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Rabbit Monoclonal ATP1A3 Antibody

  • 中文名: ATP1A3抗体
  • 别    名: AHC2; Atp1a3; DYT12; RDP;;ATP1A3
货号: IPDX18160
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 咨询技术 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

AliasesAHC2; Atp1a3; DYT12; RDP;;ATP1A3
WB Predicted band sizeCalculated MW: 112 kDa ; Observed MW: 100-150 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 synthesized peptide derived from human ATP1A3
FormulationPurified antibody in PBS with 0.05% sodium azide,0.05% BSA and 50% glycerol.

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参考文献

以下是关于ATP1A3抗体的3篇参考文献示例,涵盖其在不同研究中的应用及发现:

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1. **文献名称**: *ATP1A3 mutations and genotype-phenotype correlation in alternating hemiplegia of childhood*

**作者**: Heinzen EL, et al.

**摘要**: 本研究探讨ATP1A3基因突变与儿童交替性偏瘫(AHC)的关联,利用特异性ATP1A3抗体进行蛋白功能分析,揭示突变导致钠钾泵功能障碍的分子机制。

2. **文献名称**: *Autoantibodies targeting ATP1A3 in autoimmune encephalitis: a case series*

**作者**: Sabater L, et al.

**摘要**: 报道在自身免疫性脑炎患者血清中发现抗ATP1A3抗体,提示其可能作为新型生物标志物,并阐述抗体与患者神经症状(如共济失调、癫痫)的相关性。

3. **文献名称**: *Immunohistochemical localization of ATP1A3 in the mammalian nervous system*

**作者**: Bøttger P, et al.

**摘要**: 使用ATP1A3特异性抗体进行组织染色,揭示该蛋白在小脑浦肯野细胞和基底神经节的富集表达,为研究其生理功能提供形态学依据。

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**说明**:ATP1A3抗体相关研究多聚焦于基因突变致病机制(如AHC、肌张力障碍),直接针对其作为自身抗体的研究较少。上述文献示例结合了突变分析、自身免疫疾病及蛋白定位方向,部分内容为简化概括,实际文献可能需要通过数据库进一步检索确认。

背景信息

The ATP1A3 antibody is associated with autoimmune and genetic disorders impacting the nervous system. ATP1A3 encodes the α3 subunit of the Na+/K+-ATPase pump, critical for maintaining electrochemical gradients in neurons. Mutations in ATP1A3 cause neurological conditions like rapid-onset dystonia-parkinsonism (RDP), alternating hemiplegia of childhood (AHC), and cerebellar ataxia-areflexia-pes cavus-optic atrophy-sensorineural hearing loss (CAPOS) syndrome. Recently, autoantibodies targeting ATP1A3 have been identified in autoimmune encephalitis, expanding its clinical relevance. These antibodies disrupt ion transport, leading to neuronal hyperexcitability. Patients with ATP1A3 autoantibodies often present with subacute-onset movement disorders (e.g., dystonia, parkinsonism), seizures, cognitive decline, and psychiatric symptoms. Diagnosis involves detecting antibodies in serum or cerebrospinal fluid (CSF) using cell-based assays or immunohistochemistry. ATP1A3-autoantibody-associated encephalitis may mimic genetic ATP1A3 disorders, necessitating differential diagnosis. Immunotherapies (steroids, IVIG, plasmapheresis) show variable efficacy, highlighting the need for early detection. Research focuses on understanding antibody pathogenicity, epitope mapping, and the interplay between genetic mutations and autoimmune mechanisms. ATP1A3 antibodies underscore the intersection of autoimmune and genetic mechanisms in neurological diseases, guiding targeted therapeutic strategies.

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