| IED ID | IndEnz0002010796 |
| Enzyme Type ID | protease010796 |
| Protein Name |
Succinate dehydrogenase assembly factor 1, mitochondrial SDH assembly factor 1 SDHAF1 LYR motif-containing protein 8 |
| Gene Name | SDHAF1 LYRM8 |
| Organism | Homo sapiens (Human) |
| Taxonomic Lineage | cellular organisms Eukaryota Opisthokonta Metazoa Eumetazoa Bilateria Deuterostomia Chordata Craniata Vertebrata Gnathostomata (jawed vertebrates) Teleostomi Euteleostomi Sarcopterygii Dipnotetrapodomorpha Tetrapoda Amniota Mammalia Theria Eutheria Boreoeutheria Euarchontoglires Primates Haplorrhini Simiiformes Catarrhini Hominoidea (apes) Hominidae (great apes) Homininae Homo Homo sapiens (Human) |
| Enzyme Sequence | MSRHSRLQRQVLSLYRDLLRAGRGKPGAEARVRAEFRQHAGLPRSDVLRIEYLYRRGRRQLQLLRSGHATAMGAFVRPRAPTGEPGGVGCQPDDGDSPRNPHDSTGAPETRPDGR |
| Enzyme Length | 115 |
| Uniprot Accession Number | A6NFY7 |
| Absorption | |
| Active Site | |
| Activity Regulation | |
| Binding Site | |
| Calcium Binding | |
| catalytic Activity | |
| DNA Binding | |
| EC Number | |
| Enzyme Function | 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 (PubMed:24954417, PubMed:19465911). Promotes maturation of the iron-sulfur protein subunit SDHB of the SDH catalytic dimer, protecting it from the deleterious effects of oxidants (PubMed:24954417). May act together with SDHAF3 (PubMed:24954417). 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 (PubMed:26749241). {ECO:0000269|PubMed:19465911, ECO:0000269|PubMed:24954417, ECO:0000269|PubMed:26749241}. |
| Temperature Dependency | |
| PH Dependency | |
| Pathway | |
| nucleotide Binding | |
| Features | Chain (1); Compositional bias (1); Motif (2); Mutagenesis (2); Natural variant (6); Region (2); Sequence conflict (2) |
| Keywords | Chaperone;Disease variant;Mitochondrion;Primary mitochondrial disease;Reference proteome;Repeat |
| Interact With | Q9UHD4; Q8IWL3; Q7Z3Y8; P21912 |
| Induction | |
| Subcellular Location | SUBCELLULAR LOCATION: Mitochondrion matrix {ECO:0000305|PubMed:19465911}. |
| Modified Residue | |
| Post Translational Modification | |
| Signal Peptide | |
| Structure 3D | |
| Cross Reference PDB | - |
| Mapped Pubmed ID | 23174333; |
| Motif | MOTIF 14..16; /note=LYR motif 1; required for interaction with HSC20; /evidence=ECO:0000269|PubMed:26749241; MOTIF 53..55; /note=LYR motif 2; not required for interaction with HSC20; /evidence=ECO:0000269|PubMed:26749241 |
| Gene Encoded By | |
| Mass | 12,806 |
| Kinetics | |
| Metal Binding | |
| Rhea ID | |
| Cross Reference Brenda |