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PMID: 21689593 Published · ppublish English Journal Article Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't Research Support, U.S. Gov't, Non-P.H.S. Review

Mitochondria: the next (neurode)generation.

Neuron ·Vol. 70 ·No. 6 ·2011-06-23 ·Pages 1033-53

Schon EA, Przedborski S

Abstract

Adult-onset neurodegenerative disorders are disabling and often fatal diseases of the nervous system whose underlying mechanisms of cell death remain unknown. Defects in mitochondrial respiration had previously been proposed to contribute to the occurrence of many, if not all, of the most common neurodegenerative disorders. However, the discovery of genes mutated in hereditary forms of these enigmatic diseases has additionally suggested defects in mitochondrial dynamics. Such disturbances can lead to changes in mitochondrial trafficking, in interorganellar communication, and in mitochondrial quality control. These new mechanisms by which mitochondria may also be linked to neurodegeneration will likely have far-reaching implications for our understanding of the pathophysiology and treatment of adult-onset neurodegenerative disorders.

MeSH Terms
Adult Energy Metabolism/genetics,physiology Heredodegenerative Disorders, Nervous System/genetics,metabolism,pathology Humans Middle Aged Mitochondria/genetics,metabolism,pathology Neurodegenerative Diseases/genetics,metabolism,pathology Neurons/metabolism,pathology Protein Transport
Authors & Affiliations
2 authors, click to expand affiliations / ORCID
Schon Eric A
Department of Neurology, Columbia University, New York, NY 10032, USA.
Przedborski Serge
References (156)
156 references, click to expand
  1. The kinase domain of mitochondrial PINK1 faces the cytoplasm.
    Proc Natl Acad Sci U S A. 2008 Aug 19;105(33):12022-7 PMID: 18687899
  2. Mitochondrial transport dynamics in axons and dendrites.
    Results Probl Cell Differ. 2009;48:107-39 PMID: 19582407
  3. Optineurin links myosin VI to the Golgi complex and is involved in Golgi organization and exocytosis.
    J Cell Biol. 2005 Apr 25;169(2):285-95 PMID: 15837803
  4. Mitofusin 2 tethers endoplasmic reticulum to mitochondria.
    Nature. 2008 Dec 4;456(7222):605-10 PMID: 19052620
  5. Chemical inhibition of the mitochondrial division dynamin reveals its role in Bax/Bak-dependent mitochondrial outer membrane permeabilization.
    Dev Cell. 2008 Feb;14(2):193-204 PMID: 18267088
  6. Deletion at ITPR1 underlies ataxia in mice and spinocerebellar ataxia 15 in humans.
    PLoS Genet. 2007 Jun;3(6):e108 PMID: 17590087
  7. Mutant huntingtin binds the mitochondrial fission GTPase dynamin-related protein-1 and increases its enzymatic activity.
    Nat Med. 2011 Mar;17(3):377-82 PMID: 21336284
  8. High levels of mitochondrial DNA deletions in substantia nigra neurons in aging and Parkinson disease.
    Nat Genet. 2006 May;38(5):515-7 PMID: 16604074
  9. Ca2+-dependent regulation of mitochondrial dynamics by the Miro-Milton complex.
    Int J Biochem Cell Biol. 2009 Oct;41(10):1972-6 PMID: 19481172
  10. Maternal inheritance and mitochondrial DNA variants in familial Parkinson's disease.
    BMC Med Genet. 2010 Apr 01;11:53 PMID: 20356410
  11. Moderate expansion of a normally biallelic trinucleotide repeat in spinocerebellar ataxia type 2.
    Nat Genet. 1996 Nov;14(3):269-76 PMID: 8896555
  12. Spastic paraplegia and OXPHOS impairment caused by mutations in paraplegin, a nuclear-encoded mitochondrial metalloprotease.
