Anti-ALAS-E (C15-1317-808)

General description

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.

Specificity

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

Immunogen

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).

Quality

Evaluated by Western Blotting with recombinant hALAS2

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

Target description

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

Physical form

Format: Unpurified

Rabbit polyclonal antiserum with 0.05% sodium azide.

Unpurified

Storage and Stability

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.

Other Notes

Concentration: Please refer to lot specific datasheet.

Disclaimer

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.

biological source: rabbit. Quality Level: 100. antibody form: unpurified. antibody product type: primary antibodies. clone: polyclonal. species reactivity: human. packaging: antibody small pack of 25 . μ. L. technique(s): western blot: suitable. isotype: IgG. NCBI accession no.: NP_000023. UniProt accession no.: P22557. shipped in: ambient. target post-translational modification: unmodified. Gene Information: human ... ALAS2(212).