| WB | 1/500-1/2000 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 1/25-1/100 | Human,Mouse,Rat |
| ICC | 技术咨询 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 1/2000-1/5000 | Human,Mouse,Rat |
| Aliases | CTLN2; CITRIN; ARALAR2 |
| WB Predicted band size | 74 kDa |
| Host/Isotype | Rabbit IgG |
| Antibody Type | Primary antibody |
| Storage | Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. |
| Species Reactivity | Human, Mouse |
| Immunogen | Fusion protein of human SLC25A13 |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
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以下是关于SLC25A13抗体的3篇参考文献，按简洁格式整理：
1. **文献名称**：*Citrin Deficiency: A Novel Molecular Mechanism in Neonatal Intrahepatic Cholestasis*
**作者**：Saheki T, et al.
**摘要**：研究利用SLC25A13特异性抗体进行Western blot分析，验证Citrin蛋白在肝组织中的表达缺失，揭示其与新生儿肝内胆汁淤积症(NICCD)的关联。
2. **文献名称**：*Immunohistochemical Characterization of SLC25A13 in Human Tissues*
**作者**：Kobayashi K, et al.
**摘要**：通过免疫组化技术结合SLC25A13抗体，系统分析了该蛋白在心脏、肝脏及胰腺等组织中的分布，证实其线粒体定位及组织特异性表达差异。
3. **文献名称**：*Functional Analysis of SLC25A13 Mutants Using Antibody-based Protein Detection*
**作者**：Lin W, et al.
**摘要**：利用抗SLC25A13抗体检测突变体蛋白的稳定性及转运活性，发现部分错义突变导致Citrin蛋白降解加速，从而引发代谢功能障碍。
(注：以上文献信息为示例性质，具体内容需根据实际文献调整。建议通过PubMed或Google Scholar以“SLC25A13 antibody”为关键词检索最新研究。)
The SLC25A13 antibody targets the solute carrier family 25 member 13 (SLC25A13) protein, a mitochondrial transporter encoded by the *SLC25A13* gene. This protein, also known as citrin, functions as a calcium-binding mitochondrial aspartate/glutamate carrier (AGC) involved in the malate-aspartate shuttle, facilitating the exchange of aspartate and glutamate across the mitochondrial inner membrane. This process is critical for maintaining cellular redox balance, urea cycle function, and gluconeogenesis.
Mutations in *SLC25A13* are linked to citrin deficiency, an autosomal recessive disorder encompassing neonatal intrahepatic cholestasis (NICCD) and adult-onset type II citrullinemia (CTLN2). These conditions manifest as metabolic disturbances, including hyperammonemia, citrullinemia, and liver dysfunction. SLC25A13 antibodies are essential tools in research to study protein expression, localization, and functional alterations in disease models.
In experimental settings, these antibodies are widely used in techniques like Western blotting, immunohistochemistry, and immunofluorescence to assess citrin levels in tissues (e.g., liver, kidney, heart) and investigate its role in metabolic pathways. Their application aids in diagnosing genetic disorders, elucidating molecular mechanisms of citrin deficiency, and evaluating therapeutic interventions targeting mitochondrial transport dysfunction.
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