    Cell. 1998 Jun 12;93(6):973-83 PMID: 9635427
  13. Neuronal calcium signaling, mitochondrial dysfunction, and Alzheimer's disease.
    J Alzheimers Dis. 2010;20 Suppl 2:S487-98 PMID: 20413848
  14. Mutant huntingtin aggregates impair mitochondrial movement and trafficking in cortical neurons.
    Neurobiol Dis. 2006 May;22(2):388-400 PMID: 16473015
  15. Multiple defects in energy metabolism in Alzheimer's disease.
    Curr Drug Targets. 2010 Oct;11(10):1193-206 PMID: 20840064
  16. Mutations in the neurofilament light gene linked to Charcot-Marie-Tooth disease cause defects in transport.
    J Neurochem. 2005 May;93(4):861-74 PMID: 15857389
  17. Mitofusin 1 and mitofusin 2 are ubiquitinated in a PINK1/parkin-dependent manner upon induction of mitophagy.
    Hum Mol Genet. 2010 Dec 15;19(24):4861-70 PMID: 20871098
  18. Inhibition of metabotropic glutamate receptor signaling by the huntingtin-binding protein optineurin.
    J Biol Chem. 2005 Oct 14;280(41):34840-8 PMID: 16091361
  19. Mitochondrial functional alterations in relation to pathophysiology of Huntington's disease.
    J Bioenerg Biomembr. 2010 Jun;42(3):217-26 PMID: 20464463
  20. Nonsense mutations in CABC1/ADCK3 cause progressive cerebellar ataxia and atrophy.
    Mitochondrion. 2010 Aug;10(5):510-5 PMID: 20580948
  21. Calcium-independent phospholipase A2 localizes in and protects mitochondria during apoptotic induction by staurosporine.
    J Biol Chem. 2006 Aug 4;281(31):22275-22288 PMID: 16728389
  22. Axonopathy and transport deficits early in the pathogenesis of Alzheimer's disease.
    Science. 2005 Feb 25;307(5713):1282-8 PMID: 15731448
  23. Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS.
    Nat Neurosci. 2010 Nov;13(11):1396-403 PMID: 20953194
  24. Fis1 and Bap31 bridge the mitochondria-ER interface to establish a platform for apoptosis induction.
    EMBO J. 2011 Feb 2;30(3):556-68 PMID: 21183955
  25. Dynamin-like protein 1 reduction underlies mitochondrial morphology and distribution abnormalities in fibroblasts from sporadic Alzheimer's disease patients.
