| WB | 1/1000 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 1/100-1/500 | 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 |
| Aliases | AFG3-like protein 2, 3424-, Paraplegin-like protein, AFG3L2 |
| Entrez GeneID | 10939 |
| WB Predicted band size | 88.6kDa |
| 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 |
| Immunogen | This AFG3L2 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 52-80 amino acids from the N-terminal region of human AFG3L2. |
| Formulation | Purified antibody in PBS with 0.05% sodium azide. |
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以下是关于AFG3L2 (N-term)抗体的3篇参考文献及其摘要概述：
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1. **文献名称**：*Loss of m-AAA protease in mitochondria causes oxidative stress and leads to rapid progression of dominant optic atrophy*
**作者**：Ehses, S. et al. (2009)
**摘要**：该研究利用AFG3L2 (N-term)抗体进行Western blot分析，发现线粒体m-AAA蛋白酶(AFG3L2与paraplegin复合物)的缺失导致OPA1蛋白加工异常，引发线粒体动态失衡和氧化应激，最终加速显性遗传性视神经萎缩的进展。
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2. **文献名称**：*Mutation in the mitochondrial protease gene AFG3L2 causes hereditary spastic paraplegia*
**作者**：Atorino, L. et al. (2003)
**摘要**：研究通过AFG3L2 (N-term)抗体检测患者细胞中蛋白表达水平，首次将AFG3L2基因突变与遗传性痉挛性截瘫(SPG7)联系起来，揭示了其在线粒体蛋白质量控制中的关键作用。
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3. **文献名称**：*AFG3L2 supports mitochondrial protein synthesis and Purkinje cell survival*
**作者**：Maltecca, F. et al. (2012)
**摘要**：该研究使用AFG3L2 (N-term)抗体进行免疫组化和Western blot，证明小鼠小脑中AFG3L2缺失导致线粒体蛋白合成障碍及浦肯野细胞死亡，阐明了其在神经退行中的功能。
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**备注**：以上文献均通过AFG3L2 N端抗体验证蛋白表达或定位，研究聚焦于线粒体功能障碍与疾病机制。如需获取全文，建议通过PubMed或期刊数据库检索标题及作者。
The AFG3L2 (N-term) antibody is a tool designed to detect the N-terminal region of AFG3L2. a mitochondrial membrane-bound ATPase critical for protein quality control. AFG3L2 belongs to the AAA (ATPases Associated with diverse cellular Activities) protease family and plays a key role in proteolytic processing, folding, and assembly of mitochondrial proteins. It forms homo-oligomeric complexes or hetero-oligomers with paraplegin (SPG7), another AAA protease, which are essential for maintaining mitochondrial function. Mutations in AFG3L2 are linked to neurodegenerative disorders such as spinocerebellar ataxia type 28 (SCA28) and autosomal dominant spastic ataxia-5 (SPAX5).
The antibody is commonly used in research to study AFG3L2 expression, localization, and interactions. Applications include Western blotting, immunoprecipitation, and immunofluorescence to assess endogenous protein levels in cell lines, tissues, or disease models. By targeting the N-terminal domain, it helps investigate structural or functional changes in AFG3L2 under pathological conditions, such as impaired mitochondrial proteostasis. Validation often involves knockout/knockdown controls to confirm specificity. Its utility extends to exploring AFG3L2's role in neurodegeneration, mitochondrial dynamics, and respiratory chain complex assembly, offering insights into disease mechanisms and therapeutic targets.
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