions beyond respiration. Nat Rev Mol Cell Biol 9:532–542
    Article CAS PubMed Google Scholar
  95. Chipuk JE, Green DR (2008) How do BCL-2 proteins induce mitochondrial outer membrane permeabilization? Trends Cell Biol 18:157–164
    Article CAS PubMed Google Scholar
  96. Sugioka R, Shimizu S, Tsujimoto Y (2004) Fzo1, a protein involved in mitochondrial fusion, inhibits apoptosis. J Biol Chem 279:52726–52734
    Article CAS PubMed Google Scholar
  97. Karbowski M, Lee YJ, Gaume B, Jeong SY, Frank S, Nechushtan A, Santel A, Fuller M, Smith CL, Youle RJ (2002) Spatial and temporal association of Bax with mitochondrial fission sites, Drp1, and Mfn2 during apoptosis. J Cell Biol 159:931–938
    Article CAS PubMed Google Scholar
  98. Sheridan C, Delivani P, Cullen SP, Martin SJ (2008) Bax- or Bak-induced mitochondrial fission can be uncoupled from cytochrome C release. Mol Cell 31:570–585
    Article CAS PubMed Google Scholar
  99. Liu QA, Shio H (2008) Mitochondrial morphogenesis, dendrite development, and synapse formation in cerebellum require both Bcl-w and the glutamate receptor delta2. PLoS Genet 4:e1000097
    Article PubMed CAS Google Scholar
  100. Berman SB, Chen YB, Qi B, McCaffery JM, Rucker EB 3rd, Goebbels S, Nave KA, Arnold BA, Jonas EA, Pineda FJ, Hardwick JM (2009) Bcl-x L increases mitochondrial fission, fusion, and biomass in neurons. J Cell Biol 184:707–719
    Article CAS PubMed Google Scholar
  101. Li H, Chen Y, Jones AF, Sanger RH, Collis LP, Flannery R, McNay EC, Yu T, Schwarzenbacher R, Bossy B, Bossy-Wetzel E, Bennett MV, Pypaert M, Hickman JA, Smith PJ, Hardwick JM, Jonas EA (2008) Bcl-xL induces Drp1-dependent synapse formation in cultured hippocampal neurons. Proc Natl Acad Sci USA 105:2169–2174
    Article CAS PubMed Google Scholar
  102. Autret A, Martin SJ (2009) Emerging role for members of the Bcl-2 family in mitochondrial morphogenesis. Mol Cell 36:355–363
    Article CAS PubMed Google Scholar
  103. Breckenridge DG, Stojanovic M, Marcellus RC, Shore GC (2003) Caspase cleavage product of BAP31 induces mitochondrial fission through endoplasmic reticulum calcium signals, enhancing cytochrome c release to the cytosol. J Cell Biol 160:1115–1127
    Article CAS PubMed Google Scholar
  104. Hom JR, Gewandter JS, Michael L, Sheu SS, Yoon Y (2007) Thapsigargin induces biphasic fragmentation of mitochondria through calcium-mediated mitochondrial fission and apoptosis. J Cell Physiol 212:498–508
    Article CAS PubMed Google Scholar
  105. Kong D, Xu L, Yu Y, Zhu W, Andrews DW, Yoon Y, Kuo TH (2005) Regulation of Ca2+-induced permeability transition by Bcl-2 is antagonized by Drpl and hFis1. Mol Cell Biochem 272:187–199
    Article CAS PubMed Google Scholar
  106. Alirol E, James D, Huber D, Marchetto A, Vergani L, Martinou JC, Scorrano L (2006) The mitochondrial fission protein hFis1 requires the endoplasmic reticulum gateway to induce apoptosis. Mol Biol Cell 17:4593–4605
    Article CAS PubMed Google Scholar
  107. Parone PA, James DI, Da Cruz S, Mattenberger Y, Donzé O, Barja F, Martinou JC (2006) Inhibiting the mitochondrial fission machinery does not prevent Bax/Bak-dependent apoptosis. Mol Cell Biol 26:7397–7408
    Article CAS PubMed Google Scholar