    Am J Pathol. 2008 Aug;173(2):470-82 PMID: 18599615
  26. MAM: more than just a housekeeper.
    Trends Cell Biol. 2009 Feb;19(2):81-8 PMID: 19144519
  27. Absence of nigral degeneration in aged parkin/DJ-1/PINK1 triple knockout mice.
    J Neurochem. 2009 Nov;111(3):696-702 PMID: 19694908
  28. Association of DJ-1 and parkin mediated by pathogenic DJ-1 mutations and oxidative stress.
    Hum Mol Genet. 2005 Jan 1;14(1):71-84 PMID: 15525661
  29. Ketogenic treatment reduces deleted mitochondrial DNAs in cultured human cells.
    Ann Neurol. 2004 Nov;56(5):662-9 PMID: 15389892
  30. Fission and selective fusion govern mitochondrial segregation and elimination by autophagy.
    EMBO J. 2008 Jan 23;27(2):433-46 PMID: 18200046
  31. Myosin VI is an actin-based motor that moves backwards.
    Nature. 1999 Sep 30;401(6752):505-8 PMID: 10519557
  32. Matrilineal inheritance of complex I dysfunction in a multigenerational Parkinson's disease family.
    Ann Neurol. 1998 Dec;44(6):873-81 PMID: 9851431
  33. Control of mitochondrial motility and distribution by the calcium signal: a homeostatic circuit.
    J Cell Biol. 2004 Nov 22;167(4):661-72 PMID: 15545319
  34. Identification of novel spartin-interactors shows spartin is a multifunctional protein.
    J Neurochem. 2009 Nov;111(4):1022-30 PMID: 19765186
  35. Pink1 forms a multiprotein complex with Miro and Milton, linking Pink1 function to mitochondrial trafficking.
    Biochemistry. 2009 Mar 10;48(9):2045-52 PMID: 19152501
  36. Mitochondrial membrane potential regulates PINK1 import and proteolytic destabilization by PARL.
    J Cell Biol. 2010 Nov 29;191(5):933-42 PMID: 21115803
  37. Effect of spastic paraplegia mutations in KIF5A kinesin on transport activity.
    Hum Mol Genet. 2008 May 1;17(9):1245-52 PMID: 18203753
  38. Mitochondrial translation and beyond: processes implicated in combined oxidative phosphorylation deficiencies.
    J Biomed Biotechnol. 2010;2010:737385 PMID: 20396601
  39. DJ-1 gene deletion reveals that DJ-1 is an atypical peroxiredoxin-like peroxidase.
    Proc Natl Acad Sci U S A. 2007 Sep 11;104(37):14807-12 PMID: 17766438
  40. PGC-1α, a potential therapeutic target for early intervention in Parkinson's disease.
    Sci Transl Med. 2010 Oct 6;2(52):52ra73 PMID: 20926834
  41. Mitochondria supply membranes for autophagosome biogenesis during starvation.
    Cell. 2010 May 14;141(4):656-67 PMID: 20478256
  42. OPA1 processing reconstituted in yeast depends on the subunit composition of the m-AAA protease in mitochondria.
    Mol Biol Cell. 2007 Sep;18(9):3582-90 PMID: 17615298
  43. Mitochondrial loss, dysfunction and altered dynamics in Huntington's disease.
    Hum Mol Genet. 2010 Oct 15;19(20):3919-35 PMID: 20660112
  44. DJ-1 binds to mitochondrial complex I and maintains its activity.
    Biochem Biophys Res Commun. 2009 Dec 18;390(3):667-72 PMID: 19822128
  45. Ganglioside-induced differentiation associated protein 1 is a regulator of the mitochondrial network: new implications for Charcot-Marie-Tooth disease.
    J Cell Biol. 2005 Sep 26;170(7):1067-78 PMID: 16172208
  46. Hereditary spastic paraparesis: disrupted intracellular transport associated with spastin mutation.
    Ann Neurol. 2003 Dec;54(6):748-59 PMID: 14681884
  47. Genetic bases for glaucoma.
    Tohoku J Exp Med. 2010 May;221(1):1-10 PMID: 20431268
  48. Survival function of ERK1/2 as IL-3-activated, staurosporine-resistant Bcl2 kinases.
    Proc Natl Acad Sci U S A. 2000 Feb 15;97(4):1578-83 PMID: 10677502
  49. PARIS (ZNF746) repression of PGC-1α contributes to neurodegeneration in Parkinson's disease.
    Cell. 2011 Mar 4;144(5):689-702 PMID: 21376232
  50. Hereditary spastic paraplegia proteins REEP1, spastin, and atlastin-1 coordinate microtubule interactions with the tubular ER network.
    J Clin Invest. 2010 Apr;120(4):1097-110 PMID: 20200447
  51. Atypical Rho GTPases have roles in mitochondrial homeostasis and apoptosis.
    J Biol Chem. 2003 Feb 21;278(8):6495-502 PMID: 12482879
  52. Point mutations of the p150 subunit of dynactin (DCTN1) gene in ALS.
    Neurology. 2004 Aug 24;63(4):724-6 PMID: 15326253
  53. Direct evidence for axonal transport defects in a novel mouse model of mutant spastin-induced hereditary spastic paraplegia (HSP) and human HSP patients.
    J Neurochem. 2009 Jul;110(1):34-44 PMID: 19453301
  54. A stress-responsive system for mitochondrial protein degradation.
    Mol Cell. 2010 Nov 12;40(3):465-80 PMID: 21070972
  55. Mutant huntingtin impairs axonal trafficking in mammalian neurons in vivo and in vitro.
    Mol Cell Biol. 2004 Sep;24(18):8195-209 PMID: 15340079
  56. A unique mitochondria-associated membrane fraction from rat liver has a high capacity for lipid synthesis and contains pre-Golgi secretory proteins including nascent lipoproteins.
