5-Aminolevulinate Synthase, Erythroid-Specific, Mitochondrial (ALAS2) Antibody
195€ (20 µl)
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935106861
info@markelab.com
name
5-Aminolevulinate Synthase, Erythroid-Specific, Mitochondrial (ALAS2) Antibody
category
Primary Antibodies
provider
Abbexa
reference
abx005002
tested applications
ELISA, WB
Description
ALAS2 Antibody is a Rabbit Polyclonal antibody against ALAS2. 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.
Documents del producto
Instrucciones
Data sheet
Product specifications
| Category | Primary Antibodies |
| Immunogen Target | 5-Aminolevulinate Synthase, Erythroid-Specific, Mitochondrial (ALAS2) |
| Host | Rabbit |
| Reactivity | Human, Mouse, Rat |
| Recommended Dilution | ELISA: 1 µg/ml, WB: 1/500 - 1/2000. Optimal dilutions/concentrations should be determined by the end user. |
| Clonality | Polyclonal |
| Conjugation | Unconjugated |
| Isotype | IgG |
| Purification | Purified by affinity chromatography. |
| Size 1 | 20 µl |
| Size 2 | 100 µl |
| Size 3 | 2 × 100 µl |
| Form | Liquid |
| Tested Applications | ELISA, WB |
| Buffer | PBS, pH 7.3, containing 0.02% sodium azide, 50% glycerol. |
| Availability | Shipped within 5-10 working days. |
| Storage | Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles. |
| Dry Ice | No |
| UniProt ID | P22557 |
| Gene ID | 212 |
| NCBI Accession | NP_001033057.1 |
| Alias | ALAS2,ALASE,ASB |
| Background | Antibody anti-ALAS2 |
| Status | RUO |
| Note | Concentration: > 0.2 mg/ml - |
Descripción
5-Aminolevulinate Synthase 2 (ALAS2) is the erythroid-specific isoform of the enzyme responsible for initiating heme biosynthesis in red blood cell precursors. ALAS2 catalyzes the synthesis of 5-aminolevulinic acid (ALA) from glycine and succinyl-CoA within mitochondria, providing the precursors for hemoglobin synthesis during erythropoiesis. ALAS2 expression is regulated by erythropoietin and iron availability through the iron-responsive element in its mRNA, ensuring heme production is synchronized with hemoglobin synthesis. Mutations in the ALAS2 gene can result in X-linked sideroblastic anemia, a disorder characterized by defective heme production, iron accumulation in mitochondria, and impaired red blood cell function. Enhanced understanding of ALAS2 regulation and activity has therapeutic implications for treating anemia and disorders involving disrupted erythropoiesis, such as thalassemia and myelodysplastic syndromes.
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