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Article

Dorfin ubiquitylates mutant SOD1 and prevents mutant SOD1-mediated neurotoxicity.

The Journal of biological chemistry - 27 Sept 2002

Niwa Jun-Ichi, Ishigaki Shinsuke, Hishikawa Nozomi, Yamamoto Masahiko, Doyu Manabu, Murata Shigeo, Tanaka Keiji, Taniguchi Naoyuki, Sobue Gen

Abstract excerpt

Amyotrophic lateral sclerosis (ALS) is a progressive paralytic disorder resulting from the degeneration of motor neurons in the cerebral cortex, brainstem, and spinal cord. The cytopathological hallmark in the remaining motor neurons of ALS is the presence of ubiquitylated inclusions consisting of insoluble protein aggregates. In this paper we report that Dorfin, a RING finger-type E3 ubiquitin ligase, is...

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Identifiers

PMID: 12145308
DOI: 10.1074/jbc.M206559200

Genes

SOD1

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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Dorfin ubiquitylates mutant SOD1 and prevents mutant SOD1-mediated neurotoxicity.PMID 12145308Read ↗

Disulfide bond mediates aggregation, toxicity, and ubiquitylation of familial amyotrophic lateral sclerosis-linked mutant SOD1.Semantic proximity Read ↗

Dorfin-CHIP chimeric proteins potently ubiquitylate and degrade familial ALS-related mutant SOD1 proteins and reduce their cellular toxicity.Semantic proximity Read ↗

Dorfin prevents cell death by reducing mitochondrial localizing mutant superoxide dismutase 1 in a neuronal cell model of familial amyotrophic lateral sclerosis.Semantic proximity Read ↗

Dorfin ameliorates phenotypes in a transgenic mouse model of amyotrophic lateral sclerosis.Semantic proximity Read ↗

Dorfin Localizes to the Ubiquitylated Inclusions in Parkinson's Disease, Dementia with Lewy Bodies, Multiple System Atrophy, and Amyotrophic Lateral SclerosisSemantic proximity Read ↗

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Ataxin-3 regulates aggresome formation of copper-zinc superoxide dismutase (SOD1) by editing K63-linked polyubiquitin chains.Semantic proximity Read ↗

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