Misfolded SOD1 Associated with Motor Neuron Mitochondria Alters Mitochondrial Shape and Distribution Prior to Clinical Onset

Mutations in superoxide dismutase (SOD1) are causative for inherited amyotrophic lateral sclerosis. A proportion of SOD1 mutant protein is misfolded onto the cytoplasmic face of mitochondria in one or more spinal cord cell types. By construction of mice in which mitochondrially targeted enhanced gre...

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Published inPloS one Vol. 6; no. 7; p. e22031
Main Authors Vande Velde, Christine, McDonald, Karli K., Boukhedimi, Yasmin, McAlonis-Downes, Melissa, Lobsiger, Christian S., Bel Hadj, Samar, Zandona, Andre, Julien, Jean-Pierre, Shah, Sameer B., Cleveland, Don W.
Format Journal Article
LanguageEnglish
Published United States Public Library of Science 11.07.2011
Public Library of Science (PLoS)
Subjects
Aberration
Amyotrophic lateral sclerosis
Animals
Axon guidance
Axons
Biology
Cancer
Clustering
Cytochrome
Development and progression
Elongation
Fluorescence
Fluorescent Antibody Technique
Genetic aspects
Green fluorescent protein
Homeostasis
Immunoblotting
Medical research
Medicine
Mice
Mice, Transgenic
Mitochondria
Mitochondria - metabolism
Morphology
Motor neurons
Motor Neurons - metabolism
Multiple sclerosis
Mutants
Mutation
Neurons
Neurosciences
Pathogenesis
Permeability
Physical characteristics
Protein Folding
Recruitment
Rodents
Spinal cord
Superoxide dismutase
Superoxide Dismutase - chemistry
Superoxide Dismutase - genetics
Superoxide Dismutase - metabolism
Superoxide Dismutase-1
Superoxides
La Jolla California
United States > US
California
Online AccessGet full text
ISSN1932-6203
1932-6203
DOI10.1371/journal.pone.0022031

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Abstract Mutations in superoxide dismutase (SOD1) are causative for inherited amyotrophic lateral sclerosis. A proportion of SOD1 mutant protein is misfolded onto the cytoplasmic face of mitochondria in one or more spinal cord cell types. By construction of mice in which mitochondrially targeted enhanced green fluorescent protein is selectively expressed in motor neurons, we demonstrate that axonal mitochondria of motor neurons are primary in vivo targets for misfolded SOD1. Mutant SOD1 alters axonal mitochondrial morphology and distribution, with dismutase active SOD1 causing mitochondrial clustering at the proximal side of Schmidt-Lanterman incisures within motor axons and dismutase inactive SOD1 producing aberrantly elongated axonal mitochondria beginning pre-symptomatically and increasing in severity as disease progresses. Somal mitochondria are altered by mutant SOD1, with loss of the characteristic cylindrical, networked morphology and its replacement by a less elongated, more spherical shape. These data indicate that mutant SOD1 binding to mitochondria disrupts normal mitochondrial distribution and size homeostasis as early pathogenic features of SOD1 mutant-mediated ALS.
AbstractList Mutations in superoxide dismutase (SOD1) are causative for inherited amyotrophic lateral sclerosis. A proportion of SOD1 mutant protein is misfolded onto the cytoplasmic face of mitochondria in one or more spinal cord cell types. By construction of mice in which mitochondrially targeted enhanced green fluorescent protein is selectively expressed in motor neurons, we demonstrate that axonal mitochondria of motor neurons are primary in vivo targets for misfolded SOD1. Mutant SOD1 alters axonal mitochondrial morphology and distribution, with dismutase active SOD1 causing mitochondrial clustering at the proximal side of Schmidt-Lanterman incisures within motor axons and dismutase inactive SOD1 producing aberrantly elongated axonal mitochondria beginning pre-symptomatically and increasing in severity as disease progresses. Somal mitochondria are altered by mutant SOD1, with loss of the characteristic cylindrical, networked morphology and its replacement by a less elongated, more spherical shape. These data indicate that mutant SOD1 binding to mitochondria disrupts normal mitochondrial distribution and size homeostasis as early pathogenic features of SOD1 mutant-mediated ALS.
