ABS2121

Anti-ALAS-E

from rabbit

• 别名: 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
• UNSPSC代码: 12352203
• eCl@ss: 32160702
• NACRES: NA.41

属性

• 生物来源: rabbit
• 质量水平: 100
• 抗体形式: unpurified
• 抗体产品类型: primary antibodies
• 克隆: polyclonal
• 种属反应性: human
• 包装: antibody small pack of 25 μL
• 技术: western blot: suitable
• 同位素/亚型: IgG
• NCBI登记号: NP_000023
• UniProt登记号: P22557
• 运输: ambient
• 靶向翻译后修饰: unmodified
• 基因信息: human ... ALAS2(212)

说明

一般描述

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.

特异性

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

免疫原

Epitope: unknown

Full length recombinant human 5-aminolevulinate synthase.

应用

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).

质量

Evaluated by Western Blotting with recombinant hALAS2

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

目标描述

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

外形

Unpurified

Format: Unpurified

Rabbit polyclonal antiserum with 0.05% sodium azide.

储存及稳定性

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.

其他说明

Concentration: Please refer to lot specific datasheet.

免责声明

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.