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

Dorfin prevents cell death by reducing mitochondrial localizing mutant superoxide dismutase 1 in a neuronal cell model of familial amyotrophic lateral sclerosis. Research Abstract Details 

Research Abstract Table of Contents

Jump to the:

Dorfin prevents cell death by reducing mitochondrial localizing mutant superoxide dismutase 1 in a neuronal cell model of familial amyotrophic lateral sclerosis. Abstract Text:

Hideyuki Takeuchi,Jun-ichi Niwa,Nozomi Hishikawa,Shinsuke Ishigaki,Fumiaki Tanaka,Manabu Doyu,Gen Sobue,

Abstract Dorfin is a RING-finger type ubiquitin ligase for mutant superoxide dismutase 1 (SOD1) that enhances its degradation. Mutant SOD1s cause familial amyotrophic lateral sclerosis (FALS) through the gain of unelucidated toxic properties. We previously showed that the accumulation of mutant SOD1 in the mitochondria triggered the release of cytochrome c, followed by the activation of the caspase cascade and induction of neuronal cell death. In the present study, therefore, we investigated whether Dorfin can modulate the level of mutant SOD1 in the mitochondria and subsequent caspase activation. We showed that Dorfin significantly reduced the amount of mutant SOD1 in the mitochondria, the release of cytochrome c and the activation of the following caspase cascade, thereby preventing eventual neuronal cell death in a neuronal cell model of FALS. These results suggest that reducing the accumulation of mutant SOD1 in the mitochondria may be a new therapeutic strategy for mutant SOD1-associated FALS, and that Dorfin may play a significant role in this.

Dorfin prevents cell death by reducing mitochondrial localizing mutant superoxide dismutase 1 in a neuronal cell model of familial amyotrophic lateral sclerosis. Publishing Authors By Initials

H Takeuchi,J Niwa,N Hishikawa,S Ishigaki,F Tanaka,M Doyu,G Sobue,

For similar enzymes and coenzymes: enzymes: oxidoreductases: superoxide dismutase research abstracts see: enzymes and coenzymes: enzymes: oxidoreductases: superoxide dismutase research

PUBMED ID PMID:

MEDLINE DATE:

Dorfin prevents cell death by reducing mitochondrial localizing mutant superoxide dismutase 1 in a neuronal cell model of familial amyotrophic lateral sclerosis. Journal Published:

PUBLICATION TYPE: Research Support, Non-U.S. Gov

Journal: Journal of neurochemistry

VOLUME: 89

Page Numbers: 64-72

Journal Abbreviation: J. Neurochem.

ISSN: 0022-3042

DAY: 8

MONTH: Apr

YEAR: 2004

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

Number of References:

LANGUAGE: eng

NlmUniqueID: 2985190

Dorfin prevents cell death by reducing mitochondrial localizing mutant superoxide dismutase 1 in a neuronal cell model of familial amyotrophic lateral sclerosis. Keywords Mesh Terms:

KEYWORDS: Superoxide Dismutase

MESH TERMS: metabolism

Chemical & Substance for Abstract: Dorfin prevents cell death by reducing mitochondrial localizing mutant superoxide dismutase 1 in a neuronal cell model of familial amyotrophic lateral sclerosis. Information

Substance Name: RNF19A protein, human

Registry Number: EC 6.3.2.-

Grant and Affiliation Information for Dorfin prevents cell death by reducing mitochondrial localizing mutant superoxide dismutase 1 in a neuronal cell model of familial amyotrophic lateral sclerosis.

AFFILIATION: Department of Neurology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya 466-8550, Japan.

Country: England

AGENCY:

GRANT:

ACRONYM:

MEDLINETA: J Neurochem

REFSOURCE:

DATABASENAME:

ACCESSION NUMBER:

Number Hits: 0

Dorfin prevents cell death by reducing mitochondrial localizing mutant superoxide dismutase 1 in a neuronal cell model of familial amyotrophic lateral sclerosis Related Publications

• A message from the New Editor-in-Chief.
• A novel centrosomal ring-finger protein, dorfin, mediates ubiquitin ligase activity.
• CAG repeat size correlates to electrophysiological motor and sensory phenotypes in SBMA.
• Cancer incidence and incidence rates in Japan in 2001 based on the data from 10 population-based cancer registries.
• Cisplatin neurotoxicity presenting as reversible posterior leukoencephalopathy syndrome.
• Clinicopathologic characteristics of five autopsied cases of dura mater-associated Creutzfeldt-Jakob disease.
• Contrast-enhanced 2D cine phase MR angiography for measurement of basilar artery blood flow in posterior circulation ischemia.
• Correlation between multiple phosphorylation of gizzard myosin light chains and actin-activated myosin ATPase activity.
• Cyclosporin A inhibits late steps of T lymphocyte activation after transmembrane signaling.
• Dobutamine stress test unmasks cardiac sympathetic denervation in Parkinson's disease.
• Dorfin localizes to Lewy bodies and ubiquitylates synphilin-1.
• Dorfin localizes to the ubiquitylated inclusions in Parkinson's disease, dementia with Lewy bodies, multiple system atrophy, and amyotrophic lateral sclerosis.
• Dorfin prevents cell death by reducing mitochondrial localizing mutant superoxide dismutase 1 in a neuronal cell model of familial amyotrophic lateral sclerosis.
• Dorfin ubiquitylates mutant SOD1 and prevents mutant SOD1-mediated neurotoxicity.
• Dual roles of myocardin-related transcription factors in epithelial mesenchymal transition via slug induction and actin remodeling.
• Isolation and characterization of a calmodulin binding fragment of chicken gizzard caldesmon.
• Multiple cerebellar infarcts: clinical and pathophysiologic features.
• Nonmyelinating Schwann cell involvement with well-preserved unmyelinated axons in Charcot-Marie-Tooth disease type 1A.
• Physical and functional interaction between Dorfin and Valosin-containing protein that are colocalized in ubiquitylated inclusions in neurodegenerative disorders.
• Pupillary supersensitivity and visual disturbance in Parkinson's disease.
• Pyramidal tract degeneration in sporadic Creutzfeldt-Jakob disease.
• Quantitative determinations of calmodulin in the supernatant and particulate fractions of mammalian tissues.
• Small neurons may be preferentially affected in ganglionopathy.
• The Japan cancer surveillance report: incidence of childhood, bone, penis and testis cancers.
• The video images of sleep attacks in Parkinson's disease.
• Usefulness of combined fractional anisotropy and apparent diffusion coefficient values for detection of involvement in multiple system atrophy.
• [Animal models of neurodegenerative diseases on the road to disease-modifying therapy: spinal and bulbar muscular atrophy]
• [Envisioning quality measurement systems of cancer care]
• [Gene expression profile of spinal ventral horn in ALS]
• [Neurodegenerative diseases and Dorfin]