Mitochondrial fragmentation in neurodegeneration

Mitochondria are remarkably dynamic organelles that migrate, divide and fuse. Cycles of mitochondrial fission and fusion ensure metabolite and mitochondrial DNA mixing and dictate organelle shape, number and bioenergetic functionality. There is mounting evidence that mitochondrial dysfunction is an early and causal event in neurodegeneration. Mutations in the mitochondrial fusion GTPases mitofusin 2 and optic atrophy 1, neurotoxins and oxidative stress all disrupt the cable-like morphology of functional mitochondria. This results in impaired bioenergetics and mitochondrial migration, and can trigger neurodegeneration. These findings suggest potential new treatment avenues for neurodegenerative diseases.

[1]  F. Sanger,et al.  Sequence and organization of the human mitochondrial genome , 1981, Nature.

[2]  J. Bereiter-Hahn,et al.  Dynamics of mitochondria in living cells: Shape changes, dislocations, fusion, and fission of mitochondria , 1994, Microscopy research and technique.

[3]  A. Murray,et al.  Mitochondrial transmission during mating in Saccharomyces cerevisiae is determined by mitochondrial fusion and fission and the intramitochondrial segregation of mitochondrial DNA. , 1997, Molecular biology of the cell.

[4]  M. McNiven,et al.  The dynamins: redundant or distinct functions for an expanding family of related GTPases? , 1997, Proceedings of the National Academy of Sciences of the United States of America.

[5]  David S. Park,et al.  Cyclin Dependent Kinase Inhibitors and Dominant Negative Cyclin Dependent Kinase 4 and 6 Promote Survival of NGF-Deprived Sympathetic Neurons , 1997, The Journal of Neuroscience.

[6]  David S. Park,et al.  Cyclin-dependent Kinases Participate in Death of Neurons Evoked by DNA-damaging Agents , 1998, The Journal of cell biology.

[7]  K. Herrup,et al.  Ectopic Cell Cycle Proteins Predict the Sites of Neuronal Cell Death in Alzheimer’s Disease Brain , 1998, The Journal of Neuroscience.

[8]  J. Thatcher,et al.  Mitochondrial Fusion in Yeast Requires the Transmembrane GTPase Fzo1p , 1998, The Journal of cell biology.

[9]  Sergio Cocozza,et al.  Spastic Paraplegia and OXPHOS Impairment Caused by Mutations in Paraplegin, a Nuclear-Encoded Mitochondrial Metalloprotease , 1998, Cell.

[10]  F. Gage,et al.  Running increases cell proliferation and neurogenesis in the adult mouse dentate gyrus , 1999, Nature Neuroscience.

[11]  C. Moraes,et al.  Mechanisms of human mitochondrial DNA maintenance: the determining role of primary sequence and length over function. , 1999, Molecular biology of the cell.

[12]  R. Jensen,et al.  Division versus Fusion: Dnm1p and Fzo1p Antagonistically Regulate Mitochondrial Shape , 1999, The Journal of cell biology.

[13]  A. Labrousse,et al.  elegans Dynamin-Related Protein DRP-1 Controls Severing of the Mitochondrial Outer , 1999 .

[14]  E. Shoubridge,et al.  Gene shifting: a novel therapy for mitochondrial myopathy. , 1999, Human molecular genetics.

[15]  T J Sejnowski,et al.  Running enhances neurogenesis, learning, and long-term potentiation in mice. , 1999, Proceedings of the National Academy of Sciences of the United States of America.

[16]  A. M. van der Bliek,et al.  C. elegans dynamin-related protein DRP-1 controls severing of the mitochondrial outer membrane. , 1999, Molecular cell.

[17]  J. Ikeda,et al.  Cyclin-dependent kinases as a therapeutic target for stroke. , 2000, Proceedings of the National Academy of Sciences of the United States of America.

[18]  Todd B. Sherer,et al.  Chronic systemic pesticide exposure reproduces features of Parkinson's disease , 2000, Nature Neuroscience.