Mutations in superoxide dismutase (SOD1) are causative for inherited amyotrophic lateral sclerosis. A proportion of SOD1 mutant protein is misfolded onto the cytoplasmic face of mitochondria in one or more spinal cord cell types. By construction of mice in which mitochondrially targeted enhanced green fluorescent protein is selectively expressed in motor neurons, we demonstrate that axonal mitochondria of motor neurons are primary in vivo targets for misfolded SOD1. Mutant SOD1 alters axonal mitochondrial morphology and distribution, with dismutase active SOD1 causing mitochondrial clustering at the proximal side of Schmidt-Lanterman incisures within motor axons and dismutase inactive SOD1 producing aberrantly elongated axonal mitochondria beginning pre-symptomatically and increasing in severity as disease progresses. Somal mitochondria are altered by mutant SOD1, with loss of the characteristic cylindrical, networked morphology and its replacement by a less elongated, more spherical shape. These data indicate that mutant SOD1 binding to mitochondria disrupts normal mitochondrial distribution and size homeostasis as early pathogenic features of SOD1 mutant-mediated ALS.
Mutations in superoxide dismutase (SOD1) are causative for inherited amyotrophic lateral sclerosis. A proportion of SOD1 mutant protein is misfolded onto the cytoplasmic face of mitochondria in one or more spinal cord cell types. By construction of mice in which mitochondrially targeted enhanced green fluorescent protein is selectively expressed in motor neurons, we demonstrate that axonal mitochondria of motor neurons are primary in vivo targets for misfolded SOD1. Mutant SOD1 alters axonal mitochondrial morphology and distribution, with dismutase active SOD1 causing mitochondrial clustering at the proximal side of Schmidt-Lanterman incisures within motor axons and dismutase inactive SOD1 producing aberrantly elongated axonal mitochondria beginning pre-symptomatically and increasing in severity as disease progresses. Somal mitochondria are altered by mutant SOD1, with loss of the characteristic cylindrical, networked morphology and its replacement by a less elongated, more spherical shape. These data indicate that mutant SOD1 binding to mitochondria disrupts normal mitochondrial distribution and size homeostasis as early pathogenic features of SOD1 mutant-mediated ALS.Mutations in superoxide dismutase (SOD1) are causative for inherited amyotrophic lateral sclerosis. A proportion of SOD1 mutant protein is misfolded onto the cytoplasmic face of mitochondria in one or more spinal cord cell types. By construction of mice in which mitochondrially targeted enhanced green fluorescent protein is selectively expressed in motor neurons, we demonstrate that axonal mitochondria of motor neurons are primary in vivo targets for misfolded SOD1. Mutant SOD1 alters axonal mitochondrial morphology and distribution, with dismutase active SOD1 causing mitochondrial clustering at the proximal side of Schmidt-Lanterman incisures within motor axons and dismutase inactive SOD1 producing aberrantly elongated axonal mitochondria beginning pre-symptomatically and increasing in severity as disease progresses. Somal mitochondria are altered by mutant SOD1, with loss of the characteristic cylindrical, networked morphology and its replacement by a less elongated, more spherical shape. These data indicate that mutant SOD1 binding to mitochondria disrupts normal mitochondrial distribution and size homeostasis as early pathogenic features of SOD1 mutant-mediated ALS.
Audience Academic
Author McDonald, Karli K.
Julien, Jean-Pierre
Boukhedimi, Yasmin
Vande Velde, Christine
Lobsiger, Christian S.
Shah, Sameer B.
Cleveland, Don W.
