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Rabbit Anti-ALAS1/FITC Conjugated antibody
background:
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.
Subunit:
Homodimer.
Subcellular Location:
Mitochondrion matrix
Similarity:
Belongs to the class-II pyridoxal-phosphate-dependent aminotransferase family.
Database links:
Entrez Gene: 211 Human
Entrez Gene: 11655 Mouse
Entrez Gene: 65155 Rat
Omim: 125290 Human
SwissProt: P13196 Human
SwissProt: Q8VC19 Mouse
SwissProt: P13195 Rat
Unigene: 476308 Human
Unigene: 290578 Mouse
Unigene: 97126 Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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