Misfolded SOD1 associated with motor neuron mitochondria alters mitochondrial shape and distribution prior to clinical onset. (Q33968208)

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8 February 2020

title

Misfolded SOD1 associated with motor neuron mitochondria alters mitochondrial shape and distribution prior to clinical onset (English)

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10

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8

Jean-Pierre Julien

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Christine Vande Velde

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Christine Vande Velde

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author name string

Karli K McDonald

2

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Yasmin Boukhedimi

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Melissa McAlonis-Downes

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Christian S Lobsiger

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Samar Bel Hadj

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Andre Zandona

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Sameer B Shah

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language of work or name

English

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publication date

11 July 2011

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volume

6

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issue

7

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page(s)

e22031

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Creative Commons Attribution 4.0 International

8 February 2020

copyright license

start time

11 July 2011

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April 2022 Public Data File from Crossref

copyright status

copyrighted

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cites work

ALS-linked mutant superoxide dismutase 1 (SOD1) alters mitochondrial protein composition and decreases protein import.

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Misfolded mutant SOD1 directly inhibits VDAC1 conductance in a mouse model of inherited ALS

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Phosphorylation of highly conserved neurofilament medium KSP repeats is not required for myelin-dependent radial axonal growth

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Selective association of misfolded ALS-linked mutant SOD1 with the cytoplasmic face of mitochondria.

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

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Amyotrophic lateral sclerosis-associated SOD1 mutant proteins bind and aggregate with Bcl-2 in spinal cord mitochondria

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ALS-associated mutant SOD1G93A causes mitochondrial vacuolation by expansion of the intermembrane space and by involvement of SOD1 aggregation and peroxisomes

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Active suppression of interneuron programs within developing motor neurons revealed by analysis of homeodomain factor HB9.

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Requirement for the homeobox gene Hb9 in the consolidation of motor neuron identity

5 July 2018

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ALS-linked SOD1 mutant G85R mediates damage to astrocytes and promotes rapidly progressive disease with SOD1-containing inclusions.

5 July 2018

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Development of central nervous system pathology in a murine transgenic model of human amyotrophic lateral sclerosis

5 July 2018

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Fine structural observations of neurofilamentous changes in amyotrophic lateral sclerosis

5 July 2018

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An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria

5 July 2018

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Toxicity of familial ALS-linked SOD1 mutants from selective recruitment to spinal mitochondria.

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Morphometric Comparison of the Vulnerability of Peripheral Motor and Sensory Neurons in Amyotrophic Lateral Sclerosis

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Fine structural study of neurofibrillary changes in a family with amyotrophic lateral sclerosis

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Dendritic synapses of anterior horn neurons in amyotrophic lateral sclerosis: an ultrastructural study

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Massive mitochondrial degeneration in motor neurons triggers the onset of amyotrophic lateral sclerosis in mice expressing a mutant SOD1

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The homeodomain transcription factors Islet 1 and HB9 are expressed in adult alpha and gamma motoneurons identified by selective retrograde tracing

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Intracerebroventricular infusion of monoclonal antibody or its derived Fab fragment against misfolded forms of SOD1 mutant delays mortality in a mouse model of ALS

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