BBS5 Antibody, HRP conjugated

Datasheet
Code CSB-PA847625LB01HU
Size US$299
Uniprot No. Q8N3I7
Protocols ELISA Protocol
Immunogen Recombinant Human Bardet-Biedl syndrome 5 protein (224-317AA)
Raised in Rabbit
Species Reactivity Human
Tested Applications ELISA
Relevance 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 BBSome complex ciliary localization but not for the proper complex assembly.
Form Liquid
Conjugate HRP
Storage Buffer Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
Purification Method >95%, Protein G purified
Isotype IgG
Clonality Polyclonal
Alias Bardet-Biedl syndrome 5 protein, BBS5
Immunogen Species Homo sapiens (Human)
Research Area Others
Target Names BBS5
Storage Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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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 BBSome complex ciliary localization but not for the proper complex assembly.
Involvement in disease Bardet-Biedl syndrome 5 (BBS5)
Subcellular Location Cell projection, cilium membrane, Cytoplasm, Cytoplasm, cytoskeleton, cilium basal body, Cytoplasm, cytoskeleton, microtubule organizing center, centrosome, centriolar satellite
Protein Families BBS5 family
Database Links

HGNC: 970

OMIM: 603650

KEGG: hsa:129880

STRING: 9606.ENSP00000295240

UniGene: Hs.233398

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