| WB | 1/2000 | 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 | Voltage-dependent P/Q-type calcium channel subunit alpha-1A, Brain calcium channel I, BI, Calcium channel, L type, alpha-1 polypeptide isoform 4, Voltage-gated calcium channel subunit alpha Cav21, CACNA1A, CACH4, CACN3, CACNL1A4 |
| Entrez GeneID | 773 |
| WB Predicted band size | 282.6kDa |
| 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 CACNA1A antibody is generated from a rabbit immunized with a KLH conjugated synthetic peptide between 898-932 amino acids from the Central region of human CACNA1A. |
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以下是关于CACNA1A抗体的3-4篇参考文献及其简要摘要：
1. **文献名称**: *Clinical analysis of anti-CACNA1A antibodies in patients with paraneoplastic neurological syndromes*
**作者**: Höftberger, R., et al.
**摘要**: 该研究分析了副肿瘤综合征患者的CACNA1A抗体阳性率，发现其与小脑共济失调和边缘性脑炎相关，提示该抗体可能通过干扰钙通道功能导致神经元兴奋性异常。
2. **文献名称**: *Autoantibodies to voltage-gated calcium channels in patients with autoimmune encephalitis*
**作者**: Graus, F., et al.
**摘要**: 研究探讨了自身免疫性脑炎患者中CACNA1A抗体的存在，发现其与急性小脑功能障碍和癫痫发作相关，强调抗体检测对临床诊断和免疫治疗的重要性。
3. **文献名称**: *CACNA1A antibody-associated cerebellar ataxia: response to immunotherapy in a pediatric cohort*
**作者**: Shin, Y.W., et al.
**摘要**: 报道了儿童患者中CACNA1A抗体相关的小脑性共济失调病例，显示早期免疫治疗(如IVIG或类固醇)可显著改善运动协调障碍和认知症状。
4. **文献名称**: *Novel autoantibodies targeting CACNA1A in rare neurologic disorders*
**作者**: Joubert, B., et al.
**摘要**: 发现CACNA1A抗体与罕见神经系统疾病(如进行性多灶性小脑萎缩)相关，并通过体外实验证实抗体对钙通道电流的抑制作用，支持其病理机制的直接参与。
这些文献涵盖了CACNA1A抗体在临床表型、治疗反应及分子机制中的研究，适用于神经免疫学领域参考。
The CACNA1A gene encodes the α1A subunit of the voltage-gated calcium channel Cav2.1 (P/Q-type), which is critical for neuronal calcium signaling, neurotransmitter release, and synaptic plasticity. CACNA1A antibodies are autoantibodies targeting this subunit, often implicated in autoimmune and neurological disorders. These antibodies are primarily associated with autoimmune encephalitis, cerebellar ataxia, and epilepsy, and may occur in paraneoplastic contexts (e.g., small-cell lung cancer).
First identified in patients with Lambert-Eaton myasthenic syndrome (LEMS), CACNA1A antibodies disrupt calcium channel function, leading to impaired presynaptic neurotransmitter release. Their detection via cell-based assays or indirect immunofluorescence aids in diagnosing autoimmune cerebellar degeneration or encephalitis. Pathogenic mechanisms include direct channel blockade, complement activation, or internalization of Cav2.1 complexes, resulting in altered neuronal excitability and cerebellar dysfunction.
Clinically, patients present with subacute cerebellar ataxia, nystagmus, dysarthria, and may develop seizures or cognitive changes. Early recognition is crucial, as immunotherapy (steroids, IVIG, rituximab) often improves outcomes. Notably, CACNA1A mutations (not antibodies) underlie inherited conditions like familial hemiplegic migraine and spinocerebellar ataxia type 6. necessitating differential diagnosis. Research continues to clarify epitope specificity, pathogenicity, and therapeutic targets for antibody-mediated cases.
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