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Article

Dorfin ameliorates phenotypes in a transgenic mouse model of amyotrophic lateral sclerosis.

Journal of neuroscience research - 1 Jan 2010

Sone Jun, Niwa Jun-ichi, Kawai Kaori, Ishigaki Shinsuke, Yamada Shin-ichi, Adachi Hiroaki, Katsuno Masahisa, Tanaka Fumiaki, Doyu Manabu, Sobue Gen

Abstract excerpt

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that is characterized by progressive motor neuron degeneration and leads to death within a few years of diagnosis. One of the pathogenic mechanisms of ALS is proposed to be a dysfunction in the protein quality-control machin...

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Source

Identifiers

PMID: 19610091
DOI: 10.1002/jnr.22175

Genes

SOD1

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 2 Sept 2026. Prepared record publication-19610091-2026-09-02.2. Manifest publication-graph-cohort-2026-09-02.2.

Source receipt: PubMed via Helena Literature Corpus, retrieved 2 Sept 2026.

Related research

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Dorfin ameliorates phenotypes in a transgenic mouse model of amyotrophic lateral sclerosis.PMID 19610091Read ↗

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

FUDNC1-dependent mitophagy ameliorate motor neuron death in an amyotrophic lateral sclerosis mouse modelSemantic 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 ↗

[Exploration of pathogenesis-associated molecules and development of disease models for sporadic ALS].Semantic proximity Read ↗

From Mouse Models to Human Disease: An Approach for Amyotrophic Lateral Sclerosis.Semantic proximity Read ↗

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

DJ-1 knockout augments disease severity and shortens survival in a mouse model of ALS.Semantic proximity Read ↗

DDX3X overexpression decreases dipeptide repeat proteins in a mouse model of C9ORF72-ALS/FTDSemantic proximity Read ↗

Expression of amyotrophic lateral sclerosis associated protein disulfide isomerase A3 D217N variant recapitulates early morphological alterations at the neuromuscular junction.Semantic proximity Read ↗

ALS-linked protein disulfide isomerase variants cause motor dysfunction.Semantic proximity Read ↗

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