  108. Mizushima N, Levine B, Cuervo AM, Klionsky DJ (2008) Autophagy fights disease through cellular self-digestion. Nature 451:1069–1075
    Article CAS PubMed Google Scholar
  109. Levine B, Kroemer G (2008) Autophagy in the pathogenesis of disease. Cell 132:27–42
    Article CAS PubMed Google Scholar
  110. Twig G, Hyde B, Shirihai OS (2008) Mitochondrial fusion, fission and autophagy as a quality control axis: the bioenergetic view. Biochim Biophys Acta 1777:1092–1097
    Article CAS PubMed Google Scholar
  111. Gomes LC, Scorrano L (2008) High levels of Fis1, a pro-fission mitochondrial protein, trigger autophagy. Biochim Biophys Acta 1777:860–866
    Article CAS PubMed Google Scholar
  112. Twig G, Elorza A, Molina AJ, Mohamed H, Wikstrom JD, Walzer G, Stiles L, Haigh SE, Katz S, Las G, Alroy J, Wu M, Py BF, Yuan J, Deeney JT, Corkey BE, Shirihai OS (2008) Fission and selective fusion govern mitochondrial segregation and elimination by autophagy. EMBO J 27:433–446
    Article CAS PubMed Google Scholar
  113. Dagda RK, Cherra SJ 3rd, Kulich SM, Tandon A, Park D, Chu CT (2009) Loss of PINK1 function promotes mitophagy through effects on oxidative stress and mitochondrial fission. J Biol Chem 284:13843–13855
    Article CAS PubMed Google Scholar
  114. Kanki T, Wang K, Cao Y, Baba M, Klionsky DJ (2009) Atg32 is a mitochondrial protein that confers selectivity during mitophagy. Dev Cell 17:98–109
    Article CAS PubMed Google Scholar
  115. Okamoto K, Kondo-Okamoto N, Ohsumi Y (2009) Mitochondria-anchored receptor Atg32 mediates degradation of mitochondria via selective autophagy. Dev Cell 17:87–97
    Article CAS PubMed Google Scholar
  116. Detmer SA, Chan DC (2007) Complementation between mouse Mfn1 and Mfn2 protects mitochondrial fusion defects caused by CMT2A disease mutations. J Cell Biol 176:405–414
    Article CAS PubMed Google Scholar
  117. Baloh RH, Schmidt RE, Pestronk A, Milbrandt J (2007) Altered axonal mitochondrial transport in the pathogenesis of Charcot-Marie-Tooth disease from mitofusin 2 mutations. J Neurosci 27:422–430
    Article CAS PubMed Google Scholar
  118. Detmer SA, Vande Velde C, Cleveland DW, Chan DC (2008) Hindlimb gait defects due to motor axon loss and reduced distal muscles in a transgenic mouse model of Charcot-Marie-Tooth type 2A. Hum Mol Genet 17:367–375
    Article CAS PubMed Google Scholar
  119. Cuesta A, Pedrola L, Sevilla T, García-Planells J, Chumillas MJ, Mayordomo F, LeGuern E, Marín I, Vílchez JJ, Palau F (2002) The gene encoding ganglioside-induced differentiation-associated protein 1 is mutated in axonal Charcot-Marie-Tooth type 4A disease. Nat Genet 30:22–25
    Article CAS PubMed Google Scholar
  120. Berger P, Niemann A, Suter U (2006) Schwann cells and the pathogenesis of inherited motor and sensory neuropathies (Charcot-Marie-Tooth disease). Glia 54:243–257
    Article PubMed Google Scholar
  121. Ferré M, Amati-Bonneau P, Tourmen Y, Malthièry Y, Reynier P (2005) eOPA1: an online database for OPA1 mutations. Hum Mutat 25:423–428
    Article PubMed CAS Google Scholar
  122. Waterham HR, Koster J, van Roermund CW, Mooyer PA, Wanders RJ, Leonard JV (2007) A lethal defect of mitochondrial and peroxisomal fission. N Engl J Med 356:1736–1741
