产品名称
BBS4 Rabbit Polyclonal Antibody
蛋白名称
Bardet-Biedl syndrome 4 protein
存储缓冲液
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.
Human Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=585
Human Swissprot No.
Q96RK4
Human Swissprot Link
https://www.uniprot.org/uniprot/Q96RK4
Mouse Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=102774
Mouse Swissprot No.
Q8C1Z7
Mouse Swissprot Link
https://www.uniprot.org/uniprotkb/Q8C1Z7/entry
特异性
This antibody detects endogenous levels of BBS4 at Human, Mouse
运输及保存条件
-15°C to -25°C/1 year(Do not lower than -25°C)
组织表达
Ubiquitously expressed. The highest level of expression is found in the kidney.
细胞定位
Cytoplasm, cytoskeleton, microtubule organizing center, centrosome . Cell projection, cilium membrane . Cytoplasm . Cytoplasm, cytoskeleton, microtubule organizing center, centrosome, centriolar satellite . Cell projection, cilium, flagellum . Cell projection, cilium . Localizes to the pericentriolar material. Centrosomal localization requires dynein (By similarity). Localizes to the connecting cilium of photoreceptor cells (By similarity). .
功能
The BBSome complex is thought to function as a coat complex required for sorting of specific membrane proteins to the primary cilia. The BBSome complex is required for ciliogenesis but is dispensable for centriolar satellite function. This ciliogenic function is mediated in part by the Rab8 GDP/GTP exchange factor, which localizes to the basal body and contacts the BBSome. Rab8(GTP) enters the primary cilium and promotes extension of the ciliary membrane. Firstly the BBSome associates with the ciliary membrane and binds to RAB3IP/Rabin8, the guanosyl exchange factor (GEF) for Rab8 and then the Rab8-GTP localizes to the cilium and promotes docking and fusion of carrier vesicles to the base of the ciliary membrane. The BBSome complex, together with the LTZL1, controls SMO ciliary trafficking and contributes to the sonic hedgehog (SHH) pathway regulation. Required for proper BBSome complex assembly and its ciliary localization. Required for microtubule anchoring at the centrosome but not for microtubule nucleation. May be required for the dynein-mediated transport of pericentriolar proteins to the centrosome.
