| 产品编号 | bs-102316P |
| 英文名称 | Recombinant Human TSC1 Protein, N-His |
| 中文名称 | |
| 别 名 | TSC; Hamartin; Tuberous sclerosis 1 protein; KIAA0243; TSC1 |
| 克 隆 号 | |
| 理论分子量 | 19.83 kDa |
| 性 状 | Lyophilized |
| 纯 度 | >90% as determined by SDS-PAGE. |
| 缓 冲 液 | Lyophilized from a solution in PBS pH 7.4, 0.02% NLS, 1mM EDTA, 4% Trehalose, 1% Mannitol. |
| 保存条件 | Use a manual defrost freezer and avoid repeated freeze thaw cycles. Store at 2 to 8°C for frequent use. Store at -20 to -80°C for twelve months from the date of receipt. |
| 注意事项 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| 产品介绍 |
Tuberous sclerosis complex (TSC) is an autosomal dominant genetic disorder characterized by mental retardation and the widespread development of distinctive tumors termed hamartomas. Two different genetic loci have been linked to TSC; one of these loci, the tuberous sclerosis-2 gene (TSC2), encodes a protein called tuberin and the other loci, tuberous sclerosis-1 gene (TSC1), encodes a protein called hamartin. Tuberin and hamartin interact with each other forming a cystoplasmic complex. Hamartin interacts with the ezrin-radixin-moesin (ERM) family of actin-binding proteins and inhibition of hamartin activity results in loss of cell adhesion. Hamartin is present in most adult tissues with strong expression in brain, heart, and kidney. SWISS: Q92574 Gene ID: 7248 结节性硬化症为常染色体显性遗传，也常见散发病例。是肿瘤抑制基因，基因产物分别为Hamartin和tuberin，两者均调节细胞生长。 结节性硬化症(tuberous sclerosis)又称结节性脑硬化，Bourneville病。本病可归类于神经皮肤综合征(亦称斑痣性错构瘤病)，是源于外胚层的器官发育异常所致，病变累及神经系统、皮肤和眼，也可累及中胚层，内胚层器官如心、肺、骨，肾和胃肠等。皮脂腺瘤是皮肤神经末梢、增生的结缔组织和血管组成，视网膜可见胶质瘤、神经节细胞瘤，心、肾、肺、 |
| 1、抗体溶解方法 | |
| 2、抗体修复方式 | |
| 3、常用试剂的配制 | |
| 4、免疫组化操作步骤 | |
| 5、免疫组化问题解答 | |
| 6、Western Blotting 操作步骤 | |
| 7、Western Blotting 问题解答 | |
| 8、关于肽链的设计 | |
| 9、多肽的溶解与保存 | |
| 10、酶标抗体效价测定程序 | |