[19]  J. Saffitz,et al.  Peroxisome proliferator-activated receptor gamma coactivator-1 promotes cardiac mitochondrial biogenesis. , 2000, The Journal of clinical investigation.

[20]  H. Jacobs,et al.  No sex please, we're mitochondria: a hypothesis on the somatic unit of inheritance of mammalian mtDNA. , 2000, BioEssays : news and reviews in molecular, cellular and developmental biology.

[21]  D. Turnbull,et al.  Random intracellular drift explains the clonal expansion of mitochondrial DNA mutations with age. , 2001, American journal of human genetics.

[22]  S. McKiernan,et al.  Mitochondrial DNA deletion mutations are concomitant with ragged red regions of individual, aged muscle fibers: analysis by laser-capture microdissection. , 2001, Nucleic acids research.

[23]  A. M. van der Bliek,et al.  Dynamin-related protein Drp1 is required for mitochondrial division in mammalian cells. , 2001, Molecular biology of the cell.

[24]  A. Nunomura,et al.  Oxidative Damage Is the Earliest Event in Alzheimer Disease , 2001, Journal of neuropathology and experimental neurology.

[25]  D. McCloskey,et al.  Exercise increases metabolic capacity in the motor cortex and striatum, but not in the hippocampus , 2001, Brain Research.

[26]  S. Frank,et al.  The role of dynamin-related protein 1, a mediator of mitochondrial fission, in apoptosis. , 2001, Developmental cell.

[27]  S. Frank,et al.  Spatial and temporal association of Bax with mitochondrial fission sites, Drp1, and Mfn2 during apoptosis , 2002, The Journal of cell biology.

[28]  I. Marín,et al.  The gene encoding ganglioside-induced differentiation-associated protein 1 is mutated in axonal Charcot-Marie-Tooth type 4A disease , 2002, Nature Genetics.

[29]  M. Beal,et al.  Mutated Human SOD1 Causes Dysfunction of Oxidative Phosphorylation in Mitochondria of Transgenic Mice* , 2002, The Journal of Biological Chemistry.

[30]  M. Bayona-Bafaluy,et al.  Human mitochondrial DNA with large deletions repopulates organelles faster than full-length genomes under relaxed copy number control. , 2002, Nucleic acids research.

[31]  G. Lenaers,et al.  The human dynamin‐related protein OPA1 is anchored to the mitochondrial inner membrane facing the inter‐membrane space , 2002, FEBS letters.

[32]  James R. Burke,et al.  Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines , 2002, Nature Neuroscience.

[33]  J. Gilbert,et al.  Ganglioside-induced differentiation-associated protein-1 is mutant in Charcot-Marie-Tooth disease type 4A/8q21 , 2002, Nature Genetics.

[34]  J. Zierath,et al.  Mitofusin-2 Determines Mitochondrial Network Architecture and Mitochondrial Metabolism , 2003, The Journal of Biological Chemistry.

[35]  J. Martinou,et al.  hFis1, a Novel Component of the Mammalian Mitochondrial Fission Machinery* , 2003, Journal of Biological Chemistry.

[36]  Kara L. Cerveny,et al.  The WD-repeats of Net2p interact with Dnm1p and Fis1p to regulate division of mitochondria. , 2003, Molecular biology of the cell.

[37]  D. Hood,et al.  Regulation of Mitochondrial Biogenesis in Muscle by Endurance Exercise , 2003, Sports medicine.

[38]  Erik E. Griffin,et al.  Mitofusins Mfn1 and Mfn2 coordinately regulate mitochondrial fusion and are essential for embryonic development , 2003, The Journal of cell biology.

[39]  Jiandie D. Lin,et al.  Bioenergetic Analysis of Peroxisome Proliferator-activated Receptor γ Coactivators 1α and 1β (PGC-1α and PGC-1β) in Muscle Cells* , 2003, Journal of Biological Chemistry.

