人 alpha 1 Spectrin 抗体对
01AB03174
Spectrin alpha, erythrocytic 1 (SPTA1) encodes the alpha-spectrin chain, the major structural component of the erythrocyte membrane skeleton. It forms antiparallel heterodimers with beta-spectrin, which self-associate into a tetrameric lattice anchoring the plasma membrane to the actin cytoskeleton. This architecture maintains red blood cell biconcave shape, deformability, and mechanical stability. Pathogenic variants in SPTA1 disrupt spectrin assembly or abundance, leading to membrane instability and hereditary hemolytic anemias, including hereditary elliptocytosis, pyropoikilocytosis, and spherocytosis type 3, characterized by hemolytic destruction of fragile erythrocytes.
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技术参数
- 靶点基因名
- SPTA1
- 基因别名
- SPTA
- 蛋白别名
- spectrin alpha, erythrocytic 1; alpha 1 Spectrin
- UniProt
- P02549
- 物种
- Human
产品详情
- 靶点名称
- alpha 1 Spectrin
- 蛋白名称
- spectrin alpha, erythrocytic 1
- 蛋白别名
- Spectrin alpha chain; erythrocytic 1; Erythroid alpha-spectrin
- 靶点基因名
- SPTA1
- 基因别名
- SPTA
- UniProt
- P02549
- 来源物种
- Homo sapiens (Human)
- 物种反应性
- Human
背景介绍
Spectrin alpha, erythrocytic 1 (SPTA1) encodes the alpha-spectrin chain, the major structural component of the erythrocyte membrane skeleton. It forms antiparallel heterodimers with beta-spectrin, which self-associate into a tetrameric lattice anchoring the plasma membrane to the actin cytoskeleton. This architecture maintains red blood cell biconcave shape, deformability, and mechanical stability. Pathogenic variants in SPTA1 disrupt spectrin assembly or abundance, leading to membrane instability and hereditary hemolytic anemias, including hereditary elliptocytosis, pyropoikilocytosis, and spherocytosis type 3, characterized by hemolytic destruction of fragile erythrocytes.