Anti-ALAS2/ASB Antibody Picoband® Fluoro488 Conjugated
Product Specifications
Background
Delta-aminolevulinate synthase 2 also known as ALAS2 is a protein that in humans is encoded by the ALAS2 gene. The product of this gene specifies an erythroid-specific mitochondrially located enzyme. The encoded protein catalyzes the first step in the heme biosynthetic pathway. Defects in this gene cause X-linked pyridoxine-responsive sideroblastic anemia. Alternatively spliced transcript variants encoding different isoforms have been identified.
Synonyms
Endothelin-converting enzyme 1; ECE-1;3.4.24.71; ECE1
Gene Name
ALAS2
Gene ID
212
UniProt
P22557
Host
Rabbit
Reactivity
Human, Mouse, Rat
Cross Reactivity
No cross-reactivity with other proteins.
Immunogen
E.coli-derived human ALAS2/ASB recombinant protein (Position: M1-D190) .
Clonality
Polyclonal
Tissue Specificity
All isoforms are expressed in umbilical vein endothelial cells, polynuclear neutrophils, fibroblasts, atrium cardiomyocytes and ventricles. Isoforms A, B and C are also expressed in placenta, lung, heart, adrenal gland and phaeochromocytoma; isoforms A and C in liver, testis and small intestine; isoform B, C and D in endothelial cells and umbilical vein smooth muscle cells; isoforms C and D in saphenous vein cells, and isoform C in kidney. .
Applications
Flow Cytometry
Field of Research
Cancer, Cancer Metabolism, Cardiovascular, Growth Factors/Hormones, Metabolism, Metabolism Processes, Pathways and Processes, Response To Hypoxia, Signal Transduction, Vasculature
Purification
Immunogen affinity purified.
Form
Liquid
Function
Converts big endothelin-1 to endothelin-1. .
References & Citations
1. Aoki, Y., Urata, G., Takaku, F. Delta-aminolevulinic acid synthetase activity in erythroblasts of patients with primary sideroblastic anemia. Acta Haemat. Jpn. 36: 74-77, 1973. 2. Astner, I., Schulze, J. O., van den Heuvel, J., Jahn, D., Schubert, W.-D., Heinz, D. W. Crystal structure of 5-aminolevulinate synthase, the first enzyme of heme biosynthesis, and its link to XLSA in humans. EMBO J. 24: 3166-3177, 2005. 3. Astrin, K. H., Bishop, D. F. Assignment of human erythroid delta-aminolevulinate synthase (ALAS2) to the X chromosome. (Abstract) Cytogenet. Cell Genet. 51: 953-954, 1989.
Storage Conditions
At -20 ̊C for one year from date of receipt. Avoid repeated freezing and thawing. Protect from light.
Calculated Molecular Weight
87164 MW
Applications Notes
6
Gene Name Synonym
Endothelin-converting enzyme 1
Subcellular Location
Cell membrane; Single-pass type II membrane protein.
Protein Name
Endothelin-converting enzyme 1
Isotype
Rabbit IgG
Contents
Each vial contains 50% glycerol, 0.9% NaCl, 0.2% Na2HPO4, 0.02% NaN3.
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