    Article CAS PubMed Google Scholar
  123. LaFerla FM, Green KN, Oddo S (2007) Intracellular amyloid-beta in Alzheimer’s disease. Nat Rev Neurosci 8:499–509
    Article CAS PubMed Google Scholar
  124. Brunden KR, Trojanowski JQ, Lee VM (2009) Advances in tau-focused drug discovery for Alzheimer’s disease and related tauopathies. Nat Rev Drug Discov 8:783–793
    Article CAS PubMed Google Scholar
  125. Yankner BA, Lu T (2009) Amyloid beta-protein toxicity and the pathogenesis of Alzheimer disease. J Biol Chem 284:4755–4759
    Article CAS PubMed Google Scholar
  126. Haass C, Selkoe DJ (2007) Soluble protein oligomers in neurodegeneration: lessons from the Alzheimer’s amyloid beta-peptide. Nat Rev Mol Cell Biol 8:101–112
    Article CAS PubMed Google Scholar
  127. Selkoe DJ (2000) Toward a comprehensive theory for Alzheimer’s disease. Hypothesis: Alzheimer’s disease is caused by the cerebral accumulation and cytotoxicity of amyloid beta-protein. Ann N Y Acad Sci 924:17–25
    Article CAS PubMed Google Scholar
  128. Wyss-Coray T (2006) Inflammation in Alzheimer disease: driving force, bystander or beneficial response? Nat Med 12:1005–1015
    CAS PubMed Google Scholar
  129. Blass JP (2000) The mitochondrial spiral. An adequate cause of dementia in the Alzheimer’s syndrome. Ann N Y Acad Sci 924:170–183
    Article CAS PubMed Google Scholar
  130. Chen JX, Yan SD (2007) Amyloid-beta-induced mitochondrial dysfunction. J Alzheimers Dis 12:177–184
    CAS PubMed Google Scholar
  131. Hansson Petersen CA, Alikhani N, Behbahani H, Wiehager B, Pavlov PF, Alafuzoff I, Leinonen V, Ito A, Winblad B, Glaser E, Ankarcrona M (2008) The amyloid beta-peptide is imported into mitochondria via the TOM import machinery and localized to mitochondrial cristae. Proc Natl Acad Sci USA 105:13145–13150
    Article CAS PubMed Google Scholar
  132. Casley CS, Canevari L, Land JM, Clark JB, Sharpe MA (2002) Beta-amyloid inhibits integrated mitochondrial respiration and key enzyme activities. J Neurochem 80:91–100
    Article CAS PubMed Google Scholar
  133. Crouch PJ, Blake R, Duce JA, Ciccotosto GD, Li QX, Barnham KJ, Curtain CC, Cherny RA, Cappai R, Dyrks T, Masters CL, Trounce IA (2005) Copper-dependent inhibition of human cytochrome c oxidase by a dimeric conformer of amyloid-beta1–42. J Neurosci 25:672–679
    Article CAS PubMed Google Scholar
  134. Lustbader JW, Cirilli M, Lin C, Xu HW, Takuma K, Wang N, Caspersen C, Chen X, Pollak S, Chaney M, Trinchese F, Liu S, Gunn-Moore F, Lue LF, Walker DG, Kuppusamy P, Zewier ZL, Arancio O, Stern D, Yan SS, Wu H (2004) ABAD directly links Abeta to mitochondrial toxicity in Alzheimer’s disease. Science 304:448–452
    Article CAS PubMed Google Scholar
  135. Barsoum MJ, Yuan H, Gerencser AA, Liot G, Kushnareva Y, Gräber S, Kovacs I, Lee WD, Waggoner J, Cui J, White AD, Bossy B, Martinou JC, Youle RJ, Lipton SA, Ellisman MH, Perkins GA, Bossy-Wetzel E (2006) Nitric oxide-induced mitochondrial fission is regulated by dynamin-related GTPases in neurons. EMBO J 25:3900–3911
    Article CAS PubMed Google Scholar
  136. Rui Y, Tiwari P, Xie Z, Zheng JQ (2006) Acute impairment of mitochondrial trafficking by beta-amyloid peptides in hippocampal neurons. J Neurosci 26:10480–10487
