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Article

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

Neurobiology of disease - 1 Feb 2007

Ishigaki Shinsuke, Niwa Jun-ichi, Yamada Shin-ichi, Takahashi Miho, Ito Takashi, Sone Jun, Doyu Manabu, Urano Fumihiko, Sobue Gen

Abstract excerpt

The ubiquitin-proteasome system (UPS) is involved in the pathogenetic mechanisms of neurodegenerative disorders, including amyotrophic lateral sclerosis (ALS). Dorfin is a ubiquitin ligase (E3) that degrades mutant SOD1 proteins, which are responsible for familial ALS. Although Dorfin has potential as an anti-ALS molecule, its life in cells is short. To improve its stability and enhance its E3 activity, we...

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Source

Identifiers

PMID: 17157513
DOI: 10.1016/j.nbd.2006.09.017

Genes

SOD1

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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Dorfin-CHIP chimeric proteins potently ubiquitylate and degrade familial ALS-related mutant SOD1 proteins and reduce their cellular toxicity.PMID 17157513Read ↗

Dorfin ubiquitylates mutant SOD1 and prevents mutant SOD1-mediated neurotoxicity.Semantic proximity Read ↗

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

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

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Emerging role and mechanism of HACE1 in the pathogenesis of neurodegenerative diseases: A promising targetSemantic proximity Read ↗

C-terminus of Hsp70 Interacting Protein (CHIP) and Neurodegeneration: Lessons from the Bench and Bedside.Semantic proximity Read ↗

CHIP promotes proteasomal degradation of familial ALS-linked mutant SOD1 by ubiquitinating Hsp/Hsc70.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 ↗

Most mutations that cause spinocerebellar ataxia autosomal recessive type 16 (SCAR16) destabilize the protein quality-control E3 ligase CHIP.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 ↗

Regulation of protein quality control by UBE4B and LSD1 through p53-mediated transcription.Semantic proximity Read ↗

Co-chaperone CHIP stabilizes aggregate-prone malin, a ubiquitin ligase mutated in Lafora disease.Semantic proximity Read ↗

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