Mitochondrial protein quality control in health and disease

British Journal of Pharmacology

Volumn 171, Issue 8, 2014, Pages 1870-1889

  Baker, Michael J ^a   Palmer, Catherine S ^b   Stojanovski, Diana ^a,c  

^a UNIVERSITY OF MELBOURNE   (Australia)

^b LA TROBE UNIVERSITY   (Australia)

^c LA TROBE UNIVERSITY   (Australia)

Author keywords

apoptosis; mitochondria; mitochondrial morphology; neurodegenerative disease; protease; protein quality control

Indexed keywords

MITOCHONDRIAL PROTEIN; PROTEOME;

ALZHEIMER DISEASE; AMYOTROPHIC LATERAL SCLEROSIS; APOPTOSIS; AUTOSOMAL DOMINANT OPTIC ATROPHY; CELL FUNCTION; CELL METABOLISM; CELL ORGANELLE; CELL POPULATION; CELL SURVIVAL; CELL TRANSPORT; CELLULAR DISTRIBUTION; DEGENERATIVE DISEASE; HEREDITARY MOTOR SENSORY NEUROPATHY; HUMAN; HUNTINGTON CHOREA; MITOCHONDRIAL DYNAMICS; MITOCHONDRION; MITOPHAGY; NONHUMAN; PARKINSON DISEASE; PRIORITY JOURNAL; QUALITY CONTROL; REVIEW; SPINOCEREBELLAR DEGENERATION; BIOLOGICAL MODEL; CELL DEATH; HOMEOSTASIS; METABOLISM;

CELL DEATH; HOMEOSTASIS; HUMANS; MITOCHONDRIAL DEGRADATION; MITOCHONDRIAL PROTEINS; MITOCHONDRIAL TURNOVER; MODELS, BIOLOGICAL; NEURODEGENERATIVE DISEASES;

EID: 84897390686     PISSN: 00071188     EISSN: 14765381     Source Type: Journal    
DOI: 10.1111/bph.12430     Document Type: Review

References (259)

