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BBS1

Bardet-Biedl syndrome 1

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.

Gene Name: Bardet-Biedl syndrome 1
Synonyms: BBS1, BBS2-like protein 2, Bardet-Biedl syndrome 1, BBS2L2
Target Sequences: NM_024649 NP_078925.3 Q8NFJ9

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Proteins (4)
Over-Expression Lysate (2)
Recombinant (2)
BBS1 (4)
No (4)
GST (1)
GST, N-terminus (1)
Myc-DDK (Flag) (2)
Human (3)
293T Cells (1)
HEK 293 Cells (1)
Wheat Germ Extract (2)
Purified (1)
BBS1 Protein - 12.5% SDS-PAGE Stained with Coomassie Blue.
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Wheat Germ Extract
GST, N-terminus
10 µg/$479; 25 µg/$670
BBS1 Protein - Western validation with an anti-DDK antibody * L: Control HEK293 lysate R: Over-expression lysate
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293T Cells
Myc-DDK (Flag)
64.9 kDa
20 µg/$150
BBS1 Protein - 12.5% SDS-PAGE of human BBS1 stained with Coomassie Blue
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Wheat Germ Extract
GST
77.8 kDa
2 µg/$439
BBS1 Protein - Western validation with an anti-DDK antibody * L: Control HEK293 lysate R: Over-expression lysate
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HEK 293 Cells
Myc-DDK (Flag)
64.9 kDa
100 µg/$494
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If you do not find the reagent or information you require, please contact Customer.Support@LSBio.com to inquire about additional products in development.

PLEASE NOTE

For RESEARCH USE ONLY. Intended for use by laboratory professionals. Not intended for human diagnostic or therapeutic purposes.

The data on this page has been compiled from LifeSpan internal sources, the National Center for Biotechnology Information (NCBI), and The Universal Protein Resource (UniProt).