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Rabbit Anti-BBS4 Antibody (CSB-PA839407ESR2HU)
Supplier: CUSABIO BIOTECH CO.
BBS4 AntibodyMore alternative names for the antibody
Recommended applications: ELISA, WB, IHC
Recommended dilution: Recommended dilution:WB:1:200-1000,IHC:1:20-1:200
Recommended protocols: check protocols
|Catalogue No.|| |
Recombinant human Bardet-Biedl syndrome 4 protein (350-519AA)
ELISA, WB, IHC
|Recommended dilution|| |
|Molecular weight|| |
Antigen Affinity Purified
Shipped at 4 Celcius Degree. Upon delivery aliquot and store at -20 Celcius Degree or -80 Celcius Degree. Avoid repeated freeze.
|Protein 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. Required for microtubule anchoring at the centrosome but not for microtubule nucleation. May be required for the dynein-mediated transport of pericentriolar proteins to the centrosome. .
|Protein tissue specificity|| |
Ubiquitously expressed. The highest level of expression is found in the kidney.
|Involvement in disease|| |
Bardet-Biedl syndrome 4 (BBS4) [MIM:615982]: A syndrome characterized by usually severe pigmentary retinopathy, early-onset obesity, polydactyly, hypogenitalism, renal malformation and mental retardation. Secondary features include diabetes mellitus, hypertension and congenital heart disease. Bardet-Biedl syndrome inheritance is autosomal recessive, but three mutated alleles (two at one locus, and a third at a second locus) may be required for clinical manifestation of some forms of the disease. . Note=The disease is caused by mutations affecting the gene represented in this entry.
|Protein sequence and domain|| |
Belongs to the BBS4 family.
|Protein cellular localization|| |
Cytoplasm, cytoskeleton, microtubule organizing center, centrosome . Cell projection, cilium membrane . Cytoplasm . Cytoplasm, cytoskeleton, microtubule organizing center, centrosome, centriolar satellite . Cell projection, cilium, flagellum . Cell projection, cilium . Note=Localizes to the pericentriolar material. Centrosomal localization requires dynein (By similarity). Localizes to the connecting cilium of photoreceptor cells (By similarity). .
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CUSABIO BIOTECH CO.
|Product type|| |
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