1. Alexander C, Votruba M, Pesch UE, Thiselton DL, Mayer S, Moore A, et al. (2000). OPA1, encoding a dynamin-related GTPase, is mutated in autosomal dominant optic atrophy linked to chromosome 3q28. Nat Genet 26: 211-215.
2. Anand R, Langer T, Baker MJ, (2013). Proteolytic control of mitochondrial function and morphogenesis. Biochim Biophys Acta 1833: 195-204.
3. Anesti V, Scorrano L, (2006). The relationship between mitochondrial shape and function and the cytoskeleton. Biochim Biophys Acta 1757: 692-699.
4. Anton F, Fres JM, Schauss A, Pinson B, Praefcke GJ, Langer T, et al. (2011). Ugo1 and Mdm30 act sequentially during Fzo1-mediated mitochondrial outer membrane fusion. J Cell Sci 124 (Pt 7): 1126-1135.
5. Anton F, Dittmar G, Langer T, Escobar-Henriques M, (2013). Two deubiquitylases act on mitofusin and regulate mitochondrial fusion along independent pathways. Mol Cell 49: 487-498.
6. Arlt H, Steglich G, Perryman R, Guiard B, Neupert W, Langer T, (1998). The formation of respiratory chain complexes in mitochondria is under the proteolytic control of the m-AAA protease. EMBO J 17: 4837-4847.
7. Azzu V, Brand MD, (2010). Degradation of an intramitochondrial protein by the cytosolic proteasome. J Cell Sci 123 (Pt 4): 578-585.
8. Baker BM, Haynes CM, (2011). Mitochondrial protein quality control during biogenesis and aging. Trends Biochem Sci 36: 254-261.
9. Baker MJ, Tatsuta T, Langer T, (2011). Quality control of mitochondrial proteostasis. Cold Spring Harb Perspect Biol 3: a007559.
10. Baker MJ, Mooga VP, Guiard B, Langer T, Ryan MT, Stojanovski D, (2012). Impaired folding of the mitochondrial small TIM chaperones induces clearance by the i-AAA protease. J Mol Biol 424: 227-239.
11. Bateman JM, Iacovino M, Perlman PS, Butow RA, (2002). Mitochondrial DNA instability mutants of the bifunctional protein Ilv5p have altered organization in mitochondria and are targeted for degradation by Hsp78 and the Pim1p protease. J Biol Chem 277: 47946-47953.
12. Baxter RV, Ben Othmane K, Rochelle JM, Stajich JE, Hulette C, Dew-Knight S, et al. (2002). Ganglioside-induced differentiation-associated protein-1 is mutant in Charcot-Marie-Tooth disease type 4A/8q21. Nat Genet 30: 21-22.
13. Becker T, Vogtle FN, Stojanovski D, Meisinger C, (2008). Sorting and assembly of mitochondrial outer membrane proteins. Biochim Biophys Acta 1777: 557-563.
14. Becker T, Gebert M, Pfanner N, van der Laan M, (2009). Biogenesis of mitochondrial membrane proteins. Curr Opin Cell Biol 21: 484-493.
15. Bereiter-Hahn J, Voth M, (1994). Dynamics of mitochondria in living cells: shape changes, dislocations, fusion, and fission of mitochondria. Microsc Res Tech 27: 198-219.
16. Bleazard W, McCaffery JM, King EJ, Bale S, Mozdy A, Tieu Q, et al. (1999). The dynamin-related GTPase Dnm1 regulates mitochondrial fission in yeast. Nat Cell Biol 1: 298-304.
17. Boldogh IR, Pon LA, (2007). Mitochondria on the move. Trends Cell Biol 17: 502-510.
18. Bonda DJ, Wang X, Perry G, Nunomura A, Tabaton M, Zhu X, et al. (2010). Oxidative stress in Alzheimer disease: a possibility for prevention. Neuropharmacology 59: 290-294.
19. Bonn F, Pantakani K, Shoukier M, Langer T, Mannan AU, (2010). Functional evaluation of paraplegin mutations by a yeast complementation assay. Hum Mutat 31: 617-621.
20. Bonn F, Tatsuta T, Petrungaro C, Riemer J, Langer T, (2011). Presequence-dependent folding ensures MrpL32 processing by the m-AAA protease in mitochondria. EMBO J 30: 2545-2556.
21. Bota DA, Davies KJ, (2002). Lon protease preferentially degrades oxidized mitochondrial aconitase by an ATP-stimulated mechanism. Nat Cell Biol 4: 674-680.
22. Bragoszewski P, Gornicka A, Sztolsztener ME, Chacinska A, (2013). The ubiquitin-proteasome system regulates mitochondrial intermembrane space proteins. Mol Cell Biol 33: 2136-2148.
23. Braschi E, Zunino R, McBride HM, (2009). MAPL is a new mitochondrial SUMO E3 ligase that regulates mitochondrial fission. EMBO Rep 10: 748-754.
24. de Brito OM, Scorrano L, (2008). Mitofusin 2 tethers endoplasmic reticulum to mitochondria. Nature 456: 605-610.
25. Bross P, Naundrup S, Hansen J, Nielsen MN, Christensen JH, Kruhoffer M, et al. (2008). The Hsp60-(p.V98I) mutation associated with hereditary spastic paraplegia SPG13 compromises chaperonin function both in vitro and in vivo. J Biol Chem 283: 15694-15700.
26. Cagnoli C, Stevanin G, Brussino A, Barberis M, Mancini C, Margolis RL, et al. (2010). Missense mutations in the AFG3L2 proteolytic domain account for approximately 1.5% of European autosomal dominant cerebellar ataxias. Hum Mutat 31: 1117-1124.
27. Casari G, De Fusco M, Ciarmatori S, Zeviani M, Mora M, Fernandez P, et al. (1998). Spastic paraplegia and OXPHOS impairment caused by mutations in paraplegin, a nuclear-encoded mitochondrial metalloprotease. Cell 93: 973-983.
28. Caspersen C, Wang N, Yao J, Sosunov A, Chen X, Lustbader JW, et al. (2005). Mitochondrial Abeta: a potential focal point for neuronal metabolic dysfunction in Alzheimer's disease. FASEB J 19: 2040-2041.
29. Cassereau J, Chevrollier A, Gueguen N, Desquiret V, Verny C, Nicolas G, et al. (2011). Mitochondrial dysfunction and pathophysiology of Charcot-Marie-Tooth disease involving GDAP1 mutations. Exp Neurol 227: 31-41.
30. Cereghetti GM, Stangherlin A, Martins de Brito O, Chang CR, Blackstone C, Bernardi P, et al. (2008). Dephosphorylation by calcineurin regulates translocation of Drp1 to mitochondria. Proc Natl Acad Sci U S A 105: 15803-15808.
31. Cerveny KL, Jensen RE, (2003). The WD-repeats of Net2p interact with Dnm1p and Fis1p to regulate division of mitochondria. Mol Biol Cell 14: 4126-4139.
32. Chacinska A, Pfannschmidt S, Wiedemann N, Kozjak V, Sanjuan Szklarz LK, Schulze-Specking A, et al. (2004). Essential role of Mia40 in import and assembly of mitochondrial intermembrane space proteins. EMBO J 23: 3735-3746.
33. Chacinska A, Koehler CM, Milenkovic D, Lithgow T, Pfanner N, (2009). Importing mitochondrial proteins: machineries and mechanisms. Cell 138: 628-644.
34. Chada SR, Hollenbeck PJ, (2004). Nerve growth factor signaling regulates motility and docking of axonal mitochondria. Curr Biol 14: 1272-1276.
35. Chan DC, (2012). Fusion and fission: interlinked processes critical for mitochondrial health. Annu Rev Genet 46: 265-287.
36. Chan NC, Salazar AM, Pham AH, Sweredoski MJ, Kolawa NJ, Graham RL, et al. (2011). Broad activation of the ubiquitin-proteasome system by Parkin is critical for mitophagy. Hum Mol Genet 20: 1726-1737.