    J Biol Chem. 1994 Nov 4;269(44):27494-502 PMID: 7961664
  57. Presenilins are enriched in endoplasmic reticulum membranes associated with mitochondria.
    Am J Pathol. 2009 Nov;175(5):1810-6 PMID: 19834068
  58. N-terminal mutant huntingtin associates with mitochondria and impairs mitochondrial trafficking.
    J Neurosci. 2008 Mar 12;28(11):2783-92 PMID: 18337408
  59. Parkin ubiquitinates Drp1 for proteasome-dependent degradation: implication of dysregulated mitochondrial dynamics in Parkinson disease.
    J Biol Chem. 2011 Apr 1;286(13):11649-58 PMID: 21292769
  60. Mitochondrial and axonal abnormalities precede disruption of the neurofilament network in a model of charcot-marie-tooth disease type 2E and are prevented by heat shock proteins in a mutant-specific fashion.
    J Neuropathol Exp Neurol. 2009 Jun;68(6):642-52 PMID: 19458545
  61. Charcot-Marie-Tooth disease type 2A caused by mutation in a microtubule motor KIF1Bbeta.
    Cell. 2001 Jun 1;105(5):587-97 PMID: 11389829
  62. During autophagy mitochondria elongate, are spared from degradation and sustain cell viability.
    Nat Cell Biol. 2011 May;13(5):589-98 PMID: 21478857
  63. Mitochondrial DNA deletions are abundant and cause functional impairment in aged human substantia nigra neurons.
    Nat Genet. 2006 May;38(5):518-20 PMID: 16604072
  64. Oxidant stress evoked by pacemaking in dopaminergic neurons is attenuated by DJ-1.
    Nature. 2010 Dec 2;468(7324):696-700 PMID: 21068725
  65. DNA/RNA helicase gene mutations in a form of juvenile amyotrophic lateral sclerosis (ALS4).
    Am J Hum Genet. 2004 Jun;74(6):1128-35 PMID: 15106121
  66. Structures of the atlastin GTPase provide insight into homotypic fusion of endoplasmic reticulum membranes.
    Proc Natl Acad Sci U S A. 2011 Mar 8;108(10):3976-81 PMID: 21368113
  67. Imaging interorganelle contacts and local calcium dynamics at the ER-mitochondrial interface.
    Mol Cell. 2010 Jul 9;39(1):121-32 PMID: 20603080
  68. Spartin activates atrophin-1-interacting protein 4 (AIP4) E3 ubiquitin ligase and promotes ubiquitination of adipophilin on lipid droplets.
    BMC Biol. 2010 May 26;8:72 PMID: 20504295
  69. FIP-2, a coiled-coil protein, links Huntingtin to Rab8 and modulates cellular morphogenesis.
    Curr Biol. 2000 Dec 14-28;10(24):1603-6 PMID: 11137014
  70. Mitochondrial dynamics--fusion, fission, movement, and mitophagy--in neurodegenerative diseases.
    Hum Mol Genet. 2009 Oct 15;18(R2):R169-76 PMID: 19808793
  71. The role of abnormal mitochondrial dynamics in the pathogenesis of Alzheimer's disease.
    J Neurochem. 2009 May;109 Suppl 1:153-9 PMID: 19393022
  72. Mitochondrial import and enzymatic activity of PINK1 mutants associated to recessive parkinsonism.
    Hum Mol Genet. 2005 Nov 15;14(22):3477-92 PMID: 16207731
  73. GRIF-1 and OIP106, members of a novel gene family of coiled-coil domain proteins: association in vivo and in vitro with kinesin.
    J Biol Chem. 2005 Apr 15;280(15):14723-32 PMID: 15644324
  74. PML regulates apoptosis at endoplasmic reticulum by modulating calcium release.
    Science. 2010 Nov 26;330(6008):1247-51 PMID: 21030605
  75. Senataxin, the ortholog of a yeast RNA helicase, is mutant in ataxia-ocular apraxia 2.
    Nat Genet. 2004 Mar;36(3):225-7 PMID: 14770181
  76. The mitochondrial inner membrane protein mitofilin controls cristae morphology.
    Mol Biol Cell. 2005 Mar;16(3):1543-54 PMID: 15647377
  77. Effects of ALS-related SOD1 mutants on dynein- and KIF5-mediated retrograde and anterograde axonal transport.
    Biochim Biophys Acta. 2010 Sep;1802(9):707-16 PMID: 20510358
  78. Charcot-Marie-Tooth disease neurofilament mutations disrupt neurofilament assembly and axonal transport.
    Hum Mol Genet. 2002 Nov 1;11(23):2837-44 PMID: 12393795
  79. Direct membrane association drives mitochondrial fission by the Parkinson disease-associated protein alpha-synuclein.