[40]  J. Julien,et al.  Cell Cycle Regulators in the Neuronal Death Pathway of Amyotrophic Lateral Sclerosis Caused by Mutant Superoxide Dismutase 1 , 2003, The Journal of Neuroscience.

[41]  A. M. van der Bliek,et al.  Composition and dynamics of human mitochondrial nucleoids. , 2003, Molecular biology of the cell.

[42]  T. Kirchhausen,et al.  Constriction and Dnm1p recruitment are distinct processes in mitochondrial fission. , 2003, Molecular biology of the cell.

[43]  Henriette Pilegaard,et al.  Exercise induces transient transcriptional activation of the PGC‐1α gene in human skeletal muscle , 2003, The Journal of physiology.

[44]  J. Nunnari,et al.  The intramitochondrial dynamin-related GTPase, Mgm1p, is a component of a protein complex that mediates mitochondrial fusion , 2003, The Journal of cell biology.

[45]  M. McNiven,et al.  The Mitochondrial Protein hFis1 Regulates Mitochondrial Fission in Mammalian Cells through an Interaction with the Dynamin-Like Protein DLP1 , 2003, Molecular and Cellular Biology.

[46]  K. Hayasaka,et al.  Mitochondrial GTPase mitofusin 2 mutation in Charcot–Marie–Tooth neuropathy type 2A , 2004, Human Genetics.

[47]  J. McCaffery,et al.  Mitochondrial Fusion Intermediates Revealed in Vitro , 2004, Science.

[48]  R. Scarpulla,et al.  Transcriptional regulatory circuits controlling mitochondrial biogenesis and function. , 2004, Genes & development.

[49]  Robert H. Brown,et al.  Amyotrophic Lateral Sclerosis-Associated SOD1 Mutant Proteins Bind and Aggregate with Bcl-2 in Spinal Cord Mitochondria , 2004, Neuron.

[50]  V. Carelli,et al.  Mitochondrial dysfunction as a cause of optic neuropathies , 2004, Progress in Retinal and Eye Research.

[51]  M. Pericak-Vance,et al.  Mutations in the mitochondrial GTPase mitofusin 2 cause Charcot-Marie-Tooth neuropathy type 2A , 2004, Nature Genetics.

[52]  L. Scorrano,et al.  OPA1 requires mitofusin 1 to promote mitochondrial fusion. , 2004, Proceedings of the National Academy of Sciences of the United States of America.

[53]  David S. Park,et al.  CDKs: taking on a role as mediators of dopaminergic loss in Parkinson's disease. , 2004, Trends in molecular medicine.

[54]  Howard T. Jacobs,et al.  Premature ageing in mice expressing defective mitochondrial DNA polymerase , 2004, Nature.

[55]  K. Mihara,et al.  Mitofusin 1 and 2 play distinct roles in mitochondrial fusion reactions via GTPase activity , 2004, Journal of Cell Science.

[56]  Ole Gredal,et al.  Toxicity of Familial ALS-Linked SOD1 Mutants from Selective Recruitment to Spinal Mitochondria , 2004, Neuron.

[57]  Yasunori Hayashi,et al.  The Importance of Dendritic Mitochondria in the Morphogenesis and Plasticity of Spines and Synapses , 2004, Cell.

[58]  H. McBride,et al.  Sumo1 Conjugates Mitochondrial Substrates and Participates in Mitochondrial Fission , 2004, Current Biology.

[59]  R. Youle,et al.  Roles of the mammalian mitochondrial fission and fusion mediators Fis1, Drp1, and Opa1 in apoptosis. , 2004, Molecular biology of the cell.

[60]  Jeong W. Pak,et al.  Molecular analyses of mtDNA deletion mutations in microdissected skeletal muscle fibers from aged rhesus monkeys , 2004, Aging cell.

[61]  J. McCaffery,et al.  Structural Basis of Mitochondrial Tethering by Mitofusin Complexes , 2004, Science.

[62]  P. Hammerstein,et al.  On the relevance of mitochondrial fusions for the accumulation of mitochondrial deletion mutants: A modelling study , 2005, Aging cell.