37. Chang CR, Blackstone C, (2007). Cyclic AMP-dependent protein kinase phosphorylation of Drp1 regulates its GTPase activity and mitochondrial morphology. J Biol Chem 282: 21583-21587.
38. Chaturvedi RK, Adhihetty P, Shukla S, Hennessy T, Calingasan N, Yang L, et al. (2009). Impaired PGC-1alpha function in muscle in Huntington's disease. Hum Mol Genet 18: 3048-3065.
39. 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.
40. Chen H, Chomyn A, Chan DC, (2005). Disruption of fusion results in mitochondrial heterogeneity and dysfunction. J Biol Chem 280: 26185-26192.
41. Chen H, McCaffery JM, Chan DC, (2007). Mitochondrial fusion protects against neurodegeneration in the cerebellum. Cell 130: 548-562.
42. Chen Y, Dorn GW 2nd, (2013). PINK1-phosphorylated mitofusin 2 is a Parkin receptor for culling damaged mitochondria. Science 340: 471-475.
43. Chinta SJ, Andersen JK, (2005). Dopaminergic neurons. Int J Biochem Cell Biol 37: 942-946.
44. Cho DH, Nakamura T, Fang J, Cieplak P, Godzik A, Gu Z, et al. (2009). S-nitrosylation of Drp1 mediates beta-amyloid-related mitochondrial fission and neuronal injury. Science 324: 102-105.
45. Clausen T, Kaiser M, Huber R, Ehrmann M, (2011). HTRA proteases: regulated proteolysis in protein quality control. Nat Rev Mol Cell Biol 12: 152-162.
46. Cohen MM, Leboucher GP, Livnat-Levanon N, Glickman MH, Weissman AM, (2008). Ubiquitin-proteasome-dependent degradation of a mitofusin, a critical regulator of mitochondrial fusion. Mol Biol Cell 19: 2457-2464.
47. Costa V, Giacomello M, Hudec R, Lopreiato R, Ermak G, Lim D, et al. (2010). Mitochondrial fission and cristae disruption increase the response of cell models of Huntington's disease to apoptotic stimuli. EMBO Mol Med 2: 490-503.
48. Cox RT, Spradling AC, (2006). Milton controls the early acquisition of mitochondria by Drosophila oocytes. Development 133: 3371-3377.
49. 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.
50. Cui L, Jeong H, Borovecki F, Parkhurst CN, Tanese N, Krainc D, (2006). Transcriptional repression of PGC-1alpha by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration. Cell 127: 59-69.
51. 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.
52. De Vos KJ, Chapman AL, Tennant ME, Manser C, Tudor EL, Lau KF, et al. (2007). Familial amyotrophic lateral sclerosis-linked SOD1 mutants perturb fast axonal transport to reduce axonal mitochondria content. Hum Mol Genet 16: 2720-2728.
53. Delettre C, Lenaers G, Griffoin JM, Gigarel N, Lorenzo C, Belenguer P, et al. (2000). Nuclear gene OPA1, encoding a mitochondrial dynamin-related protein, is mutated in dominant optic atrophy. Nat Genet 26: 207-210.
54. Delettre C, Griffoin JM, Kaplan J, Dollfus H, Lorenz B, Faivre L, et al. (2001). Mutation spectrum and splicing variants in the OPA1 gene. Hum Genet 109: 584-591.
55. Devi L, Prabhu BM, Galati DF, Avadhani NG, Anandatheerthavarada HK, (2006). Accumulation of amyloid precursor protein in the mitochondrial import channels of human Alzheimer's disease brain is associated with mitochondrial dysfunction. J Neurosci 26: 9057-9068.
56. Di Bella D, Lazzaro F, Brusco A, Plumari M, Battaglia G, Pastore A, et al. (2010). Mutations in the mitochondrial protease gene AFG3L2 cause dominant hereditary ataxia SCA28. Nat Genet 42: 313-321.
57. Dolezal P, Likic V, Tachezy J, Lithgow T, (2006). Evolution of the molecular machines for protein import into mitochondria. Science 313: 314-318.
58. Dudek J, Rehling P, van der Laan M, (2013). Mitochondrial protein import: common principles and physiological networks. Biochim Biophys Acta 1833: 274-285.
59. Duvezin-Caubet S, Koppen M, Wagener J, Zick M, Israel L, Bernacchia A, et al. (2007). OPA1 processing reconstituted in yeast depends on the subunit composition of the m-AAA protease in mitochondria. Mol Biol Cell 18: 3582-3590.
60. Duyckaerts C, Delatour B, Potier MC, (2009). Classification and basic pathology of Alzheimer disease. Acta Neuropathol (Berl) 118: 5-36.
61. Ehses S, Raschke I, Mancuso G, Bernacchia A, Geimer S, Tondera D, et al. (2009). Regulation of OPA1 processing and mitochondrial fusion by m-AAA protease isoenzymes and OMA1. J Cell Biol 187: 1023-1036.
62. Elgass K, Pakay J, Ryan MT, Palmer CS, (2013). Recent advances into the understanding of mitochondrial fission. Biochim Biophys Acta 1833: 150-161.
63. Endo T, Yamano K, (2009). Multiple pathways for mitochondrial protein traffic. Biol Chem 390: 723-730.
64. Escobar-Henriques M, Westermann B, Langer T, (2006). Regulation of mitochondrial fusion by the F-box protein Mdm30 involves proteasome-independent turnover of Fzo1. J Cell Biol 173: 645-650.
65. Exner N, Treske B, Paquet D, Holmstrom K, Schiesling C, Gispert S, et al. (2007). Loss-of-function of human PINK1 results in mitochondrial pathology and can be rescued by parkin. J Neurosci 27: 12413-12418.
66. Ferri A, Cozzolino M, Crosio C, Nencini M, Casciati A, Gralla EB, et al. (2006). Familial ALS-superoxide dismutases associate with mitochondria and shift their redox potentials. Proc Natl Acad Sci U S A 103: 13860-13865.
67. Ferri A, Fiorenzo P, Nencini M, Cozzolino M, Pesaresi MG, Valle C, et al. (2010). Glutaredoxin 2 prevents aggregation of mutant SOD1 in mitochondria and abolishes its toxicity. Hum Mol Genet 19: 4529-4542.
68. Figueroa-Romero C, Iniguez-Lluhi JA, Stadler J, Chang CR, Arnoult D, Keller PJ, et al. (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.
69. Fischer F, Hamann A, Osiewacz HD, (2012). Mitochondrial quality control: an integrated network of pathways. Trends Biochem Sci 37: 284-292.
70. Frank S, Gaume B, Bergmann-Leitner ES, Leitner WW, Robert EG, Catez F, et al. (2001). The role of dynamin-related protein 1, a mediator of mitochondrial fission, in apoptosis. Dev Cell 1: 515-525.
71. Fransson A, Ruusala A, Aspenstrom P, (2003). Atypical Rho GTPases have roles in mitochondrial homeostasis and apoptosis. J Biol Chem 278: 6495-6502.
72. Fransson S, Ruusala A, Aspenstrom P, (2006). The atypical Rho GTPases Miro-1 and Miro-2 have essential roles in mitochondrial trafficking. Biochem Biophys Res Commun 344: 500-510.
73. Frederick RL, Shaw JM, (2007). Moving mitochondria: establishing distribution of an essential organelle. Traffic 8: 1668-1675.
74. Frezza C, Cipolat S, Martins de Brito O, Micaroni M, Beznoussenko GV, Rudka T, et al. (2006). OPA1 controls apoptotic cristae remodeling independently from mitochondrial fusion. Cell 126: 177-189.
