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Bardet-Biedl syndrome 1 protein

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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.

Below are the list of possible Bardet-Biedl syndrome 1 protein products. If you cannot find the target and/or product is not available in our catalog, please click here to contact us and request the product or submit your request for custom elisa kit production, custom recombinant protein production or custom antibody production. Custom ELISA Kits, Recombinant Proteins and Antibodies can be designed, manufactured and produced according to the researcher's specifications.
 

Bardet-Biedl syndrome 1 protein

 Bardet-Biedl syndrome 1 protein ELISA Kit
 Bardet-Biedl syndrome 1 protein Recombinant
 Bardet-Biedl syndrome 1 protein Antibody
Also known as Bardet-Biedl syndrome 1 protein (BBS2-like protein 2).
BBS1: defects in BBS1 are a cause of Bardet-Biedl syndrome type (BBS). BBS is characterized by usually severe pigmentary retinopathy, early onset obesity, polydactyly, hypogenitalism, renal malformation and mental retardation. Two alternatively splioced isoforms have been reported.

Protein type: Unknown function
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>Chromosomal Location of Human Ortholog: 11q13

Cellular Component: axoneme; centrosome; cytosol

Molecular Function: patched binding; protein binding; smoothened binding

Biological Process: Golgi to plasma membrane protein transport; photoreceptor cell maintenance; retinal homeostasis

Disease: Bardet-biedl Syndrome 1
 BBS1 ELISA Kit
 BBS1 Recombinant
 BBS1 Antibody
 BBS2L2 ELISA Kit
 BBS2L2 Recombinant
 BBS2L2 Antibody
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Bardet-Biedl syndrome 1 protein homolog

 Bardet-Biedl syndrome 1 protein homolog ELISA Kit
 Bardet-Biedl syndrome 1 protein homolog Recombinant
 Bardet-Biedl syndrome 1 protein homolog Antibody
Component of the BBSome complex (). 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 (). Required for proper BBSome complex assembly and its ciliary localization (PubMed:22922713). Required for cilia biogenesis a
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nd both the assembly and movement of intraflagellar transport proteins along the ciliary axoneme (PubMed:15231740, PubMed:22022287, PubMed:22922713).
 bbs-1 ELISA Kit
 bbs-1 Recombinant
 bbs-1 Antibody
 Y105E8A.5 ELISA Kit
 Y105E8A.5 Recombinant
 Y105E8A.5 Antibody
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