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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

CSB-PA885774LA01HU

Western Blot
Positive WB detected in: K562 whole cell lysate(30μg), MCF-7 whole cell lysate(30μg), Mouse Kidney tissue lysate(30μg)
All lanes: SMARCAL1 antibody at 1:1000
Secondary
Goat polyclonal to rabbit IgG at 1/40000 dilution
Predicted band size: 106 kDa
Observed band size: 106 kDa
Exposure time: 120s

CSB-PA885774LA01HU

IHC image of CSB-PA885774LA01HU diluted at 1:100 and staining in paraffin-embedded human brain tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a Goat anti-rabbit polymer IgG labeled by HRP and visualized using 0.05% DAB. Secondary antibody only control: uses 1% BSA instead of primary antibody

SMARCAL1 Antibodies

SMARCAL1 for Homo sapiens (Human)

SMARCAL1 Proteins

SMARCAL1 Proteins for Homo sapiens (Human)



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