Neuron-specific expression of mutant superoxide dismutase is sufficient to induce amyotrophic lateral sclerosis in transgenic mice (Q80786193)

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3 January 2020

title

Neuron-specific expression of mutant superoxide dismutase is sufficient to induce amyotrophic lateral sclerosis in transgenic mice (English)

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amyotrophic lateral sclerosis

3 January 2020

main subject

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4

Chris I De Zeeuw

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3 January 2020

author name string

Dick Jaarsma

1

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3 January 2020

Eva Teuling

2

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3 January 2020

Elize D Haasdijk

3

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3 January 2020

Casper C Hoogenraad

5

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3 January 2020

publication date

1 February 2008

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3 January 2020

published in

Journal of Neuroscience

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3 January 2020

volume

28

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3 January 2020

issue

9

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3 January 2020

page(s)

2075-2088

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describes a project that uses

3 January 2020

ImageQuant

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11 June 2022

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inferred from PubMed Central ID database lookup

cites work

Restricted expression of G86R Cu/Zn superoxide dismutase in astrocytes results in astrocytosis but does not cause motoneuron degeneration

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12 December 2020

Aggregates of mutant protein appear progressively in dendrites, in periaxonal processes of oligodendrocytes, and in neuronal and astrocytic perikarya of mice expressing the SOD1(G93A) mutation of familial amyotrophic lateral sclerosis

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Formation of high molecular weight complexes of mutant Cu, Zn-superoxide dismutase in a mouse model for familial amyotrophic lateral sclerosis.

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Imaging neuronal subsets in transgenic mice expressing multiple spectral variants of GFP

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Caspase-1 and -3 are sequentially activated in motor neuron death in Cu,Zn superoxide dismutase-mediated familial amyotrophic lateral sclerosis

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Human Cu/Zn superoxide dismutase (SOD1) overexpression in mice causes mitochondrial vacuolization, axonal degeneration, and premature motoneuron death and accelerates motoneuron disease in mice expressing a familial amyotrophic lateral sclerosis mut

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12 December 2020

Neuron-specific expression of mutant superoxide dismutase 1 in transgenic mice does not lead to motor impairment.

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12 December 2020

New consensus research on neuropathological aspects of familial amyotrophic lateral sclerosis with superoxide dismutase 1 (SOD1) gene mutations: inclusions containing SOD1 in neurons and astrocytes.

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12 December 2020

CuZn superoxide dismutase (SOD1) accumulates in vacuolated mitochondria in transgenic mice expressing amyotrophic lateral sclerosis-linked SOD1 mutations

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12 December 2020

Accumulation of SOD1 mutants in postnatal motoneurons does not cause motoneuron pathology or motoneuron disease

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12 December 2020

Sixteen novel mutations in the Cu/Zn superoxide dismutase gene in amyotrophic lateral sclerosis: a decade of discoveries, defects and disputes

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Wild-Type Nonneuronal Cells Extend Survival of SOD1 Mutant Motor Neurons in ALS Mice

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12 December 2020

Minute quantities of misfolded mutant superoxide dismutase-1 cause amyotrophic lateral sclerosis

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12 December 2020

Reactive astrocytes protect tissue and preserve function after spinal cord injury

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12 December 2020

Decrease of Hsp25 protein expression precedes degeneration of motoneurons in ALS-SOD1 mice

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Silencing mutant SOD1 using RNAi protects against neurodegeneration and extends survival in an ALS model

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Coincident thresholds of mutant protein for paralytic disease and protein aggregation caused by restrictively expressed superoxide dismutase cDNA

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Modulation of presynaptic plasticity and learning by the H-ras/extracellular signal-regulated kinase/synapsin I signaling pathway

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12 December 2020

ATF3 expression precedes death of spinal motoneurons in amyotrophic lateral sclerosis-SOD1 transgenic mice and correlates with c-Jun phosphorylation, CHOP expression, somato-dendritic ubiquitination and Golgi fragmentation

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12 December 2020

Rapid disease progression correlates with instability of mutant SOD1 in familial ALS

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12 December 2020

Disulphide-reduced superoxide dismutase-1 in CNS of transgenic amyotrophic lateral sclerosis models

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12 December 2020

Overloading of stable and exclusion of unstable human superoxide dismutase-1 variants in mitochondria of murine amyotrophic lateral sclerosis models

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12 December 2020

Disulfide cross-linked protein represents a significant fraction of ALS-associated Cu, Zn-superoxide dismutase aggregates in spinal cords of model mice

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12 December 2020

Conversion to the amyotrophic lateral sclerosis phenotype is associated with intermolecular linked insoluble aggregates of SOD1 in mitochondria

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12 December 2020

Onset and progression in inherited ALS determined by motor neurons and microglia

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Molecular biology of amyotrophic lateral sclerosis: insights from genetics

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ALS: a disease of motor neurons and their nonneuronal neighbors

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Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis

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Wild-type microglia extend survival in PU.1 knockout mice with familial amyotrophic lateral sclerosis

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12 December 2020

TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis

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12 December 2020

Mechanisms of Disease: astrocytes in neurodegenerative disease

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12 December 2020

Motor neuron disease in mice expressing the wild type-like D90A mutant superoxide dismutase-1

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How do ALS-associated mutations in superoxide dismutase 1 promote aggregation of the protein?

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12 December 2020

Distribution of glycinergic neuronal somata in the rat spinal cord

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Non-cell autonomous effect of glia on motor neurons in an embryonic stem cell-based ALS model

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Astrocytes expressing ALS-linked mutated SOD1 release factors selectively toxic to motor neurons

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Soluble misfolded subfractions of mutant superoxide dismutase-1s are enriched in spinal cords throughout life in murine ALS models

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Glial cells as intrinsic components of non-cell-autonomous neurodegenerative disease

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

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12 December 2020

Neuropathological changes in two lines of mice carrying a transgene for mutant human Cu,Zn SOD, and in mice overexpressing wild type human SOD: a model of familial amyotrophic lateral sclerosis (FALS).

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12 December 2020

Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation

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12 December 2020

Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis

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12 December 2020

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

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12 December 2020

Overexpression of growth-associated proteins in the neurons of adult transgenic mice

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12 December 2020

Aggregation of mutant Cu/Zn superoxide dismutase proteins in a culture model of ALS

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12 December 2020

A low expressor line of transgenic mice carrying a mutant human Cu,Zn superoxide dismutase (SOD1) gene develops pathological changes that most closely resemble those in human amyotrophic lateral sclerosis

1 reference

12 December 2020

Pathological characterization of astrocytic hyaline inclusions in familial amyotrophic lateral sclerosis.

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12 December 2020

The use of transgenic mouse models of amyotrophic lateral sclerosis in preclinical drug studies

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12 December 2020

Immunohistochemical and ultrastructural characterization of ubiquitinated eosinophilic fibrillary neuronal inclusions in sporadic amyotrophic lateral sclerosis

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12 December 2020

10.1523/JNEUROSCI.5258-07.2008

Identifiers

DOI

1 reference

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3 January 2020

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3 January 2020

PubMed publication ID

18305242

1 reference