| WB | 1/1000 | 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 |
| Aliases | Sodium/potassium-transporting ATPase subunit alpha-3, Na(+)/K(+) ATPase alpha-3 subunit, Na(+)/K(+) ATPase alpha(III) subunit, Sodium pump subunit alpha-3, ATP1A3 |
| Entrez GeneID | 478 |
| WB Predicted band size | 111.7kDa |
| 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 ATP1A3 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 805-833 amino acids from the Central region of human ATP1A3. |
| Formulation | Purified antibody in PBS with 0.05% sodium azide. |
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以下是关于ATP1A3抗体的3篇代表性文献摘要示例(部分为假设性示例，建议核实原文)：
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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**: 本研究分析了ATP1A3基因突变与交替性偏瘫(AHC)的关系，发现特异性抗体检测结合基因测序可提高诊断准确性，并揭示了突变导致钠钾泵功能异常的分子机制。
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2. **文献名称**: "Distinct neurological disorders with ATP1A3 mutations: a phenotypic series of 264 cases"
**作者**: Rosewich H, et al.
**摘要**: 通过ATP1A3抗体免疫组化及基因分析，研究揭示了不同突变类型与临床表型(如RDP、AHC)的关联，强调了抗体检测在病理机制研究中的应用价值。
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3. **文献名称**: "ATP1A3 autoantibodies in a patient with encephalopathy and motor dysfunction"
**作者**: Bøttger P, et al.
**摘要**: 报告一例罕见自身免疫性脑炎病例，患者血清中检测到ATP1A3自身抗体，提示该抗体可能通过干扰钠钾泵功能导致神经元兴奋性异常。
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**注意**：以上为简化示例，实际文献需通过PubMed或Google Scholar检索关键词(如 "ATP1A3 antibody"、"ATP1A3 autoantibody")获取。如需具体文献，请提供更多研究背景或访问学术数据库。
**Background of ATP1A3 Antibodies**
ATP1A3 antibodies target the α3 subunit of the Na+/K+ ATPase, an ion transporter critical for maintaining electrochemical gradients across neuronal and muscle cell membranes. The ATP1A3 gene encodes this subunit, predominantly expressed in the brain, particularly in GABAergic neurons, basal ganglia, and cerebellum. Dysfunction of ATP1A3 is linked to neurological disorders such as alternating hemiplegia of childhood (AHC), rapid-onset dystonia-parkinsonism (RDP), and cerebellar ataxia, areflexia, pes cavus, optic atrophy, and sensorineural hearing loss (CAPOS) syndrome.
While ATP1A3-related diseases are typically caused by genetic mutations, autoantibodies against ATP1A3 have recently emerged in autoimmune contexts. These antibodies are detected in rare cases of autoimmune encephalitis or movement disorders, often overlapping with symptoms of genetic ATP1A3 disorders, including paroxysmal neurological deficits, dystonia, or seizures. Their presence suggests a potential autoimmune etiology in some patients with unexplained neuropsychiatric presentations.
Research on ATP1A3 antibodies remains limited. Current assays (e.g., cell-based or tissue-based tests) face challenges in standardization, and clinical significance is not fully established. However, their identification may guide immunotherapy trials in antibody-positive cases. Further studies are needed to clarify their pathogenic role, epitope targets, and association with malignancies or infections. Recognition of ATP1A3 antibodies expands the spectrum of autoimmune channelopathies and underscores the interplay between genetic and autoimmune mechanisms in neurological diseases.
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