Formation of high molecular weight complexes of mutant Cu, Zn-superoxide dismutase in a mouse model for familial amyotrophic lateral sclerosis. (Q35818148)

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scientific article published in November 2000

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English	Formation of high molecular weight complexes of mutant Cu, Zn-superoxide dismutase in a mouse model for familial amyotrophic lateral sclerosis.	scientific article published in November 2000

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scholarly article

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1 November 2019

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

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1 November 2019

amyotrophic lateral sclerosis

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Jennifer A. Johnston

1

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1 November 2019

author name string

M J Dalton

2

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1 November 2019

M E Gurney

3

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1 November 2019

R R Kopito

4

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1 November 2019

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publication date

1 November 2000

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1 November 2019

Proceedings of the National Academy of Sciences of the United States of America

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1 November 2019

97

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1 November 2019

12571-12576

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23

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1 November 2019

Intragenic deletion in the gene encoding ubiquitin carboxy-terminal hydrolase in gad mice

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Slowing of axonal transport is a very early event in the toxicity of ALS-linked SOD1 mutants to motor neurons

1 reference

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Aggresomes: a cellular response to misfolded proteins

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11 July 2018

Formation of granular cytoplasmic aggregates in COS7 cells expressing mutant Cu/Zn superoxide dismutase associated with familial amyotrophic lateral sclerosis.

1 reference

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The ubiquitin pathway in Parkinson's disease.

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11 July 2018

Aggregation and Motor Neuron Toxicity of an ALS-Linked SOD1 Mutant Independent from Wild-Type SOD1

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11 July 2018

Cytoskeletal proteins and Golgi dynamics

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Structural neurology: are seeds at the root of neuronal degeneration?

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Frameshift mutants of beta amyloid precursor protein and ubiquitin-B in Alzheimer's and Down patients

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11 July 2018

Neurofilaments and orthograde transport are reduced in ventral root axons of transgenic mice that express human SOD1 with a G93A mutation

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Breakdown of oxidized proteins as a part of secondary antioxidant defenses in mammalian cells.

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11 July 2018

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

1 reference

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11 July 2018

Molecular genetic basis of familial ALS.

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11 July 2018

Proteasome inhibition enhances the stability of mouse Cu/Zn superoxide dismutase with mutations linked to familial amyotrophic lateral sclerosis

1 reference

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11 July 2018

Motor neurons in Cu/Zn superoxide dismutase-deficient mice develop normally but exhibit enhanced cell death after axonal injury

1 reference

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11 July 2018

A gain-of-function of an amyotrophic lateral sclerosis-associated Cu,Zn-superoxide dismutase mutant: An enhancement of free radical formation due to a decrease in Km for hydrogen peroxide

1 reference

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11 July 2018

The Golgi apparatus of spinal cord motor neurons in transgenic mice expressing mutant Cu,Zn superoxide dismutase becomes fragmented in early, preclinical stages of the disease

1 reference

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11 July 2018

Transgenic mice carrying a human mutant superoxide dismutase transgene develop neuronal cytoskeletal pathology resembling human amyotrophic lateral sclerosis lesions

1 reference

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11 July 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=18805

11 July 2018

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

1 reference

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11 July 2018

Fragmentation of the Golgi apparatus of motor neurons in amyotrophic lateral sclerosis (ALS). Clinical studies in ALS of Guam and experimental studies in deafferented neurons and in beta,beta'-iminodipropionitrile axonopathy.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=18805

11 July 2018

Superoxide dismutase 1 with mutations linked to familial amyotrophic lateral sclerosis possesses significant activity

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=18805

11 July 2018

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

1 reference

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11 July 2018

Transgenic mice expressing an altered murine superoxide dismutase gene provide an animal model of amyotrophic lateral sclerosis

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Degradation of CFTR by the ubiquitin-proteasome pathway

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Ultrastructure of multiple microtubule initiation sites in mouse neuroblastoma cells

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Intermediate filaments and ubiquitin: a new thread in the understanding of chronic neurodegenerative diseases.

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Cytopathology of amyotrophic lateral sclerosis

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Characterization and dynamics of aggresome formation by a cytosolic GFP-chimera

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24 September 2018

Superoxide dismutase and neurofilament transgenic models of amyotrophic lateral sclerosis.

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24 September 2018

Specific aggregation of partially folded polypeptide chains: the molecular basis of inclusion body composition.

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24 September 2018

Intense superoxide dismutase-1 immunoreactivity in intracytoplasmic hyaline inclusions of familial amyotrophic lateral sclerosis with posterior column involvement.

1 reference

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Intracellular turnover of cystic fibrosis transmembrane conductance regulator. Inefficient processing and rapid degradation of wild-type and mutant proteins.

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24 September 2018

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

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24 September 2018

Presence of Cu/Zn superoxide dismutase (SOD) immunoreactivity in neuronal hyaline inclusions in spinal cords from mice carrying a transgene for Gly93Ala mutant human Cu/Zn SOD

1 reference

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12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Protein oxidative damage in a transgenic mouse model of familial amyotrophic lateral sclerosis

1 reference

https://pubmed.ncbi.nlm.nih.gov/11050163

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Neurofibrillary tangles in progressive supranuclear palsy brains exhibit immunoreactivity to frameshift mutant ubiquitin-B protein

1 reference

https://pubmed.ncbi.nlm.nih.gov/11050163

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Serial analysis of microtubules in cultured rat sensory axons

1 reference

https://pubmed.ncbi.nlm.nih.gov/11050163

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Neurofibrillary axonal swellings and amyotrophic lateral sclerosis

1 reference

https://pubmed.ncbi.nlm.nih.gov/11050163

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Molecular genetics of familial amyotrophic lateral sclerosis

1 reference

https://pubmed.ncbi.nlm.nih.gov/11050163

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Decreased zinc affinity of amyotrophic lateral sclerosis-associated superoxide dismutase mutants leads to enhanced catalysis of tyrosine nitration by peroxynitrite

1 reference

https://pubmed.ncbi.nlm.nih.gov/11050163

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Identifiers

10.1073/PNAS.220417997

1 reference

Europe PubMed Central

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1 November 2019

2000PNAS...9712571J

0 references

1 reference

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1 November 2019

1 reference

Europe PubMed Central

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1 November 2019

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