货号：AB15009

品牌：Merck Millipore

规格：EA

目录价：￥4068.00

市场价格：￥3457.80

会员价格：￥3254.40

金山科研平台，产品价格货期咨询微信：jinshanbio Description: Anti-BBS4 Antibody | AB15009 View All» Promotional Text: Special Shipping Offer on Antibodies100% Performance Guaranteed View All» Specificity: This antibody recognizes BBS4. View All» Molecular Weight: ~ 58 kDa View All» Immunogen: KLH conjugated synthetic linear peptide. View All» Background Information: This gene encodes a protein which contains tetratricopeptide repeats (TPR), similar to O-linked N-acetyglucosamine transferase. Mutations in this gene have been observed in patients with Bardet-Biedl syndrome type 4. BBS proteins do not show extensive homology with proteins of known function. Based on the observations that BBS8 localizes to the basal body of ciliated cells and expression of the Caenorhabditis elegans orthologues of several BBS proteins are limited to ciliated cells, it has been hypothesized that BBS is the result of a defect in cilia assembly or function. The functions of the proteins encoded by the Bardet–Biedl syndrome (BBS) genes are unknown. Mutations in these genes lead to the pleiotropic human disorder BBS, which is characterized by obesity, retinopathy, polydactyly, renal and cardiac malformations, learning disabilities, and hypogenitalism. Secondary features include diabetes mellitus and hypertension. View All» Species Reactivity:

• Mouse

• Rat
  View All» Species Reactivity Note: Mouse, and Rat. Predicted to react with horse, monkey, primate and 14/16 aa Danio, 15/16 aa Xenopus.. View All» Control: Mouse brain tissue lysate View All» Quality Assurance: Western Blot Analysis: 1:5,000 dilution of this lot detected BBS4 on 10 ug of mouse brain lysate. View All» Purification Method: Antigen affinity purified View All» Presentation: Purified Rabbit polyclonal antibody in buffer containing 0.1M Tris-Glycine (pH 7.4) 15mM NaCl with 0.05% NaN3. View All» Storage Conditions: Stable for 1 year at 2-8ºC from date of receipt. View All» UniProt Number: Q96RK4 View All» Entrez Gene Number: NP_149017 View All» Usage Statement: Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals. View All» Key Applications: Western Blotting View All» Entrez Gene Summary: This gene is a member of the Bardet-Biedl syndrome (BBS) gene family. Bardet-Biedl syndrome is an autosomal recessive disorder characterized by severe pigmentary retinopathy, obesity, polydactyly, renal malformation and mental retardation. The proteins encoded by BBS gene family members are structurally diverse. The similar phenotypes exhibited by mutations in BBS gene family members are likely due to the protein's shared roles in cilia formation and function. Many BBS proteins localize to the basal bodies, ciliary axonemes, and pericentriolar regions of cells. BBS proteins may also be involved in intracellular trafficking via microtubule-related transport. The protein encoded by this gene has sequence similarity to O-linked N-acetylglucosamine (O-GlcNAc) transferases in plants and archaebacteria and in human forms a multi-protein "BBSome" complex with six other BBS proteins. Alternative splice variants have been described but their predicted protein products have not been experimentally verified. View All» UniProt Summary: 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. Subunit structure: Part of BBSome complex, that contains BBS1, BBS2, BBS4, BBS5, BBS7, BBS8, BBS9 and BBIP10. The BBSome complex binds to PCM1 and tubulin. Interacts with DCTN1. 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.Involvement in disease: Defects in BBS4 are the cause of Bardet-Biedl syndrome type 4 (BBS4) [MIM:209900]. Bardet-Biedl syndrome (BBS) is a genetically heterogeneous, autosomal recessive disorder 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. A relatively high incidence of BBS is found in the mixed Arab populations of Kuwait and in Bedouin tribes throughout the Middle East, most likely due to the high rate of consaguinity in these populations and a founder effect. Sequence similarities: Belongs to the BBS4 family.Contains 10 TPR repeats. View All» Product Name: Anti-BBS4 View All» Concentration: 1 mg/mL View All» Antibody Type: Polyclonal Antibody View All» Qty/Pk: 100 µg View All» Format: Purified View All» Host: Rabbit View All»
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