75. Fritz S, Weinbach N, Westermann B, (2003). Mdm30 is an F-box protein required for maintenance of fusion-competent mitochondria in yeast. Mol Biol Cell 14: 2303-2313.
76. Gandhi S, Wood-Kaczmar A, Yao Z, Plun-Favreau H, Deas E, Klupsch K, et al. (2009). PINK1-associated Parkinson's disease is caused by neuronal vulnerability to calcium-induced cell death. Mol Cell 33: 627-638.
77. 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.
78. Gebert N, Ryan MT, Pfanner N, Wiedemann N, Stojanovski D, (2011). Mitochondrial protein import machineries and lipids: a functional connection. Biochim Biophys Acta 1808: 1002-1011.
79. Gegg ME, Cooper JM, Chau KY, Rojo M, Schapira AH, Taanman JW, (2010). Mitofusin 1 and mitofusin 2 are ubiquitinated in a PINK1/parkin-dependent manner upon induction of mitophagy. Hum Mol Genet 19: 4861-4870.
80. Glater EE, Megeath LJ, Stowers RS, Schwarz TL, (2006). Axonal transport of mitochondria requires milton to recruit kinesin heavy chain and is light chain independent. J Cell Biol 173: 545-557.
81. Gomes LC, Di Benedetto G, Scorrano L, (2011). During autophagy mitochondria elongate, are spared from degradation and sustain cell viability. Nat Cell Biol 13: 589-598.
82. Griffin EE, Graumann J, Chan DC, (2005). The WD40 protein Caf4p is a component of the mitochondrial fission machinery and recruits Dnm1p to mitochondria. J Cell Biol 170: 237-248.
83. Griparic L, Kanazawa T, van der Bliek AM, (2007). Regulation of the mitochondrial dynamin-like protein Opa1 by proteolytic cleavage. J Cell Biol 178: 757-764.
84. Grundke-Iqbal I, Iqbal K, Quinlan M, Tung YC, Zaidi MS, Wisniewski HM, (1986). Microtubule-associated protein tau. A component of Alzheimer paired helical filaments. J Biol Chem 261: 6084-6089.
85. Gschwind M, Huber G, (1995). Apoptotic cell death induced by beta-amyloid 1-42 peptide is cell type dependent. J Neurochem 65: 292-300.
86. Han XJ, Lu YF, Li SA, Kaitsuka T, Sato Y, Tomizawa K, et al. (2008). CaM kinase I alpha-induced phosphorylation of Drp1 regulates mitochondrial morphology. J Cell Biol 182: 573-585.
87. Hansen JJ, Durr A, Cournu-Rebeix I, Georgopoulos C, Ang D, Nielsen MN, et al. (2002). Hereditary spastic paraplegia SPG13 is associated with a mutation in the gene encoding the mitochondrial chaperonin Hsp60. Am J Hum Genet 70: 1328-1332.
88. Harder Z, Zunino R, McBride H, (2004). Sumo1 conjugates mitochondrial substrates and participates in mitochondrial fission. Curr Biol 14: 340-345.
89. Harding AE, (1993). Hereditary spastic paraplegias. Semin Neurol 13: 333-336.
90. Hawlitschek G, Schneider H, Schmidt B, Tropschug M, Hartl FU, Neupert W, (1988). Mitochondrial protein import: identification of processing peptidase and of PEP, a processing enhancing protein. Cell 53: 795-806.
91. Haynes CM, Ron D, (2010). The mitochondrial UPR-protecting organelle protein homeostasis. J Cell Sci 123 (Pt 22): 3849-3855.
92. Haynes CM, Petrova K, Benedetti C, Yang Y, Ron D, (2007). ClpP mediates activation of a mitochondrial unfolded protein response in C. elegans. Dev Cell 13: 467-480.
93. Haynes CM, Yang Y, Blais SP, Neubert TA, Ron D, (2010). The matrix peptide exporter HAF-1 signals a mitochondrial UPR by activating the transcription factor ZC376.7 in C. elegans. Mol Cell 37: 529-540.
94. Head B, Griparic L, Amiri M, Gandre-Babbe S, van der Bliek AM, (2009). Inducible proteolytic inactivation of OPA1 mediated by the OMA1 protease in mammalian cells. J Cell Biol 187: 959-966.
95. Horn SR, Thomenius MJ, Johnson ES, Freel CD, Wu JQ, Coloff JL, et al. (2011). Regulation of mitochondrial morphology by APC/CCdh1-mediated control of Drp1 stability. Mol Biol Cell 22: 1207-1216.
96. Huntington's Disease Collaborative Research Group (1993). A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. Cell 72: 971-983.
97. Ingerman E, Perkins EM, Marino M, Mears JA, McCaffery JM, Hinshaw JE, et al. (2005). Dnm1 forms spirals that are structurally tailored to fit mitochondria. J Cell Biol 170: 1021-1027.
98. Irrcher I, Aleyasin H, Seifert EL, Hewitt SJ, Chhabra S, Phillips M, et al. (2010). Loss of the Parkinson's disease-linked gene DJ-1 perturbs mitochondrial dynamics. Hum Mol Genet 19: 3734-3746.
99. Ishihara N, Eura Y, Mihara K, (2004). Mitofusin 1 and 2 play distinct roles in mitochondrial fusion reactions via GTPase activity. J Cell Sci 117 (Pt 26): 6535-6546.
100. Ishihara N, Fujita Y, Oka T, Mihara K, (2006). Regulation of mitochondrial morphology through proteolytic cleavage of OPA1. EMBO J 25: 2966-2977.
101. Ishihara N, Nomura M, Jofuku A, Kato H, Suzuki SO, Masuda K, et al. (2009). Mitochondrial fission factor Drp1 is essential for embryonic development and synapse formation in mice. Nat Cell Biol 11: 958-966.
102. 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.
103. Jeon HB, Choi ES, Yoon JH, Hwang JH, Chang JW, Lee EK, et al. (2007). A proteomics approach to identify the ubiquitinated proteins in mouse heart. Biochem Biophys Res Commun 357: 731-736.
104. Jin SM, Lazarou M, Wang C, Kane LA, Narendra DP, Youle RJ, (2010). Mitochondrial membrane potential regulates PINK1 import and proteolytic destabilization by PARL. J Cell Biol 191: 933-942.
105. Jones JM, Datta P, Srinivasula SM, Ji W, Gupta S, Zhang Z, et al. (2003). Loss of Omi mitochondrial protease activity causes the neuromuscular disorder of mnd2 mutant mice. Nature 425: 721-727.
106. Kang JS, Tian JH, Pan PY, Zald P, Li C, Deng C, et al. (2008). Docking of axonal mitochondria by syntaphilin controls their mobility and affects short-term facilitation. Cell 132: 137-148.
107. Kanki T, Wang K, Baba M, Bartholomew CR, Lynch-Day MA, Du Z, et al. (2009). A genomic screen for yeast mutants defective in selective mitochondria autophagy. Mol Biol Cell 20: 4730-4738.
108. Karbowski M, Youle RJ, (2011). Regulating mitochondrial outer membrane proteins by ubiquitination and proteasomal degradation. Curr Opin Cell Biol 23: 476-482.
109. 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.
110. Kaser M, Kambacheld M, Kisters-Woike B, Langer T, (2003). Oma1, a novel membrane-bound metallopeptidase in mitochondria with activities overlapping with the m-AAA protease. J Biol Chem 278: 46414-46423.
111. Khalimonchuk O, Jeong MY, Watts T, Ferris E, Winge DR, (2012). Selective Oma1 protease-mediated proteolysis of Cox1 subunit of cytochrome oxidase in assembly mutants. J Biol Chem 287: 7289-7300.
112. Kim DY, Kim KK, (2005). Structure and function of HtrA family proteins, the key players in protein quality control. J Biochem Mol Biol 38: 266-274.
