5-Aminolevulinate Synthase, Erythroid-Specific, Mitochondrial (ALAS2) Antibody

Este producto es parte de ALAS -5-aminolevulinate synthase erythroid (specific) mitochondrial (erythroid)
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169€ (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
abx322140
tested applications
ELISA, WB, IHC

Description

ALAS2 Antibody is a Rabbit Polyclonal against ALAS2.

Documents del producto

Instrucciones
Data sheet
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Product specifications

CategoryPrimary Antibodies
Immunogen Target5-Aminolevulinate Synthase, Erythroid-Specific, Mitochondrial (ALAS2)
HostRabbit
ReactivityHuman, Mouse
Recommended DilutionWB: 1/200 - 1/1000, IHC: 1/20 - 1/200. Optimal dilutions/concentrations should be determined by the end user.
ClonalityPolyclonal
ConjugationUnconjugated
IsotypeIgG
PurificationAntigen Affinity Chromatography.
Size 120 µl
Size 250 µl
Size 3100 µl
Size 4200 µl
Size 51 ml
FormLiquid
Tested ApplicationsELISA, WB, IHC
BufferPBS, pH 7.3, containing 0.02% sodium azide and 50% glycerol.
AvailabilityShipped within 5-10 working days.
StorageAliquot and store at -20°C. Avoid repeated freeze/thaw cycles.
Dry IceNo
UniProt IDP22557
Gene ID212
NCBI AccessionNP_000023.2, NM_000032.4
OMIM300751
AliasALAS2,ALASE,ASB
BackgroundAntibody anti-ALAS2
StatusRUO

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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