[63]  R. Jagasia,et al.  DRP-1-mediated mitochondrial fragmentation during EGL-1-induced cell death in C. elegans , 2005, Nature.

[64]  D. Wallace.  A Mitochondrial Paradigm of Metabolic and Degenerative Diseases, Aging, and Cancer: A Dawn for Evolutionary Medicine , 2005, Annual review of genetics.

[65]  G. Johnson,et al.  Mitochondrial Respiration and ATP Production Are Significantly Impaired in Striatal Cells Expressing Mutant Huntingtin* , 2005, Journal of Biological Chemistry.

[66]  J. McCaffery,et al.  Dnm1 forms spirals that are structurally tailored to fit mitochondria , 2005, The Journal of cell biology.

[67]  C. Masters,et al.  Copper-Dependent Inhibition of Human Cytochrome c Oxidase by a Dimeric Conformer of Amyloid-β1-42 , 2005, The Journal of Neuroscience.

[68]  A. Russell,et al.  Mitofusins 1/2 and ERRα expression are increased in human skeletal muscle after physical exercise , 2005, The Journal of physiology.

[69]  P. Verstreken,et al.  Synaptic Mitochondria Are Critical for Mobilization of Reserve Pool Vesicles at Drosophila Neuromuscular Junctions , 2005, Neuron.

[70]  K. Blennow,et al.  Genetic Association of CDC2 with Cerebrospinal Fluid Tau in Alzheimer’s Disease , 2005, Dementia and Geriatric Cognitive Disorders.

[71]  R. Haller,et al.  Exercise and training in mitochondrial myopathies. , 2005, Medicine and science in sports and exercise.

[72]  Axel Niemann,et al.  Ganglioside-induced differentiation associated protein 1 is a regulator of the mitochondrial network , 2005, The Journal of cell biology.

[73]  M. Tarnopolsky,et al.  Resistance exercise training decreases oxidative damage to DNA and increases cytochrome oxidase activity in older adults , 2005, Experimental Gerontology.

[74]  T. Südhof,et al.  Parkinson-like syndrome induced by continuous MPTP infusion: convergent roles of the ubiquitin-proteasome system and alpha-synuclein. , 2005, Proceedings of the National Academy of Sciences of the United States of America.

[75]  D. Chan,et al.  Disruption of Fusion Results in Mitochondrial Heterogeneity and Dysfunction* , 2005, Journal of Biological Chemistry.

[76]  T. D. Pugh,et al.  Mitochondrial DNA Mutations, Oxidative Stress, and Apoptosis in Mammalian Aging , 2005, Science.

[77]  M. Beal,et al.  Are mitochondria critical in the pathogenesis of Alzheimer's disease? , 2005, Brain Research Reviews.

[78]  Emily M. Coonrod,et al.  The role of Fis1p–Mdv1p interactions in mitochondrial fission complex assembly , 2005, The Journal of cell biology.

[79]  K. Nair,et al.  Decline in skeletal muscle mitochondrial function with aging in humans. , 2005, Proceedings of the National Academy of Sciences of the United States of America.

[80]  J. Quinn,et al.  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. , 2006, Human molecular genetics.

[81]  E. Araki,et al.  Activation of AMP-activated protein kinase reduces hyperglycemia-induced mitochondrial reactive oxygen species production and promotes mitochondrial biogenesis in human umbilical vein endothelial cells. , 2006, Diabetes.

[82]  A. Kochański,et al.  Early onset Charcot-Marie-Tooth disease caused by a homozygous Leu239Phe mutation in the GDAP1 gene. , 2006, Acta myologica : myopathies and cardiomyopathies : official journal of the Mediterranean Society of Myology.

[83]  J. Martinou,et al.  Inhibiting the Mitochondrial Fission Machinery Does Not Prevent Bax/Bak-Dependent Apoptosis , 2006, Molecular and Cellular Biology.

[84]  Sara Cipolat,et al.  OPA1 Controls Apoptotic Cristae Remodeling Independently from Mitochondrial Fusion , 2006, Cell.

