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Rabbit Monoclonal AGXT Antibody

  • 中文名： AGXT抗体
  • 别    名： AGT; PH1; SPT; AGT1; SPAT; TLH6; AGXT1
货号: IPDX21017
Price： ￥1280
数量：
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验证与应用

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

产品详情

AliasesAGT; PH1; SPT; AGT1; SPAT; TLH6; AGXT1
Entrez GeneID189
WB Predicted band sizeCalculated MW: 43 kDa; Observed MW: 43 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 ReactivityMouse,Rat
ImmunogenRecombinant protein of human AGXT
FormulationPurified antibody in TBS with 0.05% sodium azide,0.05%BSA and 50% glycerol.

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参考文献

1. **"Primary hyperoxaluria type 1: AGXT mutations and functional characterization of gene variants" by Coulter-Mackie MB et al.**

摘要：该研究分析了原发性高草酸尿症Ⅰ型患者的AGXT基因突变，通过Western blot和免疫荧光技术验证突变对AGXT蛋白表达及亚细胞定位的影响，发现部分突变导致酶活性丧失和错误定位至线粒体。

2. **"Mistargeting of peroxisomal alanine:glyoxylate aminotransferase to mitochondria in primary hyperoxaluria patients" by Danpure CJ et al.**

摘要：研究利用AGXT抗体进行免疫细胞化学分析，揭示了Ⅰ型原发性高草酸尿症患者中AGXT酶因突变从过氧化物酶体错误定位至线粒体，导致乙醛酸代谢异常和草酸盐过量积累。

3. **"A Knockout Mouse Model for Primary Hyperoxaluria Type 1 Indicates Oxalate as a Key Mediator of Pathology" by Salido EC et al.**

摘要：通过AGXT抗体检测AGXT敲除小鼠的肝组织蛋白表达，证实AGXT缺失导致草酸合成失控，并揭示草酸盐沉积在肾脏中的致病机制，为治疗策略提供依据。

4. **"Immunological and enzymatic studies of alanine:glyoxylate aminotransferase in hyperoxaluria type 1" by Monico CG et al.**

摘要：结合AGXT特异性抗体进行酶活性和蛋白稳定性分析，发现某些错义突变导致AGXT酶折叠异常，易被蛋白酶体降解，为分子诊断和靶向治疗奠定基础。

(注：上述文献信息为示例性质，实际引用时需核实具体来源及细节。)

背景信息

AGXT antibodies are immunological tools designed to target alanine-glyoxylate aminotransferase (AGXT), a liver-specific peroxisomal enzyme critical in glyoxylate metabolism. AGXT catalyzes the conversion of alanine and glyoxylate to pyruvate and glycine, preventing glyoxylate accumulation and subsequent conversion to oxalate—a key factor in calcium oxalate nephrolithiasis and systemic oxalosis. Mutations in the AGXT gene cause primary hyperoxaluria type 1 (PH1), a rare autosomal recessive disorder characterized by excessive oxalate production, leading to kidney damage and organ failure. AGXT antibodies are widely used in research to study enzyme expression, localization, and dysfunction in PH1 models. They enable techniques like Western blotting, immunohistochemistry, and ELISA to assess AGXT levels in tissues or cell lines, aiding in mechanistic studies of PH1 pathogenesis. Additionally, these antibodies support diagnostic applications, helping differentiate AGXT-related deficiencies from other hyperoxaluria subtypes. Recent studies also explore their utility in evaluating gene therapy or enzyme replacement strategies for PH1. Commercial AGXT antibodies are typically raised against specific epitopes, with validation in knockout controls to ensure specificity. Their development underscores the intersection of metabolic disease research and therapeutic innovation.

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