Rabbit anti-Human TTC8 Polyclonal Antibody

Uniprot Id

Q8TAM2

Target Species

Human

Target Name

TTC8

Target Full Name

Tetratricopeptide repeat protein 8

Target Function

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.

Target Involvement

Retinitis pigmentosa 51 (RP51); Bardet-Biedl syndrome 8 (BBS8)

Target Subcellular Location

Cytoplasm, cytoskeleton, microtubule organizing center, centrosome. Cell projection, cilium membrane. Cytoplasm. Cytoplasm, cytoskeleton, microtubule organizing center, centrosome, centriolar satellite. Cell projection, cilium.

Target Tissue Specificity

Widely expressed.

Target Synonyms

Bardet Biedl syndrome 8 protein; Bardet Biedl syndrome type 8; Bardet-Biedl syndrome 8 protein; BBS8; RP51; Tetratricopeptide repeat domain 8; Tetratricopeptide repeat protein 8; TPR repeat protein 8; TTC 8; Ttc8; TTC8_HUMAN