113. Kitada T, Asakawa S, Hattori N, Matsumine H, Yamamura Y, Minoshima S, et al. (1998). Mutations in the parkin gene cause autosomal recessive juvenile parkinsonism. Nature 392: 605-608.
114. Koehler CM, (2000). Protein translocation pathways of the mitochondrion. FEBS Lett 476: 27-31.
115. Koeppen AH, (2005). The pathogenesis of spinocerebellar ataxia. Cerebellum 4: 62-73.
116. Koirala S, Bui HT, Schubert HL, Eckert DM, Hill CP, Kay MS, et al. (2010). Molecular architecture of a dynamin adaptor: implications for assembly of mitochondrial fission complexes. J Cell Biol 191: 1127-1139.
117. Koirala S, Guo Q, Kalia R, Bui HT, Eckert DM, Frost A, et al. (2013). Interchangeable adaptors regulate mitochondrial dynamin assembly for membrane scission. Proc Natl Acad Sci U S A 110: E1342-E1351.
118. Koppen M, Metodiev MD, Casari G, Rugarli EI, Langer T, (2007). Variable and tissue-specific subunit composition of mitochondrial m-AAA protease complexes linked to hereditary spastic paraplegia. Mol Cell Biol 27: 758-767.
119. Korbel D, Wurth S, Kaser M, Langer T, (2004). Membrane protein turnover by the m-AAA protease in mitochondria depends on the transmembrane domains of its subunits. EMBO Rep 5: 698-703.
120. 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.
121. Koutsopoulos OS, Laine D, Osellame L, Chudakov DM, Parton RG, Frazier AE, et al. (2010). Human Miltons associate with mitochondria and induce microtubule-dependent remodeling of mitochondrial networks. Biochim Biophys Acta 1803: 564-574.
122. Kruger R, Sharma M, Riess O, Gasser T, Van Broeckhoven C, Theuns J, et al. (2011). A large-scale genetic association study to evaluate the contribution of Omi/HtrA2 (PARK13) to Parkinson's disease. Neurobiol Aging 32: 548.e9-548.e18.
123. Lackner LL, Horner JS, Nunnari J, (2009). Mechanistic analysis of a dynamin effector. Science 325: 874-877.
124. Lee CW, Peng HB, (2008). The function of mitochondria in presynaptic development at the neuromuscular junction. Mol Biol Cell 19: 150-158.
125. 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.
126. Legesse-Miller A, Massol RH, Kirchhausen T, (2003). Constriction and Dnm1p recruitment are distinct processes in mitochondrial fission. Mol Biol Cell 14: 1953-1963.
127. Leonhard K, Herrmann JM, Stuart RA, Mannhaupt G, Neupert W, Langer T, (1996). AAA proteases with catalytic sites on opposite membrane surfaces comprise a proteolytic system for the ATP-dependent degradation of inner membrane proteins in mitochondria. EMBO J 15: 4218-4229.
128. Li Q, Vande Velde C, Israelson A, Xie J, Bailey AO, Dong MQ, et al. (2010). ALS-linked mutant superoxide dismutase 1 (SOD1) alters mitochondrial protein composition and decreases protein import. Proc Natl Acad Sci U S A 107: 21146-21151.
129. Li W, Bengtson MH, Ulbrich A, Matsuda A, Reddy VA, Orth A, et al. (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.
130. Li Z, Okamoto K, Hayashi Y, Sheng M, (2004). The importance of dendritic mitochondria in the morphogenesis and plasticity of spines and synapses. Cell 119: 873-887.
131. Lin CH, Chen ML, Chen GS, Tai CH, Wu RM, (2011). Novel variant Pro143Ala in HTRA2 contributes to Parkinson's disease by inducing hyperphosphorylation of HTRA2 protein in mitochondria. Hum Genet 130: 817-827.
132. Livnat-Levanon N, Glickman MH, (2011). Ubiquitin-proteasome system and mitochondria-reciprocity. Biochim Biophys Acta 1809: 80-87.
133. Loson OC, Song Z, Chen H, Chan DC, (2013). Fis1, Mff, MiD49, and MiD51 mediate Drp1 recruitment in mitochondrial fission. Mol Biol Cell 24: 659-667.
134. Magen D, Georgopoulos C, Bross P, Ang D, Segev Y, Goldsher D, et al. (2008). Mitochondrial hsp60 chaperonopathy causes an autosomal-recessive neurodegenerative disorder linked to brain hypomyelination and leukodystrophy. Am J Hum Genet 83: 30-42.
135. Maiuri MC, Zalckvar E, Kimchi A, Kroemer G, (2007). Self-eating and self-killing: crosstalk between autophagy and apoptosis. Nat Rev Mol Cell Biol 8: 741-752.
136. Manczak M, Reddy PH, (2012). Abnormal interaction between the mitochondrial fission protein Drp1 and hyperphosphorylated tau in Alzheimer's disease neurons: implications for mitochondrial dysfunction and neuronal damage. Hum Mol Genet 21: 2538-2547.
137. Manczak M, Anekonda TS, Henson E, Park BS, Quinn J, Reddy PH, (2006). Mitochondria are a direct site of A beta accumulation in Alzheimer's disease neurons: implications for free radical generation and oxidative damage in disease progression. Hum Mol Genet 15: 1437-1449.
138. Manczak M, Calkins MJ, Reddy PH, (2011). Impaired mitochondrial dynamics and abnormal interaction of amyloid beta with mitochondrial protein Drp1 in neurons from patients with Alzheimer's disease: implications for neuronal damage. Hum Mol Genet 20: 2495-2509.
139. Margineantu DH, Emerson CB, Diaz D, Hockenbery DM, (2007). Hsp90 inhibition decreases mitochondrial protein turnover. PLoS One 2: e1066.
140. Martin JB, Gusella JF, (1986). Huntington's disease. Pathogenesis and management. N Engl J Med 315: 1267-1276.
141. Martin LJ, Gertz B, Pan Y, Price AC, Molkentin JD, Chang Q, (2009). The mitochondrial permeability transition pore in motor neurons: involvement in the pathobiology of ALS mice. Exp Neurol 218: 333-346.
142. Martins LM, Morrison A, Klupsch K, Fedele V, Moisoi N, Teismann P, et al. (2004). Neuroprotective role of the Reaper-related serine protease HtrA2/Omi revealed by targeted deletion in mice. Mol Cell Biol 24: 9848-9862.
143. Mears JA, Lackner LL, Fang S, Ingerman E, Nunnari J, Hinshaw JE, (2011). Conformational changes in Dnm1 support a contractile mechanism for mitochondrial fission. Nat Struct Mol Biol 18: 20-26.
144. Meeusen S, McCaffery JM, Nunnari J, (2004). Mitochondrial fusion intermediates revealed in vitro. Science 305: 1747-1752.
145. Miller KE, Sheetz MP, (2004). Axonal mitochondrial transport and potential are correlated. J Cell Sci 117 (Pt 13): 2791-2804.
146. Mokranjac D, Neupert W, (2010). The many faces of the mitochondrial TIM23 complex. Biochim Biophys Acta 1797: 1045-1054.
147. Montessuit S, Somasekharan SP, Terrones O, Lucken-Ardjomande S, Herzig S, Schwarzenbacher R, et al. (2010). Membrane remodeling induced by the dynamin-related protein Drp1 stimulates Bax oligomerization. Cell 142: 889-901.
148. Mozdy AD, McCaffery JM, Shaw JM, (2000). Dnm1p GTPase-mediated mitochondrial fission is a multi-step process requiring the novel integral membrane component Fis1p. J Cell Biol 151: 367-380.
149. 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.
