WB | 1/1000-1/2000 | Human,Mouse,Rat |
IF | 咨询技术 | Human,Mouse,Rat |
IHC | 咨询技术 | Human,Mouse,Rat |
ICC | 1/50-1/200 | Human,Mouse,Rat |
FCM | 1/20-1/100 | Human,Mouse,Rat |
Elisa | 咨询技术 | Human,Mouse,Rat |
Aliases | EL2; Elliptocytosis2; erythrocyte; Erythroid alpha spectrin; HPP; HS3; SPH3; SPTA; SPTA1;;Spectrin alpha chain |
WB Predicted band size | 280 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,Rat |
Immunogen | A synthesized peptide derived from human Spectrin alpha chain |
Formulation | Purified antibody in PBS with 0.05% sodium azide,0.05% BSA and 50% glycerol. |
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以下是关于α1-Spectrin红细胞抗体的3篇参考文献概览:
1. **《Anti-α1-spectrin antibodies in autoimmune hemolytic anemia》**
- 作者:M. Shibata et al.
- 摘要:研究自身免疫性溶血性贫血(AIHA)患者中α1-血影蛋白抗体的发生率及其临床意义,发现部分患者存在此类抗体,可能与红细胞膜破坏相关。
2. **《Spectrin mutations and red cell membrane disorders》**
- 作者:J. Delaunay
- 摘要:综述遗传性球形红细胞增多症等红细胞膜疾病中α1-血影蛋白基因突变的作用,并提及部分患者体内可能产生针对异常血影蛋白的自身抗体。
3. **《Autoantibodies against erythrocyte membrane components in paroxysmal nocturnal hemoglobinuria》**
- 作者:A.N. Gallagher et al.
- 摘要:探讨阵发性睡眠性血红蛋白尿(PNH)患者中红细胞膜蛋白(包括α1-血影蛋白)自身抗体的存在,分析其与补体介导溶血的关系。
注:实际文献需通过PubMed或专业数据库检索获取完整信息,以上为模拟示例。
Alpha-1 spectrin, a key component of the erythrocyte membrane skeleton, plays a critical role in maintaining red blood cell (RBC) shape, flexibility, and mechanical stability. It forms heterodimers with beta-spectrin, which assemble into a hexagonal lattice linked to the cell membrane via proteins like ankyrin and band 4.1. Antibodies targeting alpha-1 spectrin are rare but clinically significant, primarily associated with autoimmune hemolytic anemia (AIHA) and hereditary disorders. In AIHA, these autoantibodies may develop due to immune dysregulation, leading to RBC destruction through opsonization or complement activation. They are often detected via specialized serological tests, such as the indirect antiglobulin test (IAT) or enzyme-linked immunosorbent assays (ELISA).
Alpha-1 spectrin antibodies are also implicated in hereditary spherocytosis (HS), where spectrin deficiency causes membrane instability. In some HS cases, secondary autoantibodies against exposed spectrin epitopes may exacerbate hemolysis. Additionally, these antibodies have been reported in post-transfusion settings or autoimmune conditions like systemic lupus erythematosus. Their presence complicates diagnosis, as they may mimic alloantibodies or coexist with other RBC autoantibodies. Research continues to explore their role in rare AIHA subtypes and interactions with spectrin mutations. Clinical management focuses on immunosuppression (e.g., corticosteroids) or addressing underlying conditions, guided by antibody specificity and hemolytic severity.
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