SMARCAL1
SMARCAL1（SWI/SNF相關染色質重塑復合體亞基AL1），也被稱為HARP蛋白，是細胞內維護DNA穩定的“分子維修工”。當細胞分裂或遭遇外界損傷時，它能快速定位到DNA異常區域，協助修復斷裂或錯誤折疊的遺傳物質。SMARCAL1先天性缺陷會引發希-施二氏綜合征，患兒通常在幼年出現脊柱畸形、免疫系統崩潰及腎衰竭，約半數患者因器官功能惡化活不過青春期。
目前針對SMARCAL1缺陷尚無特效藥，臨床以延緩腎損傷、骨髓移植為主。美國St. Jude兒童醫院團隊嘗試用腺相關病毒載體遞送正常SMARCAL1基因，在小鼠模型中成功延長生存期50%。小分子領域，諾華公司篩選出可增強DNA修復的化合物NVS-SM1，但體外實驗顯示對復雜突變類型效果有限。這類療法普遍面臨精準調控修復活性與避免基因毒性間的矛盾，未來或需結合干細胞移植技術突破瓶頸。
熱銷產品
SMARCAL1 Antibody (CSB-PA885774LA01HU)
驗證數據
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)
| 產品貨號 | 產品名稱 | 種屬反應性 | 應用類型 |
|---|---|---|---|
| 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)
| 產品貨號 | 產品名稱 | 來源 |
|---|---|---|
| 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 |
