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Literature summary for 5.3.4.1 extracted from

  • Walker, A.K.; Farg, M.A.; Bye, C.R.; McLean, C.A.; Horne, M.K.; Atkin, J.D.
    Protein disulphide isomerase protects against protein aggregation and is S-nitrosylated in amyotrophic lateral sclerosis (2010), Brain, 133, 105-116.
    View publication on PubMed

Application

Application Comment Organism
pharmacology protein disulfide isomerase is a potential therapeutic target in amyotrophic lateral sclerosis and (+-)-trans-1,2-bis(mercaptoacetamido)cyclohexane and other molecular mimics of protein disulfide isomerase could be of benefit in amyotrophic lateral sclerosis and other neurodegenerative diseases related to protein misfolding Homo sapiens

Cloned(Commentary)

Cloned (Comment) Organism
recombinant constitutive overexpression in Mus musculus NSC-34 cells and neuroblastoma Neuro2a cells. PDI overexpression decreases mutant SOD1-induced cell death, overview Homo sapiens

Inhibitors

Inhibitors Comment Organism Structure
additional information functional inhibition of protein disulfide isomerase by S-nitrosylation Homo sapiens

Localization

Localization Comment Organism GeneOntology No. Textmining
endoplasmic reticulum
-
Homo sapiens 5783
-

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
brain
-
Homo sapiens
-
spinal cord lumbar, segments Homo sapiens
-

Synonyms

Synonyms Comment Organism
Protein disulphide isomerase
-
Homo sapiens

Expression

Organism Comment Expression
Homo sapiens the enzyme is upregulated in amyotrophic lateral sclerosis up

General Information

General Information Comment Organism
malfunction functional inhibition of protein disulfide isomerase by S-nitrosylation may contribute to pathophysiology in both mutant superoxide dismutase 1-linked disease and sporadic amyotrophic lateral sclerosis Homo sapiens
physiological function endoplasmic reticulum stress is important in the formation of mutant superoxide dismutase 1 inclusions, and protein disulfide isomerase has an important function in ameliorating mutant superoxide dismutase 1 aggregation and toxicity Homo sapiens