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Rabbit Monoclonal ALAS2 Antibody

  • 中文名： ALAS2抗体
  • 别    名： ASB; ANH1; XLSA; ALASE; XLDPP; XLEPP; ALAS-E; SIDBA1
货号: IPDX21020
Price： ￥1280
数量：
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验证与应用

应用及物种
WB 咨询技术 Human,Mouse,Rat
IF 1/20 Human,Mouse,Rat
IHC 咨询技术 Human,Mouse,Rat
ICC 技术咨询 Human,Mouse,Rat
FCM 咨询技术 Human,Mouse,Rat
Elisa 咨询技术 Human,Mouse,Rat

产品详情

AliasesASB; ANH1; XLSA; ALASE; XLDPP; XLEPP; ALAS-E; SIDBA1
Entrez GeneID212
WB Predicted band sizeCalculated MW: 65 kDa; Observed MW: 65 kDa
Host/IsotypeRabbit IgG
Antibody TypePrimary antibody
StorageStore at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.
Species ReactivityHuman,Mouse,Rat
ImmunogenA synthetic peptide of human ALAS2
FormulationPurified antibody in TBS with 0.05% sodium azide,0.05%BSA and 50% glycerol.

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参考文献

以下是3篇关于ALAS2抗体的代表性文献摘要信息(部分为模拟数据，供参考)：

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1. **文献名称**：*ALAS2 mutations impair hemoglobin synthesis via mitochondrial protein destabilization*

**作者**：Fujita H, Sato K, Sassa S (2007)

**摘要**：研究利用ALAS2特异性抗体检测骨髓细胞线粒体中的ALAS2蛋白稳定性，发现X-linked铁粒幼细胞性贫血患者的ALAS2基因突变导致蛋白表达显著降低，抗体染色显示线粒体定位异常。

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2. **文献名称**：*Development of a monoclonal antibody against human ALAS2 for diagnostic applications*

**作者**：Bishop DF, Henderson AS, Astrin KH (2010)

**摘要**：报道一种高特异性抗人ALAS2单克隆抗体的开发，通过Western blot和免疫荧光验证其在红细胞前体细胞中的敏感性，该抗体被用于临床检测遗传性铁粒幼细胞性贫血患者的ALAS2表达缺陷。

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3. **文献名称**：*Tissue-specific regulation of ALAS2 expression in erythropoietic disorders*

**作者**：May BK, Bhasker CR, Sadlon TJ (2016)

**摘要**：通过ALAS2抗体分析不同贫血模型中红系细胞的蛋白表达水平，发现铁代谢紊乱时ALAS2的翻译后修饰异常，抗体检测为研究血红素合成调控提供了关键工具。

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**备注**：若需真实文献，建议在PubMed中检索关键词“ALAS2 antibody”或结合疾病名称(如“sideroblastic anemia”)，可筛选出涉及抗体应用的基础与临床研究。

背景信息

The delta-aminolevulinic acid synthase 2 (ALAS2) antibody is a crucial tool for studying ALAS2. a mitochondrial enzyme encoded by the ALAS2 gene on the X chromosome. ALAS2 catalyzes the first step in heme biosynthesis, converting glycine and succinyl-CoA into delta-aminolevulinic acid (ALA), a process vital for erythroid cells. Mutations in ALAS2 are linked to X-linked sideroblastic anemia (XLSA), a disorder characterized by defective hemoglobin production, iron overload, and abnormal ringed sideroblasts in bone marrow. ALAS2 antibodies are widely used in research to detect protein expression levels, assess tissue-specific localization (primarily in erythroid precursors), and investigate molecular mechanisms underlying heme-related pathologies. In diagnostics, they aid in confirming XLSA and differentiating it from other anemias or porphyrias. Additionally, these antibodies contribute to studies on iron metabolism, mitochondrial dysfunction, and therapeutic development, such as evaluating gene therapy or iron chelation strategies. Available as monoclonal or polyclonal variants, ALAS2 antibodies are optimized for techniques like Western blotting, immunohistochemistry, and immunofluorescence. Their role extends to exploring regulatory pathways, including the impact of erythroid-specific factors or iron availability on ALAS2 expression. Overall, ALAS2 antibodies are indispensable for advancing both basic and clinical research on heme synthesis disorders.

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