Key Specifications Table
| Species Reactivity | Key Applications | Host | Format | Antibody Type |
|---|---|---|---|---|
| H | WB | Rb | Unpurified | Polyclonal Antibody |
| Description | |
|---|---|
| Catalogue Number | ABS2121-25UL |
| Description | Anti-ALAS-E |
| Alternate Names |
|
| Background Information | 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. |
| Product Information | |
|---|---|
| Format | Unpurified |
| Presentation | Rabbit polyclonal antiserum with 0.05% sodium azide. |
| Applications | |
|---|---|
| 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. |
| Key Applications |
|
| Application Notes | 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 ). |
| Biological Information | |
|---|---|
| Immunogen | Full length recombinant human 5-aminolevulinate synthase. |
| Concentration | Please refer to lot specific datasheet. |
| Host | Rabbit |
| Specificity | This rabbit polyclonal antibody detects erythroid-specifc 5-aminolevulinate synthase, mitochondrial in human cells. |
| Isotype | IgG |
| Species Reactivity |
|
| Species Reactivity Note | Human. |
| Antibody Type | Polyclonal Antibody |
| Gene Symbol |
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| Purification Method | Unpurified |
| UniProt Number |
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| Molecular Weight | ~58 and 72 kDa observed; Detects both the precusor and mature forms of human ALAS2. Uncharacterized bands may be observed in some lysate(s). |
| Product Usage Statements | |
|---|---|
| Quality Assurance | Evaluated by Western Blotting with recombinant hALAS2 Western Blotting Analysis: A 1:2,000 dilution of this antibody detected recombinant ALAS-E. |
| Usage Statement |
|
| Storage and Shipping Information | |
|---|---|
| Storage Conditions | Stable for 1 year at -20°C from date of receipt. |
| Packaging Information | |
|---|---|
| Material Size | 25 µL |