    J Biol Chem. 2011 Jun 10;286(23):20710-26 PMID: 21489994
  80. A new mtDNA mutation associated with a progressive encephalopathy and cytochrome c oxidase deficiency.
    Neurology. 2000 Apr 25;54(8):1693-6 PMID: 10762520
  81. Axonal transport of mitochondria requires milton to recruit kinesin heavy chain and is light chain independent.
    J Cell Biol. 2006 May 22;173(4):545-57 PMID: 16717129
  82. Mitochondrial matters in Huntington disease.
    J Bioenerg Biomembr. 2010 Jun;42(3):189-91 PMID: 20461451
  83. Loss of m-AAA protease in mitochondria causes complex I deficiency and increased sensitivity to oxidative stress in hereditary spastic paraplegia.
    J Cell Biol. 2003 Nov 24;163(4):777-87 PMID: 14623864
  84. A cold-inducible coactivator of nuclear receptors linked to adaptive thermogenesis.
    Cell. 1998 Mar 20;92(6):829-39 PMID: 9529258
  85. Understanding the molecular mechanisms of Friedreich's ataxia to develop therapeutic approaches.
    Hum Mol Genet. 2010 Apr 15;19(R1):R103-10 PMID: 20413654
  86. In vivo correction of COX deficiency by activation of the AMPK/PGC-1α axis.
    Cell Metab. 2011 Jul 6;14(1):80-90 PMID: 21723506
  87. Exome sequencing reveals VCP mutations as a cause of familial ALS.
    Neuron. 2010 Dec 9;68(5):857-64 PMID: 21145000
  88. Impaired balance of mitochondrial fission and fusion in Alzheimer's disease.
    J Neurosci. 2009 Jul 15;29(28):9090-103 PMID: 19605646
  89. PINK1 cleavage at position A103 by the mitochondrial protease PARL.
    Hum Mol Genet. 2011 Mar 1;20(5):867-79 PMID: 21138942
  90. Drosophila pink1 is required for mitochondrial function and interacts genetically with parkin.
    Nature. 2006 Jun 29;441(7097):1162-6 PMID: 16672981
  91. Altered axonal mitochondrial transport in the pathogenesis of Charcot-Marie-Tooth disease from mitofusin 2 mutations.
    J Neurosci. 2007 Jan 10;27(2):422-30 PMID: 17215403
  92. The hereditary spastic paraplegia protein spartin localises to mitochondria.
    J Neurochem. 2006 Sep;98(6):1908-19 PMID: 16945107
  93. PACS-2 controls endoplasmic reticulum-mitochondria communication and Bid-mediated apoptosis.
    EMBO J. 2005 Feb 23;24(4):717-29 PMID: 15692567
  94. Myosin VI is required for asymmetric segregation of cellular components during C. elegans spermatogenesis.
    Curr Biol. 2000 Nov 30;10(23):1489-96 PMID: 11114515
  95. A new paradigm for MAPK: structural interactions of hERK1 with mitochondria in HeLa cells.
    PLoS One. 2009 Oct 22;4(10):e7541 PMID: 19847302
  96. PINK1/Parkin-mediated mitophagy is dependent on VDAC1 and p62/SQSTM1.
    Nat Cell Biol. 2010 Feb;12(2):119-31 PMID: 20098416
  97. Parkin stabilizes microtubules through strong binding mediated by three independent domains.
    J Biol Chem. 2005 Apr 29;280(17):17154-62 PMID: 15737990
  98. The GTPase dMiro is required for axonal transport of mitochondria to Drosophila synapses.
    Neuron. 2005 Aug 4;47(3):379-93 PMID: 16055062
  99. Leber's Hereditary Optic Neuropathy.
    Curr Treat Options Neurol. 2011 Feb;13(1):109-17 PMID: 21063922
  100. Mitochondrially localized ERK2 regulates mitophagy and autophagic cell stress: implications for Parkinson's disease.
