SMARCAL1
SMARCAL1（SWI/SNF相關(guān)染色質(zhì)重塑復(fù)合體亞基AL1），也被稱為HARP蛋白，是細(xì)胞內(nèi)維護(hù)DNA穩(wěn)定的“分子維修工”。當(dāng)細(xì)胞分裂或遭遇外界損傷時(shí)，它能快速定位到DNA異常區(qū)域，協(xié)助修復(fù)斷裂或錯(cuò)誤折疊的遺傳物質(zhì)。SMARCAL1先天性缺陷會(huì)引發(fā)希-施二氏綜合征，患兒通常在幼年出現(xiàn)脊柱畸形、免疫系統(tǒng)崩潰及腎衰竭，約半數(shù)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(yī)院團(tuán)隊(duì)嘗試用腺相關(guān)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(lǐng)域，諾華公司篩選出可增強(qiáng)DNA修復(fù)的化合物NVS-SM1，但體外實(shí)驗(yàn)顯示對復(fù)雜突變類型效果有限。這類療法普遍面臨精準(zhǔn)調(diào)控修復(fù)活性與避免基因毒性間的矛盾，未來或需結(jié)合干細(xì)胞移植技術(shù)突破瓶頸。
熱銷產(chǎn)品
SMARCAL1 Antibody (CSB-PA885774LA01HU)
驗(yàn)證數(shù)據(jù)
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
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)
| 產(chǎn)品貨號(hào) | 產(chǎn)品名稱 | 種屬反應(yīng)性 | 應(yīng)用類型 |
|---|---|---|---|
| CSB-PA021804GA01HU | SMARCAL1 Antibody | Human,Mouse,Rat | ELISA,WB |
| CSB-PA885774LA01HU | SMARCAL1 Antibody | Human, Mouse | ELISA, WB, IHC |
| 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(chǎn)品貨號(hào) | 產(chǎn)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 |
