ALAS2 antibody

Este producto es parte de ALAS -5-aminolevulinate synthase erythroid (specific) mitochondrial (erythroid)
ALAS2 antibody
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Name
ALAS2 antibody
Category
Primary Antibodies
Provider
FineTest
Reference
FNab00277
Tested Applications
ELISA, WB

Documentos del producto

Instrucciones
Descargar
Data sheet
Descargar

Especificaciones del producto

Category
Primary Antibodies
Immunogen Target
aminolevulinate, delta-, synthase 2 (ALAS2)
Host
Rabbit
Reactivity
Human, Mouse, Rat
Recommended Dilution
WB: 1:200-1:2000
Clonality
polyclonal
Conjugation
Unconjugated
Isotype
IgG
Observed MW
49 kDa, 60 kDa
Purity
≥95% as determined by SDS-PAGE
Purification
Immunogen affinity purified
Size 1
100µg
Form
liquid
Tested Applications
ELISA, WB
Storage
PBS with 0.02% sodium azide and 50% glycerol pH 7.3, -20℃ for 12 months(Avoid repeated freeze / thaw cycles.)
UniProt ID
P22557
Gene ID
212
Alias
ALAS2,ALASE,ASB
Background
Antibody anti-ALAS2
Status
RUO
Note
Mol. Weight 49 kDa, 60 kDa

Background

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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