Overview |
bs-9527R-RBITC |
ALAS1 Polyclonal Antibody, RBITC Conjugated |
WB, IF(IHC-P) |
Human, Mouse, Rat |
Specifications |
RBITC |
Rabbit |
KLH conjugated synthetic peptide derived from human ALAS1/ALAS-H |
161-260/640 |
Polyclonal |
IgG |
1ug/ul |
Purified by Protein A. |
Aqueous buffered solution containing 0.01M TBS (pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles. |
Target |
211 |
mitochondrial; nonspecic; 5 aminolevulinate synthase; 5 aminolevulinate synthase nonspecic mitochondrial; 5 aminolevulinic acid synthase; 5-aminolevulinate synthase; 5-aminolevulinic acid synthase 1; Alas 1; ALAS 3; ALAS; ALAS H; ALAS N; ALAS-H; alaS1; ALAS3; ALASH; Aminolevulinate delta synthase 1; Aminolevulinic acid synthase 1; Delta ALA synthetase; Delta aminolevulinate synthase; Delta-ALA synthase 1; Delta-aminolevulinate synthase 1; HEM1_HUMAN; MIG 4; MIG4; Migration inducing protein 4. |
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. |
Application Dilution |
WB |
1:300-5000 |
IF(IHC-P) |
1:50-200 |