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Bardet-Biedl Syndrome 4 (BBS4) (N-Term) antibody

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Synonyms CG13232, Dmel\\CG13232, zgc:152964, AW537059, AW742241, D9Ertd464e
(11), (8), (7), (5), (4), (4), (4), (3), (2), (2), (1)
(73), (22), (20), (6), (6), (4), (2), (2), (2), (2)
(39), (26), (8)
(3), (3), (3), (2), (2), (2), (1), (1), (1), (1), (1), (1), (1), (1), (1)
Immunohistochemistry (IHC), Western Blotting (WB)
(62), (25), (11), (3), (3), (3), (3), (2), (1)
Pubmed 5 references available
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Quantity 100 μL
Shipping to United States ( )
Availability Will be delivered in 7 to 8 Business Days
Immunogen A synthetic peptide from the n-terminal region of human Bardet Biedl syndrome 4 protein (BBS4) conjugated to an immunogenic carrier protein was used as the antigen.
Specificity Specific for BBS4.
Purification Whole serum
Alternative Name BBS4 (BBS4 Antibody Abstract)
Background Function: May be required for the dynein-mediated transport of pericentriolar proteins to the centrosome. Required for microtubule anchoring at the centrosome but not for microtubule nucleation. 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 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.
Subcellular location: Centrosome. Cytoplasm › cytoskeleton. Cell projection › cilium membrane. Cytoplasm. Note: Localizes to the pericentriolar region throughout the cell cycle. Centrosomal localization requires dynein. Localizes to nonmembranous centriolar satellites in the cytoplasm.
Tissue specificity: Ubiquitously expressed. The highest level of expression is found in the kidney. Also known as: BBS4.
Research Area Neurology
Pathways Hedgehog Signaling
Application Notes A dilution of 1 : 300 to 1 : 2000 is recommended.
The optimal dilution should be determined by the end user.
Not yet tested in other applications.
Restrictions For Research Use only
Format Lyophilized
Reconstitution Reconstitute in 100 µL of sterile water. Centrifuge to remove any insoluble material.
Handling Advice Avoid freeze and thaw cycles.
Storage 4 °C/-20 °C
Storage Comment Maintain the lyophilised/reconstituted antibodies frozen at -20°C for long term storage and refrigerated at 2-8°C for a shorter term. When reconstituting, glycerol (1:1) may be added for an additional stability. Avoid freeze and thaw cycles.
Expiry Date 12 months
Background publications Hichri, Stoetzel, Laurier et al.: "Testing for triallelism: analysis of six BBS genes in a Bardet-Biedl syndrome family cohort." in: European journal of human genetics : EJHG, Vol. 13, Issue 5, pp. 607-16, 2005 (PubMed).

Ye, Dai, Fang et al.: "Cloning and characterization of a splice variant of human Bardet-Biedl syndrome 4 gene (BBS4)." in: DNA sequence : the journal of DNA sequencing and mapping, Vol. 15, Issue 3, pp. 213-8, 2004 (PubMed).

Kim, Badano, Sibold et al.: "The Bardet-Biedl protein BBS4 targets cargo to the pericentriolar region and is required for microtubule anchoring and cell cycle progression." in: Nature genetics, Vol. 36, Issue 5, pp. 462-70, 2004 (PubMed).

Katsanis, Eichers, Ansley et al.: "BBS4 is a minor contributor to Bardet-Biedl syndrome and may also participate in triallelic inheritance." in: American journal of human genetics, Vol. 71, Issue 1, pp. 22-9, 2002 (PubMed).

Mykytyn, Braun, Carmi et al.: "Identification of the gene that, when mutated, causes the human obesity syndrome BBS4." in: Nature genetics, Vol. 28, Issue 2, pp. 188-91, 2001 (PubMed).

Catalog No. ABIN350114
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