[85]  E. Jonas.  BCL-xL regulates synaptic plasticity. , 2006, Molecular interventions.

[86]  M. Beal,et al.  Mitochondrial dysfunction and oxidative stress in neurodegenerative diseases , 2006, Nature.

[87]  J. Löwe,et al.  A bacterial dynamin-like protein , 2006, Nature.

[88]  Rachel M. Devay,et al.  Mitochondrial Inner-Membrane Fusion and Crista Maintenance Requires the Dynamin-Related GTPase Mgm1 , 2006, Cell.

[89]  Erik E. Griffin,et al.  Domain Interactions within Fzo1 Oligomers Are Essential for Mitochondrial Fusion* , 2006, Journal of Biological Chemistry.

[90]  Erik E. Griffin,et al.  Molecular mechanism of mitochondrial membrane fusion. , 2006, Biochimica et biophysica acta.

[91]  D. Chan.  Mitochondrial fusion and fission in mammals. , 2006, Annual review of cell and developmental biology.

[92]  S. Petri,et al.  Neural mitochondrial Ca2+ capacity impairment precedes the onset of motor symptoms in G93A Cu/Zn‐superoxide dismutase mutant mice , 2006, Journal of neurochemistry.

[93]  D. Chan.  Mitochondria: Dynamic Organelles in Disease, Aging, and Development , 2006, Cell.

[94]  K. Sada,et al.  A novel mitochondrial ubiquitin ligase plays a critical role in mitochondrial dynamics , 2006, The EMBO journal.

[95]  S. Dimauro,et al.  Approaches to the treatment of mitochondrial diseases , 2006, Muscle & nerve.

[96]  Jean-Claude Martinou,et al.  Nitric oxide‐induced mitochondrial fission is regulated by dynamin‐related GTPases in neurons , 2006, The EMBO journal.

[97]  L. Scorrano,et al.  The mitochondrial fission protein hFis1 requires the endoplasmic reticulum gateway to induce apoptosis. , 2006, Molecular biology of the cell.

[98]  A. Sastry,et al.  Mitochondria in DRG neurons undergo hyperglycemic mediated injury through Bim, Bax and the fission protein Drp1 , 2006, Neurobiology of Disease.

[99]  Dale E. Bredesen,et al.  Cell death in the nervous system , 2006, Nature.

[100]  N. Nakamura,et al.  MARCH‐V is a novel mitofusin 2‐ and Drp1‐binding protein able to change mitochondrial morphology , 2006, EMBO reports.

[101]  Jiandie D. Lin,et al.  Suppression of Reactive Oxygen Species and Neurodegeneration by the PGC-1 Transcriptional Coactivators , 2006, Cell.

[102]  E. Hirsch,et al.  Involvement of mitochondrial complex II defects in neuronal death produced by N-terminus fragment of mutated huntingtin. , 2006, Molecular biology of the cell.

[103]  R. Youle,et al.  Role of Bax and Bak in mitochondrial morphogenesis , 2006, Nature.

[104]  J. Hinshaw,et al.  Mdv1 Interacts with Assembled Dnm1 to Promote Mitochondrial Division* , 2006, Journal of Biological Chemistry.

[105]  F. Baas,et al.  Axonal neuropathy with optic atrophy is caused by mutations in mitofusin 2 , 2006, Annals of neurology.

[106]  A. Santel.  Get the balance right: mitofusins roles in health and disease. , 2006, Biochimica et biophysica acta.

[107]  Dimitri Krainc,et al.  Transcriptional Repression of PGC-1α by Mutant Huntingtin Leads to Mitochondrial Dysfunction and Neurodegeneration , 2006, Cell.

[108]  I. Reynolds,et al.  Differences in mitochondrial movement and morphology in young and mature primary cortical neurons in culture , 2006, Neuroscience.

[109]  David S. Park,et al.  Calpain-Regulated p35/cdk5 Plays a Central Role in Dopaminergic Neuron Death through Modulation of the Transcription Factor Myocyte Enhancer Factor 2 , 2006, The Journal of Neuroscience.