150. Nakamura T, Cieplak P, Cho DH, Godzik A, Lipton SA, (2010). S-nitrosylation of Drp1 links excessive mitochondrial fission to neuronal injury in neurodegeneration. Mitochondrion 10: 573-578.
151. Nangaku M, Sato-Yoshitake R, Okada Y, Noda Y, Takemura R, Yamazaki H, et al. (1994). KIF1B, a novel microtubule plus end-directed monomeric motor protein for transport of mitochondria. Cell 79: 1209-1220.
152. Naoe M, Ohwa Y, Ishikawa D, Ohshima C, Nishikawa S, Yamamoto H, et al. (2004). Identification of Tim40 that mediates protein sorting to the mitochondrial intermembrane space. J Biol Chem 279: 47815-47821.
153. Narendra D, Tanaka A, Suen DF, Youle RJ, (2008). Parkin is recruited selectively to impaired mitochondria and promotes their autophagy. J Cell Biol 183: 795-803.
154. Narendra D, Kane LA, Hauser DN, Fearnley IM, Youle RJ, (2010b). p62/SQSTM1 is required for Parkin-induced mitochondrial clustering but not mitophagy; VDAC1 is dispensable for both. Autophagy 6: 1090-1106.
155. Narendra DP, Jin SM, Tanaka A, Suen DF, Gautier CA, Shen J, et al. (2010a). PINK1 is selectively stabilized on impaired mitochondria to activate Parkin. PLoS Biol 8: e1000298.
156. Neupert W, Herrmann JM, (2007). Translocation of proteins into mitochondria. Annu Rev Biochem 76: 723-749.
157. Nolden M, Ehses S, Koppen M, Bernacchia A, Rugarli EI, Langer T, (2005). The m-AAA protease defective in hereditary spastic paraplegia controls ribosome assembly in mitochondria. Cell 123: 277-289.
158. Nowikovsky K, Reipert S, Devenish RJ, Schweyen RJ, (2007). Mdm38 protein depletion causes loss of mitochondrial K+/H+ exchange activity, osmotic swelling and mitophagy. Cell Death Differ 14: 1647-1656.
159. Ohno N, Kidd GJ, Mahad D, Kiryu-Seo S, Avishai A, Komuro H, et al. (2011). Myelination and axonal electrical activity modulate the distribution and motility of mitochondria at CNS nodes of Ranvier. J Neurosci 31: 7249-7258.
160. Okamoto K, Shaw JM, (2005). Mitochondrial morphology and dynamics in yeast and multicellular eukaryotes. Annu Rev Genet 39: 503-536.
161. Okamoto K, Kondo-Okamoto N, Ohsumi Y, (2009). Mitochondria-anchored receptor Atg32 mediates degradation of mitochondria via selective autophagy. Dev Cell 17: 87-97.
162. Olichon A, Baricault L, Gas N, Guillou E, Valette A, Belenguer P, et al. (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.
163. Osellame LD, Blacker TS, Duchen MR, (2012). Cellular and molecular mechanisms of mitochondrial function. Best Pract Res Clin Endocrinol Metab 26: 711-723.
164. Osman C, Wilmes C, Tatsuta T, Langer T, (2007). Prohibitins interact genetically with Atp23, a novel processing peptidase and chaperone for the F1Fo-ATP synthase. Mol Biol Cell 18: 627-635.
165. Otera H, Wang C, Cleland MM, Setoguchi K, Yokota S, Youle RJ, et al. (2010). Mff is an essential factor for mitochondrial recruitment of Drp1 during mitochondrial fission in mammalian cells. J Cell Biol 191: 1141-1158.
166. Otsuga D, Keegan BR, Brisch E, Thatcher JW, Hermann GJ, Bleazard W, et al. (1998). The dynamin-related GTPase, Dnm1p, controls mitochondrial morphology in yeast. J Cell Biol 143: 333-349.
167. Pagliarini DJ, Calvo SE, Chang B, Sheth SA, Vafai SB, Ong SE, et al. (2008). A mitochondrial protein compendium elucidates complex I disease biology. Cell 134: 112-123.
168. Palmer CS, Osellame LD, Laine D, Koutsopoulos OS, Frazier AE, Ryan MT, (2011). MiD49 and MiD51, new components of the mitochondrial fission machinery. EMBO Rep 12: 565-573.
169. Pasinelli P, Houseweart MK, Brown RH Jr, Cleveland DW, (2000). Caspase-1 and -3 are sequentially activated in motor neuron death in Cu,Zn superoxide dismutase-mediated familial amyotrophic lateral sclerosis. Proc Natl Acad Sci U S A 97: 13901-13906.
170. Pasinelli P, Belford ME, Lennon N, Bacskai BJ, Hyman BT, Trotti D, et al. (2004). Amyotrophic lateral sclerosis-associated SOD1 mutant proteins bind and aggregate with Bcl-2 in spinal cord mitochondria. Neuron 43: 19-30.
171. Peng J, Schwartz D, Elias JE, Thoreen CC, Cheng D, Marsischky G, et al. (2003). A proteomics approach to understanding protein ubiquitination. Nat Biotechnol 21: 921-926.
172. Pilling AD, Horiuchi D, Lively CM, Saxton WM, (2006). Kinesin-1 and Dynein are the primary motors for fast transport of mitochondria in Drosophila motor axons. Mol Biol Cell 17: 2057-2068.
173. Poole AC, Thomas RE, Andrews LA, McBride HM, Whitworth AJ, Pallanck LJ, (2008). The PINK1/Parkin pathway regulates mitochondrial morphology. Proc Natl Acad Sci U S A 105: 1638-1643.
174. Potting C, Wilmes C, Engmann T, Osman C, Langer T, (2010). Regulation of mitochondrial phospholipids by Ups1/PRELI-like proteins depends on proteolysis and Mdm35. EMBO J 29: 2888-2898.
175. Prokisch H, Scharfe C, Camp DG 2nd, Xiao W, David L, Andreoli C, et al. (2004). Integrative analysis of the mitochondrial proteome in yeast. PLoS Biol 2: e160.
176. Quiros PM, Ramsay AJ, Sala D, Fernandez-Vizarra E, Rodriguez F, Peinado JR, et al. (2012). Loss of mitochondrial protease OMA1 alters processing of the GTPase OPA1 and causes obesity and defective thermogenesis in mice. EMBO J 31: 2117-2133.
177. Radke S, Chander H, Schafer P, Meiss G, Kruger R, Schulz JB, et al.
178. Reddy PH, Mao P, Manczak M, (2009). Mitochondrial structural and functional dynamics in Huntington's disease. Brain Res Rev 61: 33-48.
179. Rehling P, Brandner K, Pfanner N, (2004). Mitochondrial import and the twin-pore translocase. Nat Rev Mol Cell Biol 5: 519-530.
180. Reid E, (1997). Pure hereditary spastic paraplegia. J Med Genet 34: 499-503.
181. Riemer J, Fischer M, Herrmann JM, (2011). Oxidation-driven protein import into mitochondria: insights and blind spots. Biochim Biophys Acta 1808: 981-989.
182. Rodriguez-Enriquez S, Kim I, Currin RT, Lemasters JJ, (2006). Tracker dyes to probe mitochondrial autophagy (mitophagy) in rat hepatocytes. Autophagy 2: 39-46.
183. Rowland LP, (1984). Looking for the cause of amyotrophic lateral sclerosis. N Engl J Med 311: 979-981.
184. Russo GJ, Louie K, Wellington A, Macleod GT, Hu F, Panchumarthi S, et al. (2009). Drosophila Miro is required for both anterograde and retrograde axonal mitochondrial transport. J Neurosci 29: 5443-5455.
185. Saotome M, Safiulina D, Szabadkai G, Das S, Fransson A, Aspenstrom P, et al. (2008). Bidirectional Ca2+-dependent control of mitochondrial dynamics by the Miro GTPase. Proc Natl Acad Sci U S A 105: 20728-20733.
