ArticleJournal of neurochemistry2010
An examination of alpha B-crystallin as a modifier of SOD1 aggregate pathology and toxicity in models of familial amyotrophic lateral sclerosis.
Article in Journal of neurochemistry, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Who cites it
16 citing papers in PubMed, 21 citations in OpenAlex.
- The Role of Small Heat Shock Proteins in Protein Misfolding Associated Motoneuron Diseases.International journal of molecular sciences · 2022Review
- AAV2/9-mediated overexpression of MIF inhibits SOD1 misfolding, delays disease onset, and extends survival in mouse models of ALS.Proceedings of the National Academy of Sciences of the United States of America · 2019Article
- Endogenous macrophage migration inhibitory factor reduces the accumulation and toxicity of misfolded SOD1 in a mouse model of ALS.Proceedings of the National Academy of Sciences of the United States of America · 2016Article
- Substantially elevating the levels of αB-crystallin in spinal motor neurons of mutant SOD1 mice does not significantly delay paralysis or attenuate mutant protein aggregation.Journal of neurochemistry · 2015Article
- Macrophage migration inhibitory factor as a chaperone inhibiting accumulation of misfolded SOD1.Neuron · 2015Article
- Small heat-shock proteins: important players in regulating cellular proteostasis.Cellular and molecular life sciences : CMLS · 2015Review
- Conformational specificity of the C4F6 SOD1 antibody; low frequency of reactivity in sporadic ALS cases.Acta neuropathologica communications · 2014Article
- Analysis of mutant SOD1 electrophoretic mobility by Blue Native gel electrophoresis; evidence for soluble multimeric assemblies.PloS one · 2014Article
- The α crystallin domain of small heat shock protein b8 (Hspb8) acts as survival and differentiation factor in adult hippocampal neurogenesis.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2013Article
- The small heat shock proteins αB-crystallin and Hsp27 suppress SOD1 aggregation in vitro.Cell stress & chaperones · 2013Article
- The anabolic/androgenic steroid nandrolone exacerbates gene expression modifications induced by mutant SOD1 in muscles of mice models of amyotrophic lateral sclerosis.Pharmacological research · 2012Article
- Study of αB-crystallin expression in Gerbil BCAO model of transient global cerebral ischemia.Oxidative medicine and cellular longevity · 2012Article
- Role of disulfide cross-linking of mutant SOD1 in the formation of inclusion-body-like structures.PloS one · 2012Article
- Superoxide dismutase 1 encoding mutations linked to ALS adopts a spectrum of misfolded states.Molecular neurodegeneration · 2011Article
- An examination of wild-type SOD1 in modulating the toxicity and aggregation of ALS-associated mutant SOD1.Human molecular genetics · 2010Article
- Analysis of chaperone mRNA expression in the adult mouse brain by meta analysis of the Allen Brain Atlas.PloS one · 2010Article
Corrections and comments
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Authors and funding
2 authors at 1 institution in 1 country.
Funding
Abstract
Amyotrophic lateral sclerosis is a progressively paralytic neurodegenerative disease that can be caused by mutations in Cu,Zn-superoxide dismutase 1 (SOD1). Transgenic mice that over-express mutant SOD1 develop paralysis and accumulate aggregates of mutant protein in the brainstem and spinal cord. The present study uses a cell culture model to demonstrate alpha B-crystallin is capable of reducing aggregation of mutant SOD1. To test the role of alpha B-crystallin in modulating SOD1 aggregation in vivo, alpha B-crystallin deficient mice were bred to mice expressing two different SOD1 mutants (G37R and L126Z). Although completely eliminating alpha B-crystallin reduced the interval to disease endstage by 20-30 days in mice expressing either mutant, there were no detectable changes in the levels of sedimentable, SOD1 aggregates in the spinal cord of symptomatic mice. Because alpha B-crystallin is most abundantly expressed in muscle, we expected that the loss of this chaperone would leave this tissue vulnerable to mutant SOD1 aggregation. However, there was no evidence of mutant SOD1 aggregation in the muscle of mice lacking alpha B-crystallin. Our findings indicate that a significant perturbation to the protein homeostasis network of muscle is not sufficient to induce the aggregation of misfolded mutant SOD1. These outcomes have implications regarding the role of chaperones in modulating the tissue specific accumulations of misfolded SOD1.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.