[110]  J. Estaquier,et al.  Inhibiting Drp1-mediated mitochondrial fission selectively prevents the release of cytochrome c during apoptosis , 2007, Cell Death and Differentiation.

[111]  S. Baudet,et al.  Drp1 Mediates Caspase-Independent Type III Cell Death in Normal and Leukemic Cells , 2007, Molecular and Cellular Biology.

[112]  K. White,et al.  Mitochondrial disruption in Drosophila apoptosis. , 2007, Developmental cell.

[113]  D. Wallace.  Why do we still have a maternally inherited mitochondrial DNA? Insights from evolutionary medicine. , 2007, Annual review of biochemistry.

[114]  Alan Hubbard,et al.  Resistance Exercise Reverses Aging in Human Skeletal Muscle , 2007, PloS one.

[115]  Toshihiko Oka,et al.  Mitotic Phosphorylation of Dynamin-related GTPase Drp1 Participates in Mitochondrial Fission* , 2007, Journal of Biological Chemistry.

[116]  H. McBride,et al.  Bax/Bak promote sumoylation of DRP1 and its stable association with mitochondria during apoptotic cell death , 2007, The Journal of cell biology.

[117]  G. Braus,et al.  Cyclin-dependent kinase 5 is an upstream regulator of mitochondrial fission during neuronal apoptosis , 2007, Cell Death and Differentiation.

[118]  B. Spiegelman,et al.  PGC-1α regulates the neuromuscular junction program and ameliorates Duchenne muscular dystrophy , 2007 .

[119]  D. Chan,et al.  Structural basis for recruitment of mitochondrial fission complexes by Fis1 , 2007, Proceedings of the National Academy of Sciences.

[120]  E. Rugarli,et al.  OPA 1 Processing Reconstituted in Yeast Depends on the Subunit Composition of the mAAA Protease in Mitochondria , 2007 .

[121]  Robert A. Harris,et al.  Tissue heterogeneity of the mammalian mitochondrial proteome. , 2007, American journal of physiology. Cell physiology.

[122]  D. Chan,et al.  New insights into mitochondrial fusion , 2007, FEBS letters.

[123]  R. Youle,et al.  The mitochondrial E3 ubiquitin ligase MARCH5 is required for Drp1 dependent mitochondrial division , 2007, The Journal of cell biology.

[124]  S. Strack,et al.  Reversible phosphorylation of Drp1 by cyclic AMP‐dependent protein kinase and calcineurin regulates mitochondrial fission and cell death , 2007, EMBO reports.

[125]  A. M. van der Bliek,et al.  Regulation of the mitochondrial dynamin-like protein Opa1 by proteolytic cleavage , 2007, The Journal of cell biology.

[126]  A. Pestronk,et al.  Altered Axonal Mitochondrial Transport in the Pathogenesis of Charcot-Marie-Tooth Disease from Mitofusin 2 Mutations , 2007, The Journal of Neuroscience.

[127]  G A Perkins,et al.  Mitochondrial fission is an upstream and required event for bax foci formation in response to nitric oxide in cortical neurons , 2007, Cell Death and Differentiation.

[128]  D. Chan,et al.  OPA1 processing controls mitochondrial fusion and is regulated by mRNA splicing, membrane potential, and Yme1L , 2007, The Journal of cell biology.

[129]  R. Rikhy,et al.  Mutations in dynamin‐related protein result in gross changes in mitochondrial morphology and affect synaptic vesicle recycling at the Drosophila neuromuscular junction , 2007, Genes, brain, and behavior.

[130]  Laura C. Greaves,et al.  Mitochondrial DNA Mutations and Aging , 2007, Annals of the New York Academy of Sciences.

[131]  D. Chan,et al.  Complementation between mouse Mfn1 and Mfn2 protects mitochondrial fusion defects caused by CMT2A disease mutations , 2007, The Journal of cell biology.