Bel Hadj, Samar
Zandona, Andre
McAlonis-Downes, Melissa
AuthorAffiliation 3 Institut National de la Santé et de la Recherche Médicale, Unité Mixte de Recherche S975, Centre de Recherche de l'Institut du Cerveau et de la Moelle Épinière, Hôpital de la Salpêtrière, Paris, France
5 Fischell Department of Bioengineering, University of Maryland, College Park, Maryland, United States of America
1 Centre d'excellence en neuromique de l'Université de Montréal (CENUM), Centre de recherche du Centre hospitalier de l'Université de Montréal (CRCHUM), and Département de Médecine, Université de Montréal, Montréal, Québec, Canada
2 Ludwig Institute for Cancer Research and Departments of Neuroscience and Cellular and Molecular Medicine, University of California San Diego, La Jolla, California, United States of America
4 Centre de recherche du Centre hospitalier de l'Université Laval (CHUL), Université Laval, Québec, Québec, Canada
Johns Hopkins, United States of America
AuthorAffiliation_xml – name: 5 Fischell Department of Bioengineering, University of Maryland, College Park, Maryland, United States of America
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BackLink https://www.ncbi.nlm.nih.gov/pubmed/21779368$$D View this record in MEDLINE/PubMed
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Cites_doi 10.1016/S0896-6273(01)80026-X
10.1097/00005072-198111000-00008
10.1093/hmg/ddm226
10.1016/0896-6273(95)90259-7
10.1007/s004010050426
10.1073/pnas.0712209105
10.1046/j.1460-9568.1999.00631.x
10.1523/JNEUROSCI.18-09-03241.1998
10.1016/j.neuron.2010.07.019
10.1097/00005072-198409000-00001
10.1089/ars.2009.2614
10.1097/00005072-198409000-00002
10.1097/nen.0b013e31802c396b
10.1016/S0896-6273(01)80027-1
10.1016/j.bbi.2006.12.008
10.1016/j.expneurol.2009.02.015
10.1186/1471-2202-4-16
10.1016/j.neuron.2004.06.021
10.1074/jbc.M702295200
10.1371/journal.pone.0013151
10.1038/nmeth1055
10.1016/0006-8993(90)91284-N
10.1016/S0896-6273(00)80272-X
10.1016/j.cell.2006.06.010
10.1073/pnas.1014862107
10.1038/nn.2660
10.1002/ana.21748
10.1523/JNEUROSCI.3765-08.2009
10.1016/j.neuron.2006.09.018
10.1083/jcb.200908164
10.1016/j.neuron.2004.06.016
10.1111/j.1471-4159.2010.06683.x
ContentType Journal Article
Copyright COPYRIGHT 2011 Public Library of Science
2011 Vande Velde et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License: https://creativecommons.org/licenses/by/4.0/ (the “License”), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.
Vande Velde et al. 2011
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– notice: 2011 Vande Velde et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License: https://creativecommons.org/licenses/by/4.0/ (the “License”), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.
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DocumentTitleAlternate Misfolded Mutant SOD1 Alters Mitochondria
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Conceived and designed the experiments: CVV DWC. Performed the experiments: CVV KKM YB SBH MM-D CSL AZ. Analyzed the data: CVV SBS DWC. Contributed reagents/materials/analysis tools: J-PJ SBS. Wrote the paper: CVV SBS DWC.
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References F Vult von Steyern (ref17) 1999; 11
PC Wong (ref13) 1995; 14
P Pasinelli (ref25) 2004; 43
A Hirano (ref6) 1984; 43
J Liu (ref24) 2004; 43
T Misgeld (ref27) 2007; 4
H Kebir (ref33) 2009; 66
MC Dal Canto (ref10) 1994; 145
