ABS2121

Anti-ALAS-E

from rabbit

• Synonyme(s) : Erythroid-specific 5-aminolevulinate synthase mitochondrial, EC: 2.3.1.37, ALAS-E, 5-aminolevulinic acid synthase 2, Delta-ALA synthase 2, Delta-aminolevulinate synthase 2
• Code UNSPSC : 12352203
• eCl@ss : 32160702
• Nomenclature NACRES : NA.41

Propriétés

• Source biologique: rabbit
• Niveau de qualité: 100
• Forme d'anticorps: unpurified
• Type de produit anticorps: primary antibodies
• Clone: polyclonal
• Espèces réactives: human
• Conditionnement: antibody small pack of 25 μL
• Technique(s): western blot: suitable
• Isotype: IgG
• Numéro d'accès NCBI: NP_000023
• Numéro d'accès UniProt: P22557
• Conditions d'expédition: ambient
• Modification post-traductionnelle de la cible: unmodified
• Informations sur le gène: human ... ALAS2(212)

Description

Description générale

5-aminolevulinate synthase, erythroid-specific, mitochondrial (UniProt: P22557; also known as EC: 2.3.1.37, ALAS-E, 5-aminolevulinic acid synthase 2, ALAS2, Delta-ALA synthase 2, Delta-aminolevulinate synthase 2) is encoded by the ALAS2 (also known as ALASE, ASB) gene (Gene ID: 212) in human. Two forms of ALAS have been described in literature; ALAS1 (ALAS-H) and ALAS2 (ALAS-E). ALAS-H is a housekeeping protein, which mediates synthesis of early heme in the mitochondria of most cells. ALAS-E is a mitochondrial matrix homodimeric enzyme that is also involved in catalyzing the first step in heme biosynthetic pathway. Four isoforms of ALAS-E have been described that are generated by alternate splicing. ALAS-E is synthesized with a transit peptide (aa 1-49) that is cleaved to generate a mature form (aa 50-587). Defects in ALAS2 gene are linked to sideroblastic anemia that is characterized by anemia, hypochromic peripheral erythrocytes, systemic iron overload, and by the presence of bone marrow ringed sideroblasts. Defects in ALAS2 gene are also reported in X-linked dominant form of erythropoietic protoporphyria that is characterized by accumulation and increased excretion of porphyrins or their precursors.

Spécificité

This rabbit polyclonal antibody detects erythroid-specifc 5-aminolevulinate synthase, mitochondrial in human cells.

Immunogène

Epitope: unknown

Full length recombinant human 5-aminolevulinate synthase.

Application

Anti-ALAS-E, Cat. No. ABS2121, is a rabbit polyclonal antibody that detects 5-aminolevulinate synthase, erythroid-specific, mitochondrial and has been tested for use in Western Blotting.

Research Category
Signaling

Western Blotting Analysis: A 1:1,000 dilution from a representative lot detected ALAS-E in crude mitochondrial lysate differentiated from DS19 cells.

Western Blotting Analysis: A 1:10,000-25,000 dilution from a representative lot detected ALAS-E in recombinant hALAS isoforms over expressed in E Coli and HEK293T cells transfected with hALAS2 (Courtesy of Dr. Harry A. Dailey at University of Georgia).

Qualité

Evaluated by Western Blotting with recombinant hALAS2

Western Blotting Analysis: A 1:2,000 dilution of this antibody detected recombinant ALAS-E.

Description de la cible

~58 and 72 kDa observed; Detects both the precusor and mature forms of human ALAS2. Uncharacterized bands may be observed in some lysate(s).

Forme physique

Format: Unpurified

Rabbit polyclonal antiserum with 0.05% sodium azide.

Unpurified

Stockage et stabilité

Stable for 1 year at -20°C from date of receipt.

Handling Recommendations: Upon receipt and prior to removing the cap, centrifuge the vial and gently mix the solution. Aliquot into microcentrifuge tubes and store at -20°C. Avoid repeated freeze/thaw cycles, which may damage IgG and affect product performance.

Autres remarques

Concentration: Please refer to lot specific datasheet.

Clause de non-responsabilité

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.