Function | Plays an essential role in the assembly of succinate dehydrogenase (SDH), an enzyme complex (also referred to as respiratory complex II) that is a component of both the tricarboxylic acid (TCA) cycle and the mitochondrial electron transport chain, and which couples the oxidation of succinate to fumarate with the reduction of ubiquinone (coenzyme Q) to ubiquinol. Promotes maturation of the iron-sulfur protein subunit SDHB of the SDH catalytic dimer, protecting it from the deleterious effects of oxidants. May act together with SDHAF3. Contributes to iron-sulfur cluster incorporation into SDHB by binding to SDHB and recruiting the iron-sulfur transfer complex formed by HSC20, HSPA9 and ISCU through direct binding to HSC20. |
Protein Name | Succinate Dehydrogenase Assembly Factor 1 - MitochondrialSdh Assembly Factor 1Sdhaf1Lyr Motif-Containing Protein 8 |
Database Links | Reactome: R-HSA-9854311 |
Cellular Localisation | Mitochondrion Matrix |
Alternative Antibody Names | Anti-Succinate Dehydrogenase Assembly Factor 1 - Mitochondrial antibodyAnti-Sdh Assembly Factor 1 antibodyAnti-Sdhaf1 antibodyAnti-Lyr Motif-Containing Protein 8 antibodyAnti-SDHAF1 antibodyAnti-LYRM8 antibody |
Information sourced from Uniprot.org