epitope corresponding to amino acids 57-210 mapping near the N-terminus of ALAS-H of human origin
recommended for detection of precursor and mature ALAS-H of human and, to a lesser extent, mouse and rat origin by WB, IP, IF, IHC(P) and ELISA; also reactive with additional species, including equine and canine
ALAS-H Background Information 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.
ALAS-H (H-154)
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ALAS-H (H-154): sc-50531. Western blot analysis of ALAS-H expression in K-562 (A), HEL 92.1.7 (B) and Hep G2 (C) whole cell lysates.
ALAS-H (H-154): sc-50531. Immunoperoxidase staining of formalin fixed, paraffin-embedded human adrenal gland tissue showing cytoplasmic staining of cortical cells at low (A) and high (B) magnification. Kindly provided by The Swedish Human Protein Atlas (HPA) program.
ALAS-H (H-154): sc-50531. Western blot analysis of ALAS-H expression in non-transfected: sc-117752 (A) and mouse ALAS-H transfected: sc-118326 (B) 293T whole cell lysates.
ALAS-H (H-154): sc-50531. Immunofluorescence staining of formalin-fixed Hep G2 cells showing mitochondrial localization.
