Application | Comment | Organism |
---|---|---|
molecular biology | the maltose-binding protein-tagged beta-subunit of succinyl-CoA synthetase, SUCLA2, bound to an amylose resin, is used as an affinity ligand to bind FPLC-purified wild-type and some mutant erythroid-specific aminolevulinic acid synthases, ALAS2 | Homo sapiens |
Cloned (Comment) | Organism |
---|---|
expression of the maltose-binding protein-tagged beta-subunit of succinyl-CoA synthetase, SUCLA2, in Escherichia coli strain Top10 | Homo sapiens |
Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
mitochondrion | - |
Homo sapiens | 5739 | - |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
additional information | Homo sapiens | the enzyme beta-subunit binds strongly to wild-type erythroid-specific aminolevulinic acid synthase, but not to the mutants M567V and S568G, aminolevulinic acid synthase mutant R452C shows binding to the succinyl-CoA synthetase, but with reduced affinity and positive cooperativity for succinyl-CoA | ? | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | Q9P2R7 | beta-subunit | - |
Purification (Comment) | Organism |
---|---|
recombinant maltose-binding protein-tagged beta-subunit of succinyl-CoA synthetase, SUCLA2, from Escherichia coli strain Top10 by amylose affinity chromatography and gel filtration | Homo sapiens |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
erythroid cell | - |
Homo sapiens | - |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
additional information | the enzyme beta-subunit binds strongly to wild-type erythroid-specific aminolevulinic acid synthase, but not to the mutants M567V and S568G, aminolevulinic acid synthase mutant R452C shows binding to the succinyl-CoA synthetase, but with reduced affinity and positive cooperativity for succinyl-CoA | Homo sapiens | ? | - |
? |
Synonyms | Comment | Organism |
---|---|---|
Succinyl-CoA synthetase | - |
Homo sapiens |
SUCLA2 | - |
Homo sapiens |
General Information | Comment | Organism |
---|---|---|
additional information | C-terminal mutations in the erythroid-specific aminolevulinic acid synthase gene ALAS2 cause loss of binding to the beta-subunit of succinyl-CoA synthetase, SUCLA2, resulting in reduced mitochondrial enzymatic activity and X-linked sideroblastic anemia | Homo sapiens |
physiological function | probable role of an aminolevulinic acid synthase ALAS2-succinyl-CoA synthetase complex in the regulation of erythroid heme biosynthesis | Homo sapiens |