SMARCAL1
SMARCAL1（SWI/SNF相关染色质重塑复合体亚基AL1），也被称为HARP蛋白，是细胞内维护DNA稳定的“分子维修工”。当细胞分裂或遭遇外界损伤时，它能快速定位到DNA异常区域，协助修复断裂或错误折叠的遗传物质。SMARCAL1先天性缺陷会引发希-施二氏综合征，患儿通常在幼年出现脊柱畸形、免疫系统崩溃及肾衰竭，约半数患者因器官功能恶化活不过青春期。
目前针对SMARCAL1缺陷尚无特效药，临床以延缓肾损伤、骨髓移植为主。美国St. Jude儿童医院团队尝试用腺相关病毒载体递送正常SMARCAL1基因，在小鼠模型中成功延长生存期50%。小分子领域，诺华公司筛选出可增强DNA修复的化合物NVS-SM1，但体外实验显示对复杂突变类型效果有限。这类疗法普遍面临精准调控修复活性与避免基因毒性间的矛盾，未来或需结合干细胞移植技术突破瓶颈。
热销产品
SMARCAL1 Antibody (CSB-PA885774LA01HU)
验证数据
Western Blot
Positive WB detected in: Jurkat whole cell lysate, Mouse lung tissue, Mouse kidney tissue
All lanes: SMARCAL1 antibody at 3μg/ml
Secondary
Goat polyclonal to rabbit IgG at 1/50000 dilution
Predicted band size: 106 kDa
Observed band size: 106 kDa
Immunofluorescent analysis of Hela cells using CSB-PA885774LA01HU at dilution of 1:100 and Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L)
SMARCAL1 Antibodies
SMARCAL1 for Homo sapiens (Human)
| 产品货号 | 产品名称 | 种属反应性 | 应用类型 |
|---|---|---|---|
| CSB-PA021804GA01HU | SMARCAL1 Antibody | Human,Mouse,Rat | ELISA,WB |
| CSB-PA885774LA01HU | SMARCAL1 Antibody | Human, Mouse | ELISA, WB, IF |
| CSB-PA885774LB01HU | SMARCAL1 Antibody, HRP conjugated | Human | ELISA |
| CSB-PA885774LC01HU | SMARCAL1 Antibody, FITC conjugated | Human | |
| CSB-PA885774LD01HU | SMARCAL1 Antibody, Biotin conjugated | Human | ELISA |
SMARCAL1 Proteins
SMARCAL1 Proteins for Homo sapiens (Human)
| 产品货号 | 产品名称 | 来源 |
|---|---|---|
| CSB-YP885774HU CSB-EP885774HU CSB-BP885774HU CSB-MP885774HU CSB-EP885774HU-B |
Recombinant Human SWI/SNF-related matrix-associated actin-dependent regulator of chromatin subfamily A-like protein 1 (SMARCAL1), partial | Yeast E.coli Baculovirus Mammalian cell In Vivo Biotinylation in E.coli |
