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

By J. A. Johnston, Muffie J. Dalton, M. Gurney, R. Kopito. Proceedings of the National Academy of Sciences of the United States of America, 2000

635 citations recorded by the source corpus.

Subfields: Genetics, Neurology

View Paper: https://www.semanticscholar.org/paper/95810b9e62703452ac2201f827c60a4b911e0175

How researchers see this paper

The researchers whose trusted networks rank it highest, strongest advocate first. Every researcher gets a different order.

In Genetics

1. Robert H. Brown
2. Jeffrey D. Rothstein
3. P. Pasinelli
4. D. Cleveland

In Neurology

1. Robert H. Brown
2. Jeffrey D. Rothstein
3. P. Shaw
4. P. Pasinelli
5. S. Barber
6. D. Cleveland
7. Aaron Ciechanover

Citations in the corpus

• The ubiquitin-proteasome proteolytic pathway: destruction for the sake of construction. (2002, background): “While the initial hypothesis was that inclusion bodies are generated because of the inherent tendency of the abnormal proteins to associate with one another and aggregate, it is now thought that the process may be more complex and involves active cellular machineries (99, 212, 213), including inhibi”
• Amyotrophic lateral sclerosis is a distal axonopathy: evidence in mice and man. (2004, background): “Accumulation of insoluble complexes of mutant SOD1 protein (Johnston et al., 2000) or chronic glutamate toxicity (Rothstein et al., 1993) could be responsible for insufficient maintenance of the distal axon, leading to early denervation at the NMJ while the cell body remains structurally intact.”
• From charcot to lou gehrig: deciphering selective motor neuron death in als (2001, background): “Moreover, misfolded SOD1 aggregates that cannot be readily dissociated, even with strong detergents and reducing agents, can be detected biochemicall”
• The deacetylase HDAC6 regulates aggresome formation and cell viability in response to misfolded protein stress. (2003, background): “This conclusion would also predict the existence of specific adaptor(s) for loading the acetylation beyond histone metabolism and gene transcription. nonubiquitinated misfolded protein cargo, which are also likely associated with neurodegenerative diseases (Johnston et al., 2000). proteasomes (2).”
• Oxidative stress in ALS: key role in motor neuron injury and therapeutic target. (2010, background): “mutant SOD1 transgenic mice [53,87,147,148] and cell culture models [149,150].”
• Molecular biology of amyotrophic lateral sclerosis: insights from genetics (2006, background): “In these transgenic mice, these aggregates become evident by the time of disease onse”
• Paradigm shifts in Alzheimer's disease and other neurodegenerative disorders: The emerging role of oligomeric assemblies (2002, neutral): “This type of de-stabilization could facilitate SOD1 unfolding and lead to pathologic assembly of the enzyme, a process that could explain the formation of SOD aggregates observed in transgenic mice (Bruijn et al., 1998; Johnston et al., 2000).”
• ASK1 is essential for endoplasmic reticulum stress-induced neuronal cell death triggered by expanded polyglutamine repeats. (2002, methodology): “Blots were probed with antibodies to JNK1 (Santa Cruz), phospho-JNK (Cell Signaling), ASK1, phospho-ASK, and CHOP.”