[132]  T. Letellier,et al.  Mitochondrial bioenergetics and structural network organization , 2007, Journal of Cell Science.

[133]  J. McCaffery,et al.  Mitochondrial Fusion Protects against Neurodegeneration in the Cerebellum , 2007, Cell.

[134]  J. Nunnari.  The machines that divide and fuse mitochondria , 2007, Annual review of biochemistry.

[135]  Craig Brooks,et al.  Bak regulates mitochondrial morphology and pathology during apoptosis by interacting with mitofusins , 2007, Proceedings of the National Academy of Sciences.

[136]  Jennifer R. Davies,et al.  Opa1 deficiency in a mouse model of autosomal dominant optic atrophy impairs mitochondrial morphology, optic nerve structure and visual function. , 2007, Human molecular genetics.

[137]  C. Blackstone,et al.  Cyclic AMP-dependent Protein Kinase Phosphorylation of Drp1 Regulates Its GTPase Activity and Mitochondrial Morphology* , 2007, Journal of Biological Chemistry.

[138]  S. Baudet,et al.  Drp 1 Mediates Caspase-Independent Type III Cell Death in Normal and Leukemic Cells † , 2007 .

[139]  H. Waterham,et al.  A lethal defect of mitochondrial and peroxisomal fission. , 2007, The New England journal of medicine.

[140]  E. Rugarli,et al.  OPA1 processing reconstituted in yeast depends on the subunit composition of the m-AAA protease in mitochondria. , 2007, Molecular biology of the cell.

[141]  D. Chan,et al.  Functions and dysfunctions of mitochondrial dynamics , 2007, Nature Reviews Molecular Cell Biology.

[142]  D. Chan,et al.  OPA1 processing controls mitochondrial fusion and is regulated by mRNA splicing, membrane potential, and Yme1L , 2007, The Journal of cell biology.

[143]  T. Nyström,et al.  Reducing mitochondrial fission results in increased life span and fitness of two fungal ageing models , 2007, Nature Cell Biology.

[144]  R. Youle,et al.  Role of Mitochondrial Remodeling in Programmed Cell Death in Drosophila melanogaster , 2007, Developmental cell.

[145]  Robert A. Harris,et al.  Functional consequences of mitochondrial proteome heterogeneity. , 2007, American journal of physiology. Cell physiology.

[146]  David S. Park,et al.  Mitofusin 2 Protects Cerebellar Granule Neurons against Injury-induced Cell Death* , 2007, Journal of Biological Chemistry.

[147]  Robert W. Taylor,et al.  Mutation of OPA1 causes dominant optic atrophy with external ophthalmoplegia, ataxia, deafness and multiple mitochondrial DNA deletions: a novel disorder of mtDNA maintenance. , 2008, Brain : a journal of neurology.

[148]  Richard J. Flannery,et al.  Bcl-xL induces Drp1-dependent synapse formation in cultured hippocampal neurons , 2008, Proceedings of the National Academy of Sciences.

[149]  Angela C. Poole,et al.  The PINK1/Parkin pathway regulates mitochondrial morphology , 2008, Proceedings of the National Academy of Sciences.

[150]  R. Schwarzenbacher,et al.  OPA1 mutations induce mitochondrial DNA instability and optic atrophy 'plus' phenotypes. , 2008, Brain : a journal of neurology.

[151]  Min Wu,et al.  Fission and selective fusion govern mitochondrial segregation and elimination by autophagy , 2008, The EMBO journal.

[152]  D. Wallace,et al.  The Molecular Mechanisms of OPA1-Mediated Optic Atrophy in Drosophila Model and Prospects for Antioxidant Treatment , 2008, PLoS genetics.

[153]  M. Beal,et al.  Pink1 regulates mitochondrial dynamics through interaction with the fission/fusion machinery , 2008, Proceedings of the National Academy of Sciences.

[154]  D. Rousseau,et al.  The Layered Structure of Human Mitochondrial DNA Nucleoids* , 2008, Journal of Biological Chemistry.