A Israelson (ref3) 2010; 67
DA Bosco (ref32) 2010; 13
LJ Martin (ref29) 2009; 218
Q Li (ref4) 2010; 107
A Kaasik (ref19) 2007; 282
RL Price (ref21) 1990; 530
KJ De Vos (ref31) 2007; 16
C Vande Velde (ref5) 2008; 105
A Hirano (ref7) 1984; 43
LI Bruijn (ref14) 1997; 18
S Boillee (ref1) 2006; 52
WM Campana (ref22) 2007; 21
H Ilieva (ref2) 2009; 187
F Gros-Louis (ref20) 2010; 113
S Sasaki (ref9) 2007; 66
JR Sotelo-Silveira (ref28) 2009; 11
S Arber (ref16) 1999; 23
J Park (ref30) 2010; 5
J Kong (ref12) 1998; 18
S Sasaki (ref8) 1996; 91
J Thaler (ref15) 1999; 23
ML Garcia (ref26) 2009; 29
DC Chan (ref23) 2006; 125
CM Higgins (ref11) 2003; 4
Y Kawamura (ref18) 1981; 40
8834540 - Acta Neuropathol. 1996;91(3):278-83
18296640 - Proc Natl Acad Sci U S A. 2008 Mar 11;105(10):4022-7
9052802 - Neuron. 1997 Feb;18(2):327-38
17785462 - J Biol Chem. 2007 Nov 9;282(45):32821-6
20949033 - PLoS One. 2010;5(10):e13151
17558414 - Nat Methods. 2007 Jul;4(7):559-61
6540799 - J Neuropathol Exp Neurol. 1984 Sep;43(5):461-70
7605627 - Neuron. 1995 Jun;14(6):1105-16
7299423 - J Neuropathol Exp Neurol. 1981 Nov;40(6):667-75
19193875 - J Neurosci. 2009 Feb 4;29(5):1277-84
17015226 - Neuron. 2006 Oct 5;52(1):39-59
6540800 - J Neuropathol Exp Neurol. 1984 Sep;43(5):471-80
20797535 - Neuron. 2010 Aug 26;67(4):575-87
10482235 - Neuron. 1999 Aug;23(4):675-87
21078990 - Proc Natl Acad Sci U S A. 2010 Dec 7;107(49):21146-51
17204932 - J Neuropathol Exp Neurol. 2007 Jan;66(1):10-6
17725983 - Hum Mol Genet. 2007 Nov 15;16(22):2720-8
19810097 - Ann Neurol. 2009 Sep;66(3):390-402
7992831 - Am J Pathol. 1994 Dec;145(6):1271-9
16814712 - Cell. 2006 Jun 30;125(7):1241-52
19272377 - Exp Neurol. 2009 Aug;218(2):333-46
17321718 - Brain Behav Immun. 2007 Jul;21(5):522-7
19951898 - J Cell Biol. 2009 Dec 14;187(6):761-72
9547233 - J Neurosci. 1998 May 1;18(9):3241-50
12864925 - BMC Neurosci. 2003 Jul 15;4:16
15233913 - Neuron. 2004 Jul 8;43(1):5-17
15233914 - Neuron. 2004 Jul 8;43(1):19-30
19344250 - Antioxid Redox Signal. 2009 Jul;11(7):1535-45
10482234 - Neuron. 1999 Aug;23(4):659-74
20953194 - Nat Neurosci. 2010 Nov;13(11):1396-403
10336678 - Eur J Neurosci. 1999 Jun;11(6):2093-102
20345765 - J Neurochem. 2010 Jun;113(5):1188-99
1702341 - Brain Res. 1990 Oct 22;530(2):205-14
References_xml – volume: 23
  start-page: 659
  year: 1999
  ident: ref16
  article-title: Requirement for the homeobox gene Hb9 in the consolidation of motor neuron identity.
  publication-title: Neuron
  doi: 10.1016/S0896-6273(01)80026-X
– volume: 40
  start-page: 667
  year: 1981
  ident: ref18
  article-title: Morphometric comparison of the vulnerability of peripheral motor and sensory neurons in amyotrophic lateral sclerosis.
  publication-title: J Neuropathol Exp Neurol
  doi: 10.1097/00005072-198111000-00008
– volume: 16
  start-page: 2720
  year: 2007
  ident: ref31
  article-title: Familial amyotrophic lateral sclerosis-linked SOD1 mutants perturb fast axonal transport to reduce axonal mitochondria content.
  publication-title: Hum Mol Genet
  doi: 10.1093/hmg/ddm226
– volume: 14
  start-page: 1105
  year: 1995
  ident: ref13
  article-title: An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria.
  publication-title: Neuron
  doi: 10.1016/0896-6273(95)90259-7
– volume: 91
  start-page: 278
  year: 1996
  ident: ref8
  article-title: Dendritic synapses of anterior horn neurons in amyotrophic lateral sclerosis: an ultrastructural study.
