Bbs1 (mouse) Antibody (internal region)
Peptide-affinity purified goat antibody
- SPECIFICATION
- CITATIONS
- PROTOCOLS
- BACKGROUND
Application
| E |
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Primary Accession | Q8NFJ9 |
Other Accession | NP_001028300.1, 582, 52028 (mouse), 309156 (rat) |
Predicted | Human, Mouse, Rat, Dog |
Host | Goat |
Clonality | Polyclonal |
Concentration | 0.5 mg/ml |
Isotype | IgG |
Calculated MW | 65083 Da |
Gene ID | 582 |
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Other Names | Bardet-Biedl syndrome 1 protein, BBS2-like protein 2, BBS1, BBS2L2 |
Format | 0.5 mg/ml in Tris saline, 0.02% sodium azide, pH7.3 with 0.5% bovine serum albumin |
Storage | Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles. |
Precautions | Bbs1 (mouse) Antibody (internal region) is for research use only and not for use in diagnostic or therapeutic procedures. |
Name | BBS1 |
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Synonyms | BBS2L2 |
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 (PubMed:17574030, PubMed:22072986). Plays a role in olfactory cilium biogenesis/maintenance and trafficking (By similarity). |
Cellular Location | Cell projection, cilium membrane. Cytoplasm. Cytoplasm, cytoskeleton, microtubule organizing center, centrosome, centriolar satellite |
Tissue Location | Highly expressed in the kidney. Also found in fetal tissue, testis, retina, adipose tissue, heart, skeletal muscle and pancreas |
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Provided below are standard protocols that you may find useful for product applications.
References
Bardet-biedl syndrome: an atypical phenotype in brothers with a proven BBS1 mutation. Cannon PS, Clayton-Smith J, Beales PL, Lloyd IC, Ophthalmic genetics 2008 Sep 29 (3): 128-32. PMID: 18766993
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