BBS1
BBS1，全稱為Bardet-Biedl綜合征1蛋白，常見別名即BBS1，是維持細胞纖毛正常功能的重要分子。它參與纖毛的結構組成與功能調控，纖毛作為細胞表面的“微小觸角”，與身體多項生理活動緊密相關。當BBS1蛋白出現(xiàn)異常時，纖毛的形態(tài)或功能會受到影響，進而引發(fā)一系列健康問題。
最典型的相關疾病是Bardet-Biedl綜合征，患者常表現(xiàn)出視網(wǎng)膜色素變性導致的視力減退、肥胖、多指（趾）畸形、腎臟功能異常等癥狀。目前針對這類疾病的治療多以對癥支持為主，比如通過視力矯正改善視覺障礙、飲食管理控制體重、藥物或手術處理腎臟問題；而靶向BBS1蛋白的針對性治療仍在探索階段，研究人員正嘗試用基因編輯技術或小分子調節(jié)劑修復其功能，為患者帶來新的治療方向。
熱銷產(chǎn)品
Recombinant Human BBSome complex member BBS1 (BBS1), Biotinylated (CSB-EP836736HU-B)
驗證數(shù)據(jù)
(Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.
BBS1 Antibody (CSB-PA836736LA01HU)
驗證數(shù)據(jù)
Western Blot
Positive WB detected in: Mouse lung tissue
All lanes: BBS1 antibody at 1μg/ml
Secondary
Goat polyclonal to rabbit IgG at 1/50000 dilution
Predicted band size: 66, 70, 50 kDa
Observed band size: 66 kDa
Immunohistochemistry of paraffin-embedded human kidney tissue using CSB-PA836736LA01HU at dilution of 1:100
Immunohistochemistry of paraffin-embedded human liver tissue using CSB-PA836736LA01HU at dilution of 1:100
Immunofluorescent analysis of HepG2 cells using CSB-PA836736LA01HU at dilution of 1:100 and Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L)
BBS1 Antibodies
BBS1 for Homo sapiens (Human)
| 產(chǎn)品貨號 | 產(chǎn)品名稱 | 種屬反應性 | 應用類型 |
|---|---|---|---|
| CSB-PA836736LA01HU | BBS1 Antibody | Human, Mouse | ELISA, WB, IHC, IF |
BBS1 Proteins
BBS1 Proteins for Homo sapiens (Human)
| 產(chǎn)品貨號 | 產(chǎn)品名稱 | 來源 |
|---|---|---|
| CSB-YP836736HU CSB-BP836736HU CSB-MP836736HU |
Recombinant Human BBSome complex member BBS1 (BBS1) | Yeast Baculovirus Mammalian cell |
| CSB-EP836736HU | Recombinant Human BBSome complex member BBS1 (BBS1) | E.coli |
| CSB-EP836736HU-B | Recombinant Human BBSome complex member BBS1 (BBS1), Biotinylated | E.coli |