    Autophagy. 2008 Aug;4(6):770-82 PMID: 18594198
  101. Loss of the Parkinson's disease-linked gene DJ-1 perturbs mitochondrial dynamics.
    Hum Mol Genet. 2010 Oct 1;19(19):3734-46 PMID: 20639397
  102. Mutations in the novel mitochondrial protein REEP1 cause hereditary spastic paraplegia type 31.
    Am J Hum Genet. 2006 Aug;79(2):365-9 PMID: 16826527
  103. Prohibitins and the functional compartmentalization of mitochondrial membranes.
    J Cell Sci. 2009 Nov 1;122(Pt 21):3823-30 PMID: 19889967
  104. The cellular and subcellular localization of huntingtin-associated protein 1 (HAP1): comparison with huntingtin in rat and human.
    J Neurosci. 1998 Oct 1;18(19):7674-86 PMID: 9742138
  105. Inhibition of mitochondrial fusion by α-synuclein is rescued by PINK1, Parkin and DJ-1.
    EMBO J. 2010 Oct 20;29(20):3571-89 PMID: 20842103
  106. The pattern and mechanism of mitochondrial transport in axons.
    Front Biosci. 1996 Jul 01;1:d91-102 PMID: 9159217
  107. Mutant huntingtin and mitochondrial dysfunction.
    Trends Neurosci. 2008 Dec;31(12):609-16 PMID: 18951640
  108. Abnormal mitochondrial dynamics, mitochondrial loss and mutant huntingtin oligomers in Huntington's disease: implications for selective neuronal damage.
    Hum Mol Genet. 2011 Apr 1;20(7):1438-55 PMID: 21257639
  109. PINK1 is selectively stabilized on impaired mitochondria to activate Parkin.
    PLoS Biol. 2010 Jan 26;8(1):e1000298 PMID: 20126261
  110. Mitofusin 2 is necessary for transport of axonal mitochondria and interacts with the Miro/Milton complex.
    J Neurosci. 2010 Mar 24;30(12):4232-40 PMID: 20335458
  111. Infantile-onset spinocerebellar ataxia and mitochondrial recessive ataxia syndrome are associated with neuronal complex I defect and mtDNA depletion.
    Hum Mol Genet. 2008 Dec 1;17(23):3822-35 PMID: 18775955
  112. Vps35 mediates vesicle transport between the mitochondria and peroxisomes.
    Curr Biol. 2010 Jul 27;20(14):1310-5 PMID: 20619655
  113. Acyl-coenzyme A: cholesterol acyltransferase modulates the generation of the amyloid beta-peptide.
    Nat Cell Biol. 2001 Oct;3(10):905-12 PMID: 11584272
  114. Mitochondrial respiratory-chain diseases.
    N Engl J Med. 2003 Jun 26;348(26):2656-68 PMID: 12826641
  115. S-nitrosylation of Drp1 mediates beta-amyloid-related mitochondrial fission and neuronal injury.
    Science. 2009 Apr 3;324(5923):102-5 PMID: 19342591
  116. Axonal transport of mitochondria to synapses depends on milton, a novel Drosophila protein.
    Neuron. 2002 Dec 19;36(6):1063-77 PMID: 12495622
  117. Ataxin-2 intermediate-length polyglutamine expansions are associated with increased risk for ALS.
    Nature. 2010 Aug 26;466(7310):1069-75 PMID: 20740007
  118. Rapamycin protects against neuron death in in vitro and in vivo models of Parkinson's disease.
    J Neurosci. 2010 Jan 20;30(3):1166-75 PMID: 20089925
  119. Mitochondrial nucleoids maintain genetic autonomy but allow for functional complementation.
    J Cell Biol. 2008 Jun 30;181(7):1117-28 PMID: 18573913
  120. Mitochondrial bioenergetics and dynamics in Huntington's disease: tripartite synapses and selective striatal degeneration.