    Article CAS PubMed Google Scholar
  137. Wang X, Su B, Siedlak SL, Moreira PI, Fujioka H, Wang Y, Casadesus G, Zhu X (2008) Amyloid-beta overproduction causes abnormal mitochondrial dynamics via differential modulation of mitochondrial fission/fusion proteins. Proc Natl Acad Sci USA 105:19318–19323
    Article CAS PubMed Google Scholar
  138. Abou-Sleiman PM, Muqit MM, Wood NW (2006) Expanding insights of mitochondrial dysfunction in Parkinson’s disease. Nat Rev Neurosci 7:207–219
    Article CAS PubMed Google Scholar
  139. Schapira AH, Bezard E, Brotchie J, Calon F, Collingridge GL, Ferger B, Hengerer B, Hirsch E, Jenner P, Le Novère N, Obeso JA, Schwarzschild MA, Spampinato U, Davidai G (2006) Novel pharmacological targets for the treatment of Parkinson’s disease. Nat Rev Drug Discov 5:845–854
    Article CAS PubMed Google Scholar
  140. Jin J, Li GJ, Davis J, Zhu D, Wang Y, Pan C, Zhang J (2007) Identification of novel proteins associated with both alpha-synuclein and DJ-1. Mol Cell Proteomics 6:845–859
    Article CAS PubMed Google Scholar
  141. Gomez-Lazaro M, Bonekamp NA, Galindo MF, Jordán J, Schrader M (2008) 6-Hydroxydopamine (6-OHDA) induces Drp1-dependent mitochondrial fragmentation in SH-SY5Y cells. Free Radic Biol Med 44:1960–1969
    Article CAS PubMed Google Scholar
  142. Exner N, Treske B, Paquet D, Holmström K, Schiesling C, Gispert S, Carballo-Carbajal I, Berg D, Hoepken HH, Gasser T, Krüger R, Winklhofer KF, Vogel F, Reichert AS, Auburger G, Kahle PJ, Schmid B, Haass C (2007) Loss-of-function of human PINK1 results in mitochondrial pathology and can be rescued by parkin. J Neurosci 27:12413–12418
    Article CAS PubMed Google Scholar
  143. Lutz AK, Exner N, Fett ME, Schlehe JS, Kloos K, Lämmermann K, Brunner B, Kurz-Drexler A, Vogel F, Reichert AS, Bouman L, Vogt-Weisenhorn D, Wurst W, Tatzelt J, Haass C, Winklhofer KF (2009) Loss of parkin or PINK1 function increases Drp1-dependent mitochondrial fragmentation. J Biol Chem 284:22938–22951
    Article CAS PubMed Google Scholar
  144. Grünewald A, Gegg ME, Taanman JW, King RH, Kock N, Klein C, Schapira AH (2009) Differential effects of PINK1 nonsense and missense mutations on mitochondrial function and morphology. Exp Neurol 219:266–273
    Article PubMed CAS Google Scholar
  145. Deng H, Dodson MW, Huang H, Guo M (2008) The Parkinson’s disease genes pink1 and parkin promote mitochondrial fission and/or inhibit fusion in Drosophila. Proc Natl Acad Sci USA 105:14503–14508
    Article CAS PubMed Google Scholar
  146. Poole AC, Thomas RE, Andrews LA, McBride HM, Whitworth AJ, Pallanck LJ (2008) The PINK1/Parkin pathway regulates mitochondrial morphology. Proc Natl Acad Sci USA 105:1638–1643
    Article CAS PubMed Google Scholar
  147. Yang Y, Gehrke S, Imai Y, Huang Z, Ouyang Y, Wang JW, Yang L, Beal MF, Vogel H, Lu B (2006) Mitochondrial pathology and muscle and dopaminergic neuron degeneration caused by inactivation of Drosophila Pink1 is rescued by Parkin. Proc Natl Acad Sci USA 103:10793–10798
    Article CAS PubMed Google Scholar
  148. Ziviani E, Tao RN, Whitworth AJ (2010) Drosophila Parkin requires PINK1 for mitochondrial translocation and ubiquitinates mitofusin. Proc Natl Acad Sci USA 107:5018–5023