186. Sarraf SA, Raman M, Guarani-Pereira V, Sowa ME, Huttlin EL, Gygi SP, et al. (2013). Landscape of the PARKIN-dependent ubiquitylome in response to mitochondrial depolarization. Nature 496: 372-376.
187. Schmitt M, Neupert W, Langer T, (1996). The molecular chaperone Hsp78 confers compartment-specific thermotolerance to mitochondria. J Cell Biol 134: 1375-1386.
188. Schols L, Bauer P, Schmidt T, Schulte T, Riess O, (2004). Autosomal dominant cerebellar ataxias: clinical features, genetics, and pathogenesis. Lancet Neurol 3: 291-304.
189. Sesaki H, Jensen RE, (2001). UGO1 encodes an outer membrane protein required for mitochondrial fusion. J Cell Biol 152: 1123-1134.
190. Sesaki H, Southard SM, Yaffe MP, Jensen RE, (2003). Mgm1p, a dynamin-related GTPase, is essential for fusion of the mitochondrial outer membrane. Mol Biol Cell 14: 2342-2356.
191. Shen T, Zheng M, Cao C, Chen C, Tang J, Zhang W, et al. (2007). Mitofusin-2 is a major determinant of oxidative stress-mediated heart muscle cell apoptosis. J Biol Chem 282: 23354-23361.
192. Shi G, Lee JR, Grimes DA, Racacho L, Ye D, Yang H, et al. (2011). Functional alteration of PARL contributes to mitochondrial dysregulation in Parkinson's disease. Hum Mol Genet 20: 1966-1974.
193. Shirendeb U, Reddy AP, Manczak M, Calkins MJ, Mao P, Tagle DA, et al. (2011). Abnormal mitochondrial dynamics, mitochondrial loss and mutant huntingtin oligomers in Huntington's disease: implications for selective neuronal damage. Hum Mol Genet 20: 1438-1455.
194. Sickmann A, Reinders J, Wagner Y, Joppich C, Zahedi R, Meyer HE, et al. (2003). The proteome of Saccharomyces cerevisiae mitochondria. Proc Natl Acad Sci U S A 100: 13207-13212.
195. Simon-Sanchez J, Singleton AB, (2008). Sequencing analysis of OMI/HTRA2 shows previously reported pathogenic mutations in neurologically normal controls. Hum Mol Genet 17: 1988-1993.
196. 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.
197. Song W, Chen J, Petrilli A, Liot G, Klinglmayr E, Zhou Y, et al. (2011). Mutant huntingtin binds the mitochondrial fission GTPase dynamin-related protein-1 and increases its enzymatic activity. Nat Med 17: 377-382.
198. Song Z, Chen H, Fiket M, Alexander C, Chan DC, (2007). OPA1 processing controls mitochondrial fusion and is regulated by mRNA splicing, membrane potential, and Yme1L. J Cell Biol 178: 749-755.
199. Song Z, Ghochani M, McCaffery JM, Frey TG, Chan DC, (2009). Mitofusins and OPA1 mediate sequential steps in mitochondrial membrane fusion. Mol Biol Cell 20: 3525-3532.
200. Spratt DE, Martinez-Torres RJ, Noh YJ, Mercier P, Manczyk N, Barber KR, et al. (2013). A molecular explanation for the recessive nature of parkin-linked Parkinson's disease. Nat Commun 4: 1983.
201. van Spronsen M, Mikhaylova M, Lipka J, Schlager MA, van den Heuvel DJ, Kuijpers M, et al. (2013). TRAK/Milton motor-adaptor proteins steer mitochondrial trafficking to axons and dendrites. Neuron 77: 485-502.
202. Sterky FH, Lee S, Wibom R, Olson L, Larsson NG, (2011). Impaired mitochondrial transport and Parkin-independent degeneration of respiratory chain-deficient dopamine neurons in vivo. Proc Natl Acad Sci U S A 108: 12937-12942.
203. Stiburek L, Cesnekova J, Kostkova O, Fornuskova D, Vinsova K, Wenchich L, et al. (2012). YME1L controls the accumulation of respiratory chain subunits and is required for apoptotic resistance, cristae morphogenesis, and cell proliferation. Mol Biol Cell 23: 1010-1023.
204. Stojanovski D, Koutsopoulos OS, Okamoto K, Ryan MT, (2004). Levels of human Fis1 at the mitochondrial outer membrane regulate mitochondrial morphology. J Cell Sci 117 (Pt 7): 1201-1210.
205. Stojanovski D, Bragoszewski P, Chacinska A, (2012). The MIA pathway: a tight bond between protein transport and oxidative folding in mitochondria. Biochim Biophys Acta 1823: 1142-1150.
206. Strauss KM, Martins LM, Plun-Favreau H, Marx FP, Kautzmann S, Berg D, et al. (2005). Loss of function mutations in the gene encoding Omi/HtrA2 in Parkinson's disease. Hum Mol Genet 14: 2099-2111.
207. Suen DF, Norris KL, Youle RJ, (2008). Mitochondrial dynamics and apoptosis. Genes Dev 22: 1577-1590.
208. Sugioka R, Shimizu S, Tsujimoto Y, (2004). Fzo1, a protein involved in mitochondrial fusion, inhibits apoptosis. J Biol Chem 279: 52726-52734.
209. Sugiura A, Nagashima S, Tokuyama T, Amo T, Matsuki Y, Ishido S, et al. (2013). MITOL Regulates Endoplasmic Reticulum-Mitochondria Contacts via Mitofusin2. Mol Cell 51: 20-34.
210. Suzuki Y, Takahashi-Niki K, Akagi T, Hashikawa T, Takahashi R, (2004). Mitochondrial protease Omi/HtrA2 enhances caspase activation through multiple pathways. Cell Death Differ 11: 208-216.
211. 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.
212. Tanaka A, Cleland MM, Xu S, Narendra DP, Suen DF, Karbowski M, et al. (2010). Proteasome and p97 mediate mitophagy and degradation of mitofusins induced by Parkin. J Cell Biol 191: 1367-1380.
213. Tanaka K, Sugiura Y, Ichishita R, Mihara K, Oka T, (2011). KLP6: a newly identified kinesin that regulates the morphology and transport of mitochondria in neuronal cells. J Cell Sci 124 (Pt 14): 2457-2465.
214. Tanaka Y, Kanai Y, Okada Y, Nonaka S, Takeda S, Harada A, et al. (1998). Targeted disruption of mouse conventional kinesin heavy chain, kif5B, results in abnormal perinuclear clustering of mitochondria. Cell 93: 1147-1158.
215. Tatsuta T, Langer T, (2007). Studying proteolysis within mitochondria. Methods Mol Biol 372: 343-360.
216. Tatsuta T, Langer T, (2009). AAA proteases in mitochondria: diverse functions of membrane-bound proteolytic machines. Res Microbiol 160: 711-717.
217. Taylor EB, Rutter J, (2011). Mitochondrial quality control by the ubiquitin-proteasome system. Biochem Soc Trans 39: 1509-1513.
218. Terziyska N, Lutz T, Kozany C, Mokranjac D, Mesecke N, Neupert W, et al. (2005). Mia40, a novel factor for protein import into the intermembrane space of mitochondria is able to bind metal ions. FEBS Lett 579: 179-184.
219. Tieu Q, Okreglak V, Naylor K, Nunnari J, (2002). The WD repeat protein, Mdv1p, functions as a molecular adaptor by interacting with Dnm1p and Fis1p during mitochondrial fission. J Cell Biol 158: 445-452.
220. Tondera D, Grandemange S, Jourdain A, Karbowski M, Mattenberger Y, Herzig S, et al. (2009). SLP-2 is required for stress-induced mitochondrial hyperfusion. EMBO J 28: 1589-1600.
221. Twig G, Elorza A, Molina AJ, Mohamed H, Wikstrom JD, Walzer G, et al. (2008). Fission and selective fusion govern mitochondrial segregation and elimination by autophagy. EMBO J 27: 433-446.
