ALAS1 Rabbit monoclonal Antibody | BS9823M

ALAS1 Rabbit monoclonal Antibody | BS9823M | Gentaur UK, US & Europe Distribution

Host: Rabbit

Reactivity: Human

Application: WB ICC/IF

Application Range: WB: 1:1000-1:5000 ICC/IF: 1:50-1:200

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.

Storage & Stability: Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.

Specificity: This Antibody detects endogenous levels of ALAS and does not cross-react with related proteins.

Molecular Weight: ~ 71 kDa

Note: For research use only, not for use in diagnostic procedure.

Alternative Names: 5-aminolevulinate synthase, nonspecific, mitochondrial; ALAS-H; 5-aminolevulinic acid synthase 1; Delta-ALA synthase 1; Delta-aminolevulinate synthase 1; ALAS1; ALAS3; ALASH; OK/SW-cl.121

Immunogen: Recombinant Antibody.

Conjugate: Unconjugated

Modification: Unmodification

Purification & Purity: Protein A affinity purified

Pathway: