ALAS1 (clone ), anti-human
€428.00
In stock
SKU
BS9823M
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.
Alternative Name:
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
Application Dilution: WB: 1:1000-1:5000, ICC/IF: 1:50-1:200
Specificity: This antibody detects endogenous levels of ALAS and does not cross-react with related proteins.
Immunogen:
Recombinant antibody.
MW: ~ 71 kDa
Swis Prot.: P13196
Purification & Purity:
Protein A affinity purified
Format:
Recombinant Rabbit Monoclonal Antibody. 1*TBS (pH7.4), 1%BSA, 40%Glycerol. Preservative: 0.05% Sodium Azide.
Storage:
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
For research use only, not for use in diagnostic procedure.
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.
Alternative Name:
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
Application Dilution: WB: 1:1000-1:5000, ICC/IF: 1:50-1:200
Specificity: This antibody detects endogenous levels of ALAS and does not cross-react with related proteins.
Immunogen:
Recombinant antibody.
MW: ~ 71 kDa
Swis Prot.: P13196
Purification & Purity:
Protein A affinity purified
Format:
Recombinant Rabbit Monoclonal Antibody. 1*TBS (pH7.4), 1%BSA, 40%Glycerol. Preservative: 0.05% Sodium Azide.
Storage:
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
For research use only, not for use in diagnostic procedure.
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