  publication-title: Acta Neuropathol (Berl)
  doi: 10.1007/s004010050426
– volume: 105
  start-page: 4022
  year: 2008
  ident: ref5
  article-title: Selective association of misfolded ALS-linked mutant SOD1 with the cytoplasmic face of mitochondria.
  publication-title: Proc Natl Acad Sci U S A
  doi: 10.1073/pnas.0712209105
– volume: 11
  start-page: 2093
  year: 1999
  ident: ref17
  article-title: The homeodomain transcription factors Islet 1 and HB9 are expressed in adult alpha and gamma motoneurons identified by selective retrograde tracing.
  publication-title: Eur J Neurosci
  doi: 10.1046/j.1460-9568.1999.00631.x
– volume: 18
  start-page: 3241
  year: 1998
  ident: ref12
  article-title: Massive mitochondrial degeneration in motor neurons triggers the onset of amyotrophic lateral sclerosis in mice expressing a mutant SOD1.
  publication-title: J Neurosci
  doi: 10.1523/JNEUROSCI.18-09-03241.1998
– volume: 67
  start-page: 575
  year: 2010
  ident: ref3
  article-title: Misfolded mutant SOD1 directly inhibits VDAC1 conductance in a mouse model of inherited ALS.
  publication-title: Neuron
  doi: 10.1016/j.neuron.2010.07.019
– volume: 43
  start-page: 461
  year: 1984
  ident: ref6
  article-title: Fine structural observations of neurofilamentous changes in amyotrophic lateral sclerosis.
  publication-title: J Neuropathol Exp Neurol
  doi: 10.1097/00005072-198409000-00001
– volume: 11
  start-page: 1535
  year: 2009
  ident: ref28
  article-title: Axonal mitochondrial clusters containing mutant SOD1 in transgenic models of ALS.
  publication-title: Antioxid Redox Signal
  doi: 10.1089/ars.2009.2614
– volume: 43
  start-page: 471
  year: 1984
  ident: ref7
  article-title: Fine structural study of neurofibrillary changes in a family with amyotrophic lateral sclerosis.
  publication-title: J Neuropathol Exp Neurol
  doi: 10.1097/00005072-198409000-00002
– volume: 66
  start-page: 10
  year: 2007
  ident: ref9
  article-title: Mitochondrial alterations in the spinal cord of patients with sporadic amyotrophic lateral sclerosis.
  publication-title: J Neuropathol Exp Neurol
  doi: 10.1097/nen.0b013e31802c396b
– volume: 23
  start-page: 675
  year: 1999
  ident: ref15
  article-title: Active suppression of interneuron programs within developing motor neurons revealed by analysis of homeodomain factor HB9.
  publication-title: Neuron
  doi: 10.1016/S0896-6273(01)80027-1
– volume: 21
  start-page: 522
  year: 2007
  ident: ref22
  article-title: Schwann cells: activated peripheral glia and their role in neuropathic pain.
  publication-title: Brain Behav Immun
  doi: 10.1016/j.bbi.2006.12.008
– volume: 218
  start-page: 333
  year: 2009
  ident: ref29
  article-title: The mitochondrial permeability transition pore in motor neurons: Involvement in the pathobiology of ALS mice.
  publication-title: Exp Neurol
  doi: 10.1016/j.expneurol.2009.02.015
– volume: 4
  start-page: 16
  year: 2003
  ident: ref11
  article-title: ALS-associated mutant SOD1G93A causes mitochondrial vacuolation by expansion of the intermembrane space and by involvement of SOD1 aggregation and peroxisomes.
  publication-title: BMC Neurosci
  doi: 10.1186/1471-2202-4-16
– volume: 43
  start-page: 19
  year: 2004
  ident: ref25
  article-title: Amyotrophic Lateral Sclerosis-Associated SOD1 Mutant Proteins Bind and Aggregate with Bcl-2 in Spinal Cord Mitochondria.
  publication-title: Neuron
  doi: 10.1016/j.neuron.2004.06.021
– volume: 282
  start-page: 32821
  year: 2007
  ident: ref19
  article-title: Mitochondrial swelling impairs the transport of organelles in cerebellar granule neurons.