    Article CAS PubMed Google Scholar
  149. Narendra DP, Jin SM, Tanaka A, Suen DF, Gautier CA, Shen J, Cookson MR, Youle RJ (2010) PINK1 is selectively stabilized on impaired mitochondria to activate Parkin. PLoS Biol 8:e1000298
    Article PubMed CAS Google Scholar
  150. Geisler S, Holmström KM, Skujat D, Fiesel FC, Rothfuss OC, Kahle PJ, Springer W (2010) PINK1/Parkin-mediated mitophagy is dependent on VDAC1 and p62/SQSTM1. Nat Cell Biol 12:119–311
    Article CAS PubMed Google Scholar
  151. Matsuda N, Sato S, Shiba K, Okatsu K, Saisho K, Gautier CA, Sou Y, Saiki S, Kawajiri S, Sato F, Kimura M, Komatsu M, Hattori N, Tanaka K (2010) PINK1 stabilized by mitochondrial depolarization recruits Parkin to damaged mitochondria and activates latent Parkin for mitophagy. J Cell Biol 189:211–221
    Article CAS PubMed Google Scholar
  152. Bates G (2003) Huntingtin aggregation and toxicity in Huntington’s disease. Lancet 361:1642–1644
    Article CAS PubMed Google Scholar
  153. Reddy PH, Mao P, Manczak M (2009) Mitochondrial structural and functional dynamics in Huntington’s disease. Brain Res Rev 61:33–48
    Article CAS PubMed Google Scholar
  154. Tabrizi SJ, Cleeter MW, Xuereb J, Taanman JW, Cooper JM, Schapira AH (1999) Biochemical abnormalities and excitotoxicity in Huntington’s disease brain. Ann Neurol 45:25–32
    Article CAS PubMed Google Scholar
  155. Seong IS, Ivanova E, Lee JM, Choo YS, Fossale E, Anderson M, Gusella JF, Laramie JM, Myers RH, Lesort M, MacDonald ME (2005) HD CAG repeat implicates a dominant property of huntingtin in mitochondrial energy metabolism. Hum Mol Genet 14:2871–2880
    Article CAS PubMed Google Scholar
  156. Pandey M, Varghese M, Sindhu KM, Sreetama S, Navneet AK, Mohanakumar KP, Usha R (2008) Mitochondrial NAD+-linked State 3 respiration and complex-I activity are compromised in the cerebral cortex of 3-nitropropionic acid-induced rat model of Huntington’s disease. J Neurochem 104:420–434
    CAS PubMed Google Scholar
  157. Brouillet E, Hantraye P, Ferrante RJ, Dolan R, Leroy-Willig A, Kowall NW, Beal MF (1995) Chronic mitochondrial energy impairment produces selective striatal degeneration and abnormal choreiform movements in primates. Proc Natl Acad Sci USA 92:7105–7109
    Article CAS PubMed Google Scholar
  158. Panov AV, Gutekunst CA, Leavitt BR, Hayden MR, Burke JR, Strittmatter WJ, Greenamyre JT (2002) Early mitochondrial calcium defects in Huntington’s disease are a direct effect of polyglutamines. Nat Neurosci 5:731–736
    CAS PubMed Google Scholar
  159. Chang DT, Rintoul GL, Pandipati S, Reynolds IJ (2006) Mutant huntingtin aggregates impair mitochondrial movement and trafficking in cortical neurons. Neurobiol Dis 22:388–400
    Article CAS PubMed Google Scholar
  160. Wang H, Lim PJ, Karbowski M, Monteiro MJ (2009) Effects of overexpression of huntingtin proteins on mitochondrial integrity. Hum Mol Genet 18:737–752
    Article CAS PubMed Google Scholar
  161. Liot G, Bossy B, Lubitz S, Kushnareva Y, Sejbuk N, Bossy-Wetzel E (2009) Complex II inhibition by 3-NP causes mitochondrial fragmentation and neuronal cell death via an NMDA- and ROS-dependent pathway. Cell Death Differ 16:899–909
    Article CAS PubMed Google Scholar

Download references