Familial amyotrophic lateral sclerosis-linked SOD1 mutants perturb fast axonal transport to reduce axonal mitochondria content.

No abstract is available for this paper.

• Publication year

  2007

• Venue

  Human Molecular Genetics

• Publication date

  2007-11-15

• Fields of study

  Biology, Medicine

• Identifiers
  DOI 10.1093/hmg/ddm226PMID 17725983PMCID PMC4516806
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

• Visualization and quantification of mitochondrial dynamics in living animal cells.

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• Interaction between Familial Amyotrophic Lateral Sclerosis (ALS)-linked SOD1 Mutants and the Dynein Complex*

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• Mitochondrial Alterations in the Spinal Cord of Patients With Sporadic Amyotrophic Lateral Sclerosis

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• Inhibition of APP Trafficking by Tau Protein Does Not Increase the Generation of Amyloid‐β Peptides

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• The role of excitotoxicity in the pathogenesis of amyotrophic lateral sclerosis.

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• The atypical Rho GTPases Miro-1 and Miro-2 have essential roles in mitochondrial trafficking.

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• Axonal transport of mitochondria requires milton to recruit kinesin heavy chain and is light chain independent

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• JNK mediates pathogenic effects of polyglutamine-expanded androgen receptor on fast axonal transport

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• Review ALS: A Disease of Motor Neurons and Their Nonneuronal Neighbors

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• Familial ALS-superoxide dismutases associate with mitochondria and shift their redox potentials

  2006cited by this paper
• The Genetics of Axonal Transport and Axonal Transport Disorders

  2006cited by this paper
• ALS: a disease of motor neurons and their nonneuronal neighbors.

  2006cited by this paper
• Axonal transport and neurodegenerative disease.

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• The axonal transport of mitochondria

  2005cited by this paper
• Mitochondrial function and actin regulate dynamin-related protein 1-dependent mitochondrial fission.

  2005cited by this paper
• Molecular and cellular pathways of neurodegeneration in motor neurone disease

  2005cited by this paper
• A mutation in dynein rescues axonal transport defects and extends the life span of ALS mice

  2005cited by this paper
• Molecular motors in neuronal development, intracellular transport and diseases.

  2004influential reference
• Toxicity of familial ALS-linked SOD1 mutants from selective recruitment to spinal mitochondria.

  2004cited by this paper
• Image processing with ImageJ

  2004cited by this paper
• Amyotrophic lateral sclerosis-associated SOD1 mutant proteins bind and aggregate with Bcl-2 in spinal cord mitochondria.

  2004cited by this paper
• Axonal mitochondrial transport and potential are correlated

  2004influential reference
• p38alpha stress-activated protein kinase phosphorylates neurofilaments and is associated with neurofilament pathology in amyotrophic lateral sclerosis.

  2004cited by this paper
• p38α stress-activated protein kinase phosphorylates neurofilaments and is associated with neurofilament pathology in amyotrophic lateral sclerosis

  2004cited by this paper
• Activation of programmed cell death markers in ventral horn motor neurons during early presymptomatic stages of amyotrophic lateral sclerosis in a transgenic mouse model.

  2004cited by this paper
• Expression of phosphatidylinositol (4,5) bisphosphate-specific pleckstrin homology domains alters direction but not the level of axonal transport of mitochondria.

  2003cited by this paper
• Protein kinase and protein phosphatase expression in the central nervous system of G93A mSOD over‐expressing mice

  2003cited by this paper
• Persistent activation of p38 mitogen-activated protein kinase in a mouse model of familial amyotrophic lateral sclerosis correlates with disease progression.

  2003cited by this paper
• Axonal transport of neurofilaments in normal and disease states

  2002cited by this paper
• Tau blocks traffic of organelles, neurofilaments, and APP vesicles in neurons and enhances oxidative stress

  2002cited by this paper
• Motoneuron death triggered by a specific pathway downstream of Fas. potentiation by ALS-linked SOD1 mutations.

  2002cited by this paper
• Mutated Human SOD1 Causes Dysfunction of Oxidative Phosphorylation in Mitochondria of Transgenic Mice*

  2002cited by this paper
• Mutant Cu, Zn Superoxide Dismutase that Causes Motoneuron Degeneration Is Present in Mitochondria in the CNS

  2002cited by this paper
• Axonal membrane proteins are transported in distinct carriers: a two-color video microscopy study in cultured hippocampal neurons.

  2000cited by this paper
• Glutamate Slows Axonal Transport of Neurofilaments in Transfected Neurons

  2000cited by this paper
• Tumor Necrosis Factor Induces Hyperphosphorylation of Kinesin Light Chain and Inhibits Kinesin-Mediated Transport of Mitochondria

  2000cited by this paper
• Variation in the biochemical/biophysical properties of mutant superoxide dismutase 1 enzymes and the rate of disease progression in familial amyotrophic lateral sclerosis kindreds.

  1999cited by this paper
• Neurofilaments and Orthograde Transport Are Reduced in Ventral Root Axons of Transgenic Mice that Express Human SOD1 with a G93A Mutation

  1997cited by this paper
• Chloromethyl-X-Rosamine is an aldehyde-fixable potential-sensitive fluorochrome for the detection of early apoptosis.

  1996cited by this paper
• Impairment of fast axonal transport in the proximal axons of anterior horn neurons in amyotrophic lateral sclerosis

  1996cited by this paper
• Axonal transport of mitochondria along microtubules and F-actin in living vertebrate neurons

  1995cited by this paper
• Identification and transport of full-length amyloid precursor proteins in rat peripheral nervous system

  1993cited by this paper
• Precursor of amyloid protein in Alzheimer disease undergoes fast anterograde axonal transport.

  1990cited by this paper
• Van Damme A.

  1986cited by this paper

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  2026cites this paper
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  2026cites this paper
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  2024cites this paper
• hnRNP R regulates mitochondrial movement and membrane potential in axons of motoneurons.

  2024cites this paper
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  2023cites this paper
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  2023cites this paper
• Targeting mitochondrial Ca2+ uptake for the treatment of amyotrophic lateral sclerosis

  2023cites this paper
• Exposure to ambient air toxicants and the risk of amyotrophic lateral sclerosis (ALS): A matched case control study.

  2023cites this paper
• Compartment specific mitochondrial dysfunction in Drosophila knock-in model of ALS reversed by altered gene expression of OXPHOS subunits and pro-fission factor Drp1.

  2023cites this paper
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  2023cites this paper
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• Two hit mitochondrial-driven model of synapse loss in neurodegeneration.

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