  publication-title: J Biol Chem
  doi: 10.1074/jbc.M702295200
– volume: 5
  start-page: e13151
  year: 2010
  ident: ref30
  article-title: Drosophila Porin/VDAC affects mitochondrial morphology.
  publication-title: PLoS One
  doi: 10.1371/journal.pone.0013151
– volume: 4
  start-page: 559
  year: 2007
  ident: ref27
  article-title: Imaging axonal transport of mitochondria in vivo.
  publication-title: Nat Methods
  doi: 10.1038/nmeth1055
– volume: 530
  start-page: 205
  year: 1990
  ident: ref21
  article-title: Internal axonal cytoarchitecture is shaped locally by external compressive forces.
  publication-title: Brain Res
  doi: 10.1016/0006-8993(90)91284-N
– volume: 18
  start-page: 327
  year: 1997
  ident: ref14
  article-title: ALS-linked SOD1 mutant G85R mediates damage to astrocytes and promotes rapidly progressive disease with SOD1-containing inclusions.
  publication-title: Neuron
  doi: 10.1016/S0896-6273(00)80272-X
– volume: 125
  start-page: 1241
  year: 2006
  ident: ref23
  article-title: Mitochondria: dynamic organelles in disease, aging, and development.
  publication-title: Cell
  doi: 10.1016/j.cell.2006.06.010
– volume: 107
  start-page: 21146
  year: 2010
  ident: ref4
  article-title: ALS-linked mutant superoxide dismutase 1 (SOD1) alters mitochondrial protein composition and decreases protein import.
  publication-title: Proc Natl Acad Sci U S A
  doi: 10.1073/pnas.1014862107
– volume: 145
  start-page: 1271
  year: 1994
  ident: ref10
  article-title: Development of central nervous system pathology in a murine transgenic model of human amyotrophic lateral sclerosis.
  publication-title: Am J Pathol
– volume: 13
  start-page: 1396
  year: 2010
  ident: ref32
  article-title: Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS.
  publication-title: Nat Neurosci
  doi: 10.1038/nn.2660
– volume: 66
  start-page: 390
  year: 2009
  ident: ref33
  article-title: Preferential recruitment of interferon-gamma-expressing TH17 cells in multiple sclerosis.
  publication-title: Ann Neurol
  doi: 10.1002/ana.21748
– volume: 29
  start-page: 1277
  year: 2009
  ident: ref26
  article-title: Phosphorylation of highly conserved neurofilament medium KSP repeats is not required for myelin-dependent radial axonal growth.
  publication-title: J Neurosci
  doi: 10.1523/JNEUROSCI.3765-08.2009
– volume: 52
  start-page: 1
  year: 2006
  ident: ref1
  article-title: ALS: A disease of motor neurons and their nonneuronal neighbors.
  publication-title: Neuron
  doi: 10.1016/j.neuron.2006.09.018
– volume: 187
  start-page: 761
  year: 2009
  ident: ref2
  article-title: Non-cell autonomous toxicity in neurodegenerative disorders: ALS and beyond.
  publication-title: J Cell Biol
  doi: 10.1083/jcb.200908164
– volume: 43
  start-page: 5
  year: 2004
  ident: ref24
  article-title: Toxicity of Familial ALS-Linked SOD1 Mutants from Selective Recruitment to Spinal Mitochondria.
  publication-title: Neuron
  doi: 10.1016/j.neuron.2004.06.016
– volume: 113
  start-page: 1188
  year: 2010
  ident: ref20
  article-title: Intracerebroventricular infusion of monoclonal antibody or its derived Fab fragment against misfolded forms of SOD1 mutant delays mortality in a mouse model of ALS.