    J Bioenerg Biomembr. 2010 Jun;42(3):227-34 PMID: 20454921
  121. Mutations in the mitochondrial protease gene AFG3L2 cause dominant hereditary ataxia SCA28.
    Nat Genet. 2010 Apr;42(4):313-21 PMID: 20208537
  122. Optic atrophy 3 as a protein of the mitochondrial outer membrane induces mitochondrial fragmentation.
    Cell Mol Life Sci. 2010 Aug;67(16):2839-50 PMID: 20372962
  123. Human Miltons associate with mitochondria and induce microtubule-dependent remodeling of mitochondrial networks.
    Biochim Biophys Acta. 2010 May;1803(5):564-74 PMID: 20230862
  124. Altered distributions of Gemini of coiled bodies and mitochondria in motor neurons of TDP-43 transgenic mice.
    Proc Natl Acad Sci U S A. 2010 Sep 14;107(37):16325-30 PMID: 20736350
  125. Assembly of the oxidative phosphorylation system in humans: what we have learned by studying its defects.
    Biochim Biophys Acta. 2009 Jan;1793(1):200-11 PMID: 18620006
  126. p62/SQSTM1 is required for Parkin-induced mitochondrial clustering but not mitophagy; VDAC1 is dispensable for both.
    Autophagy. 2010 Nov;6(8):1090-106 PMID: 20890124
  127. The unfolded protein response is activated in Alzheimer's disease.
    Acta Neuropathol. 2005 Aug;110(2):165-72 PMID: 15973543
  128. Huntingtin and huntingtin-associated protein 1 influence neuronal calcium signaling mediated by inositol-(1,4,5) triphosphate receptor type 1.
    Neuron. 2003 Jul 17;39(2):227-39 PMID: 12873381
  129. The m-AAA protease defective in hereditary spastic paraplegia controls ribosome assembly in mitochondria.
    Cell. 2005 Oct 21;123(2):277-89 PMID: 16239145
  130. The AAA-ATPase p97 is essential for outer mitochondrial membrane protein turnover.
    Mol Biol Cell. 2011 Feb 1;22(3):291-300 PMID: 21118995
  131. Mitochondrial fission and cristae disruption increase the response of cell models of Huntington's disease to apoptotic stimuli.
    EMBO Mol Med. 2010 Dec;2(12):490-503 PMID: 21069748
  132. Presenilin 2 modulates endoplasmic reticulum (ER)-mitochondria interactions and Ca2+ cross-talk.
    Proc Natl Acad Sci U S A. 2011 Feb 15;108(7):2777-82 PMID: 21285369
  133. OPA3, mutated in 3-methylglutaconic aciduria type III, encodes two transcripts targeted primarily to mitochondria.
    Mol Genet Metab. 2010 Jun;100(2):149-54 PMID: 20350831
  134. Mitochondria and the culture of the Borg: understanding the integration of mitochondrial function within the reticulum, the cell, and the organism.
    Bioessays. 2010 Nov;32(11):958-66 PMID: 20824657
  135. GIGYF2 is present in endosomal compartments in the mammalian brains and enhances IGF-1-induced ERK1/2 activation.
    J Neurochem. 2010 Oct;115(2):423-37 PMID: 20670374
  136. Paradigm lost: milton connects kinesin heavy chain to miro on mitochondria.
    J Cell Biol. 2006 May 22;173(4):459-61 PMID: 16717123
  137. Mutations of optineurin in amyotrophic lateral sclerosis.
    Nature. 2010 May 13;465(7295):223-6 PMID: 20428114
  138. GDAP1, the protein causing Charcot-Marie-Tooth disease type 4A, is expressed in neurons and is associated with mitochondria.
    Hum Mol Genet. 2005 Apr 15;14(8):1087-94 PMID: 15772096
  139. Parkinson's disease: mechanisms and models.
    Neuron. 2003 Sep 11;39(6):889-909 PMID: 12971891
  140. Alsin is partially associated with centrosome in human cells.
    Biochim Biophys Acta. 2005 Aug 15;1745(1):84-100 PMID: 16085057
  141. Proteasome and p97 mediate mitophagy and degradation of mitofusins induced by Parkin.
    J Cell Biol. 2010 Dec 27;191(7):1367-80 PMID: 21173115
  142. The spinocerebellar ataxia 12 gene product and protein phosphatase 2A regulatory subunit Bbeta2 antagonizes neuronal survival by promoting mitochondrial fission.