222. Vale RD, (2003). The molecular motor toolbox for intracellular transport. Cell 112: 467-480.
223. Valente EM, Abou-Sleiman PM, Caputo V, Muqit MM, Harvey K, Gispert S, et al. (2004). Hereditary early-onset Parkinson's disease caused by mutations in PINK1. Science 304: 1158-1160.
224. Vande Walle L, Lamkanfi M, Vandenabeele P, (2008). The mitochondrial serine protease HtrA2/Omi: an overview. Cell Death Differ 15: 453-460.
225. Varabyova A, Stojanovski D, Chacinska A, (2013). Mitochondrial protein homeostasis. IUBMB Life 65: 191-201.
226. Verburg J, Hollenbeck PJ, (2008). Mitochondrial membrane potential in axons increases with local nerve growth factor or semaphorin signaling. J Neurosci 28: 8306-8315.
227. Verstreken P, Ly CV, Venken KJ, Koh TW, Zhou Y, Bellen HJ, (2005). Synaptic mitochondria are critical for mobilization of reserve pool vesicles at Drosophila neuromuscular junctions. Neuron 47: 365-378.
228. Vogtle FN, Wortelkamp S, Zahedi RP, Becker D, Leidhold C, Gevaert K, et al. (2009). Global analysis of the mitochondrial N-proteome identifies a processing peptidase critical for protein stability. Cell 139: 428-439.
229. Vogtle FN, Prinz C, Kellermann J, Lottspeich F, Pfanner N, Meisinger C, (2011). Mitochondrial protein turnover: role of the precursor intermediate peptidase Oct1 in protein stabilization. Mol Biol Cell 22: 2135-2143.
230. Voos W, (2013). Chaperone-protease networks in mitochondrial protein homeostasis. Biochim Biophys Acta 1833: 388-399.
231. Voos W, Ward LA, Truscott KN, (2013). The role of AAA+ proteases in mitochondrial protein biogenesis, homeostasis and activity control. Subcell Biochem 66: 223-263.
232. Vos M, Esposito G, Edirisinghe JN, Vilain S, Haddad DM, Slabbaert JR, et al. (2012). Vitamin K2 is a mitochondrial electron carrier that rescues pink1 deficiency. Science 336: 1306-1310.
233. Wakabayashi J, Zhang Z, Wakabayashi N, Tamura Y, Fukaya M, Kensler TW, et al. (2009). The dynamin-related GTPase Drp1 is required for embryonic and brain development in mice. J Cell Biol 186: 805-816.
234. Wang CY, Xu Q, Weng L, Zhang Q, Zhang HN, Guo JF, et al. (2011a). Genetic variations of Omi/HTRA2 in Chinese patients with Parkinson's disease. Brain Res 1385: 293-297.
235. Wang H, Song P, Du L, Tian W, Yue W, Liu M, et al. (2011b). Parkin ubiquitinates Drp1 for proteasome-dependent degradation: implication of dysregulated mitochondrial dynamics in Parkinson disease. J Biol Chem 286: 11649-11658.
236. Wang X, Su B, Siedlak SL, Moreira PI, Fujioka H, Wang Y, et al. (2008). Amyloid-beta overproduction causes abnormal mitochondrial dynamics via differential modulation of mitochondrial fission/fusion proteins. Proc Natl Acad Sci U S A 105: 19318-19323.
237. Wang X, Su B, Lee HG, Li X, Perry G, Smith MA, et al. (2009). Impaired balance of mitochondrial fission and fusion in Alzheimer's disease. J Neurosci 29: 9090-9103.
238. 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.
239. 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.
240. Weckbecker D, Longen S, Riemer J, Herrmann JM, (2012). Atp23 biogenesis reveals a chaperone-like folding activity of Mia40 in the IMS of mitochondria. EMBO J 31: 4348-4358.
241. Westermann B, (2010). Mitochondrial dynamics in model organisms: what yeasts, worms and flies have taught us about fusion and fission of mitochondria. Semin Cell Dev Biol 21: 542-549.
242. Wong ED, Wagner JA, Gorsich SW, McCaffery JM, Shaw JM, Nunnari J, (2000). The dynamin-related GTPase, Mgm1p, is an intermembrane space protein required for maintenance of fusion competent mitochondria. J Cell Biol 151: 341-352.
243. Wong ED, Wagner JA, Scott SV, Okreglak V, Holewinske TJ, Cassidy-Stone A, et al. (2003). The intramitochondrial dynamin-related GTPase, Mgm1p, is a component of a protein complex that mediates mitochondrial fusion. J Cell Biol 160: 303-311.
244. Xue L, Fletcher GC, Tolkovsky AM, (2001). Mitochondria are selectively eliminated from eukaryotic cells after blockade of caspases during apoptosis. Curr Biol 11: 361-365.
245. Yaffe MP, (1999). Dynamic mitochondria. Nat Cell Biol 1: E149-E150.
246. Yang Y, Ouyang Y, Yang L, Beal MF, McQuibban A, Vogel H, et al. (2008). Pink1 regulates mitochondrial dynamics through interaction with the fission/fusion machinery. Proc Natl Acad Sci U S A 105: 7070-7075.
247. Yonashiro R, Ishido S, Kyo S, Fukuda T, Goto E, Matsuki Y, et al. (2006). A novel mitochondrial ubiquitin ligase plays a critical role in mitochondrial dynamics. EMBO J 25: 3618-3626.
248. 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.
249. Youle RJ, van der Bliek AM, (2012). Mitochondrial fission, fusion, and stress. Science 337: 1062-1065.
250. Youle RJ, Narendra DP, (2011). Mechanisms of mitophagy. Nat Rev Mol Cell Biol 12: 9-14.
251. Youle RJ, Strasser A, (2008). The BCL-2 protein family: opposing activities that mediate cell death. Nat Rev Mol Cell Biol 9: 47-59.
252. Zanna C, Ghelli A, Porcelli AM, Karbowski M, Youle RJ, Schimpf S, et al. (2008). OPA1 mutations associated with dominant optic atrophy impair oxidative phosphorylation and mitochondrial fusion. Brain 131 (Pt 2): 352-367.
253. Zeng X, Neupert W, Tzagoloff A, (2007). The metalloprotease encoded by ATP23 has a dual function in processing and assembly of subunit 6 of mitochondrial ATPase. Mol Biol Cell 18: 617-626.
254. Zhao C, Takita J, Tanaka Y, Setou M, Nakagawa T, Takeda S, et al. (2001). Charcot-Marie-Tooth disease type 2A caused by mutation in a microtubule motor KIF1Bbeta. Cell 105: 587-597.
255. Zhao J, Liu T, Jin S, Wang X, Qu M, Uhlen P, et al. (2011). Human MIEF1 recruits Drp1 to mitochondrial outer membranes and promotes mitochondrial fusion rather than fission. EMBO J 30: 2762-2778.
256. Zhu X, Perry G, Smith MA, Wang X, (2013). Abnormal mitochondrial dynamics in the pathogenesis of Alzheimer's disease. J Alzheimers Dis 33 (Suppl 1): S253-S262.
257. Zuchner S, Mersiyanova IV, Muglia M, Bissar-Tadmouri N, Rochelle J, Dadali EL, et al. (2004). Mutations in the mitochondrial GTPase mitofusin 2 cause Charcot-Marie-Tooth neuropathy type 2A. Nat Genet 36: 449-451.
258. Zuchner S, De Jonghe P, Jordanova A, Claeys KG, Guergueltcheva V, Cherninkova S, et al. (2006). Axonal neuropathy with optic atrophy is caused by mutations in mitofusin 2. Ann Neurol 59: 276-281.
259. Zunino R, Braschi E, Xu L, McBride HM, (2009). Translocation of SenP5 from the nucleoli to the mitochondria modulates DRP1-dependent fission during mitosis. J Biol Chem 284: 17783-17795.