  publication-title: J Neurochem
  doi: 10.1111/j.1471-4159.2010.06683.x
– reference: 17558414 - Nat Methods. 2007 Jul;4(7):559-61
– reference: 16814712 - Cell. 2006 Jun 30;125(7):1241-52
– reference: 17204932 - J Neuropathol Exp Neurol. 2007 Jan;66(1):10-6
– reference: 12864925 - BMC Neurosci. 2003 Jul 15;4:16
– reference: 1702341 - Brain Res. 1990 Oct 22;530(2):205-14
– reference: 7299423 - J Neuropathol Exp Neurol. 1981 Nov;40(6):667-75
– reference: 19344250 - Antioxid Redox Signal. 2009 Jul;11(7):1535-45
– reference: 18296640 - Proc Natl Acad Sci U S A. 2008 Mar 11;105(10):4022-7
– reference: 10336678 - Eur J Neurosci. 1999 Jun;11(6):2093-102
– reference: 21078990 - Proc Natl Acad Sci U S A. 2010 Dec 7;107(49):21146-51
– reference: 19272377 - Exp Neurol. 2009 Aug;218(2):333-46
– reference: 15233914 - Neuron. 2004 Jul 8;43(1):19-30
– reference: 17725983 - Hum Mol Genet. 2007 Nov 15;16(22):2720-8
– reference: 20797535 - Neuron. 2010 Aug 26;67(4):575-87
– reference: 7605627 - Neuron. 1995 Jun;14(6):1105-16
– reference: 9547233 - J Neurosci. 1998 May 1;18(9):3241-50
– reference: 20949033 - PLoS One. 2010;5(10):e13151
– reference: 15233913 - Neuron. 2004 Jul 8;43(1):5-17
– reference: 6540800 - J Neuropathol Exp Neurol. 1984 Sep;43(5):471-80
– reference: 20345765 - J Neurochem. 2010 Jun;113(5):1188-99
– reference: 17321718 - Brain Behav Immun. 2007 Jul;21(5):522-7
– reference: 6540799 - J Neuropathol Exp Neurol. 1984 Sep;43(5):461-70
– reference: 20953194 - Nat Neurosci. 2010 Nov;13(11):1396-403
– reference: 7992831 - Am J Pathol. 1994 Dec;145(6):1271-9
– reference: 19951898 - J Cell Biol. 2009 Dec 14;187(6):761-72
– reference: 19193875 - J Neurosci. 2009 Feb 4;29(5):1277-84
– reference: 9052802 - Neuron. 1997 Feb;18(2):327-38
– reference: 10482235 - Neuron. 1999 Aug;23(4):675-87
– reference: 19810097 - Ann Neurol. 2009 Sep;66(3):390-402
– reference: 17015226 - Neuron. 2006 Oct 5;52(1):39-59
– reference: 8834540 - Acta Neuropathol. 1996;91(3):278-83
– reference: 17785462 - J Biol Chem. 2007 Nov 9;282(45):32821-6
– reference: 10482234 - Neuron. 1999 Aug;23(4):659-74
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Snippet Mutations in superoxide dismutase (SOD1) are causative for inherited amyotrophic lateral sclerosis. A proportion of SOD1 mutant protein is misfolded onto the...
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StartPage e22031
SubjectTerms Aberration
Amyotrophic lateral sclerosis
Animals
Axon guidance
Axons
Biology
Cancer
Clustering
Cytochrome
Development and progression
Elongation
Fluorescence
Fluorescent Antibody Technique
Genetic aspects
Green fluorescent protein
Homeostasis
Immunoblotting
Medical research
Medicine
Mice
Mice, Transgenic
Mitochondria
Mitochondria - metabolism
Morphology
Motor neurons
Motor Neurons - metabolism
Multiple sclerosis
Mutants
Mutation
Neurons
Neurosciences
Pathogenesis
Permeability
Physical characteristics
Protein Folding
Recruitment
Rodents
Spinal cord
Superoxide dismutase
Superoxide Dismutase - chemistry
Superoxide Dismutase - genetics
Superoxide Dismutase - metabolism
Superoxide Dismutase-1
Superoxides
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Title Misfolded SOD1 Associated with Motor Neuron Mitochondria Alters Mitochondrial Shape and Distribution Prior to Clinical Onset
URI https://www.ncbi.nlm.nih.gov/pubmed/21779368
https://www.proquest.com/docview/1305089687
https://www.proquest.com/docview/878821253
https://pubmed.ncbi.nlm.nih.gov/PMC3136936
https://doaj.org/article/14f4e00cf1e547a98d384acdc5447147
http://dx.doi.org/10.1371/journal.pone.0022031
Volume 6
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