| 5 aminolevulinate synthase antibody, 5 aminolevulinate synthase nonspecific mitochondrial antibody, 5 aminolevulinic acid synthase antibody, 5-aminolevulinate synthase antibody, 5-aminolevulinic acid synthase 1 antibody, Alas 1 antibody, ALAS 3 antibody, ALAS antibody, ALAS H antibody, ALAS HOUSEKEEPING TYPE antibody, ALAS N antibody, ALAS-H antibody, alaS1 antibody, ALAS3 antibody, ALASH antibody, Aminolevulinate delta synthase 1 antibody, Aminolevulinic acid synthase 1 antibody, Delta ALA synthetase antibody, Delta aminolevulinate synthase antibody, Delta-ALA synthase 1 antibody, Delta-aminolevulinate synthase 1 antibody, HEM1_HUMAN antibody, MIG 4 antibody, MIG4 antibody, Migration inducing protein 4 antibody, mitochondrial antibody, nonspecific antibody |
| 5-aminolevulinate synthase 1 (ALAS-H) and 2 (ALAS-E) are two isoforms of ALAS, an enzyme catalyzing the first step of the heme biosynthetic pathway in mammals. The erythroid-specific isoenzyme, ALAS-E, regulates the first step of hematopoietic cell differentation and iron metabolism in the liver. ALAS-H is a housekeeping protein which mediates synthesis of early heme in the mitochondria of most cells. Succinyl CoA associates with ALAS-E in protein conformation change and translocation of ALAS-E into the mitochondria and does not interact with ALAS-H. The ALAS-E 5'-flanking region contains binding sites for nuclear activators such as GATA-1, NF-E2 and EKLF. Since the ALAS gene maps to the X chromosome, mutation of the gene leads to the pyridoxine-refractory X-linked sideroblastic anemia. |
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