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Cat. Number:

bs-9527R-AF488

Quantity size:

100ul

Concentration:

1mg/ml  Buffer = 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

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.

Also known as:

mitochondrial; nonspecific; 5 aminolevulinate synthase; 5 aminolevulinate synthase nonspecific 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.

Specificity:

Storage:

0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.

Application:

Excitation spectrum: 495nm
Emission spectrum: 519nm
Not yet tested in other applications.
Optimal working dilutions must be determined by the end user.

Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.