    J Biol Chem. 2008 Dec 26;283(52):36241-8 PMID: 18940801
  143. Neurofilament transport is dependent on actin and myosin.
    J Neurosci. 2004 Oct 27;24(43):9486-96 PMID: 15509735
  144. p62/SQSTM1 cooperates with Parkin for perinuclear clustering of depolarized mitochondria.
    Genes Cells. 2010 Aug;15(8):887-900 PMID: 20604804
  145. Impairment of microtubule-dependent trafficking by overexpression of alpha-synuclein.
    Eur J Neurosci. 2006 Dec;24(11):3153-62 PMID: 17156376
  146. Structural and functional features and significance of the physical linkage between ER and mitochondria.
    J Cell Biol. 2006 Sep 25;174(7):915-21 PMID: 16982799
  147. Leucine-rich repeat kinase 2 phosphorylates brain tubulin-beta isoforms and modulates microtubule stability--a point of convergence in parkinsonian neurodegeneration?
    J Neurochem. 2009 Sep;110(5):1514-22 PMID: 19545277
  148. PINK1-dependent recruitment of Parkin to mitochondria in mitophagy.
    Proc Natl Acad Sci U S A. 2010 Jan 5;107(1):378-83 PMID: 19966284
  149. A 4-base pair deletion in the mitochondrial cytochrome b gene associated with parkinsonism/MELAS overlap syndrome.
    Ann Neurol. 1999 Jan;45(1):130-3 PMID: 9894888
  150. Is Alzheimer's disease a disorder of mitochondria-associated membranes?
    J Alzheimers Dis. 2010;20 Suppl 2:S281-92 PMID: 20421691
  151. Drosophila parkin requires PINK1 for mitochondrial translocation and ubiquitinates mitofusin.
    Proc Natl Acad Sci U S A. 2010 Mar 16;107(11):5018-23 PMID: 20194754
  152. Genetic variation of the mitochondrial complex I subunit NDUFV2 and Parkinson's disease.
    Parkinsonism Relat Disord. 2010 Dec;16(10):686-7 PMID: 20971673
  153. Mitophagy: the latest problem for Parkinson's disease.
    Trends Mol Med. 2011 Mar;17(3):158-65 PMID: 21146459
  154. Familial amyotrophic lateral sclerosis-linked SOD1 mutants perturb fast axonal transport to reduce axonal mitochondria content.
    Hum Mol Genet. 2007 Nov 15;16(22):2720-2728 PMID: 17725983
  155. The interplay between mitochondrial dynamics and mitophagy.
    Antioxid Redox Signal. 2011 May 15;14(10):1939-51 PMID: 21128700
  156. Calcium-independent phospholipase A2 (iPLA2 beta)-mediated ceramide generation plays a key role in the cross-talk between the endoplasmic reticulum (ER) and mitochondria during ER stress-induced insulin-secreting cell apoptosis.
    J Biol Chem. 2008 Dec 12;283(50):34819-32 PMID: 18936091
Article Info
Journal
Neuron
Abbr.
Neuron
ISSN
1097-4199
DOI
10.1016/j.neuron.2011.06.003
Published
2011-06-23
Pages
1033-53
Language
English
Region
United States
NLM ID
8809320
PMCID
PMC3407575
Subset
IM
Grants
NINDS NIH HHS · P50 NS038370 · United States
NICHD NIH HHS · HD32062 · United States
NINDS NIH HHS · NS042269 · United States
NINDS NIH HHS · NS38370 · United States
NINDS NIH HHS · NS070276 · United States
NINDS NIH HHS · RC2 NS070276 · United States
NINDS NIH HHS · R21 NS064191 · United States
NINDS NIH HHS · R01 NS042269 · United States
NINDS NIH HHS · R21 NS072182 · United States
NINDS NIH HHS · NS064191 · United States
NINDS NIH HHS · NS072182 · United States
NICHD NIH HHS · P01 HD032062 · United States
External Links
PubMed source DOI: 10.1016/j.neuron.2011.06.003 PMC Full Text View journal details
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