BBS4 Antibody

說明書

中文名稱：

BBS4兔多克隆抗體
貨號：

CSB-PA839407ESR2HU
規格：

￥440
圖片：
Western blot
All lanes: Bardet-Biedl syndrome 4 protein antibody at 4μg/ml
Lane 1: Hela whole cell lysate
Lane 2: U251 whole cell lysate
Lane 2: Mouse heart tissue
Secondary
Goat polyclonal to rabbit IgG at 1/10000 dilution
Predicted band size: 59, 60, 39 kDa
Observed band size: 59 kDa

Immunohistochemistry of paraffin-embedded human breast cancer using CSB-PA839407ESR2HU at dilution of 1:100

Immunohistochemistry of paraffin-embedded human kidney tissue using CSB-PA839407ESR2HU at dilution of 1:100
其他：

產品詳情
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靶點詳情

產品詳情

產品名稱：

Rabbit anti-Homo sapiens (Human) BBS4 Polyclonal antibody
Uniprot No.：

Q96RK4
基因名：

BBS4
別名：

Bardet Biedl syndrome 4 protein antibody; Bardet-Biedl syndrome 4 protein antibody; Bbs4 antibody; BBS4_HUMAN antibody
宿主：

Rabbit
反應種屬：

Human, Mouse
免疫原：

Recombinant Human Bardet-Biedl syndrome 4 protein (350-519AA)
免疫原種屬：

Homo sapiens (Human)
標記方式：

Non-conjugated
克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

Antigen Affinity Purified
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
產品提供形式：

Liquid
應用范圍：

ELISA, WB, IHC

推薦稀釋比：

Application	Recommended Dilution
WB	1:200-1:1000
IHC	1:20-1:200

Protocols：

ELISA Protocol
Western Blotting (WB) Protocol
Immunohistochemistry (IHC) Protocol
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
貨期：

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

功能：

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.
基因功能參考文獻：
1. a novel nonsense mutation in BBS4 gene in a Chinese family with Bardet-Biedl syndrome. This homozygous mutation was predicted to completely abolish the synthesis of the BBS4 protein; a rare heterozygous missense SNP in BBS10 gene was also detected PMID: 25533820
2. Results present evidence of a role for BBS4 in mediating the phosphorylation of TrkB by BDNF and its activation requires a proper localization to the ciliary axoneme. PMID: 24867303
3. Findings indicate that Bbs proteins play a central role in the regulation of the actin cytoskeleton and control the cilia length through alteration of RhoA levels. PMID: 23716571
4. Ectopic expression of human BBS4 can rescue Bardet-Biedl syndrome phenotypes in Bbs4 null mice. PMID: 23554981
5. A novel missense mutation in BBS4 that co-segregates with Leber Congenital Amaurosis was identified in a consanguineous family from Saudi Arabia. PMID: 22219648
6. BBS4 is a minor contributor to Bardet-Biedl syndrome and may also participate in triallelic inheritance. Evaluated the spectrum of mutations in the recently identified BBS4 gene with a combination of haplotype analysis and mutation screening. PMID: 12016587
7. The phenotype of patients with BBS4 mutations consists of severe retinitis pigmentosa, variable obesity, brachydactyly with variable polydactyly, small or missing teeth, genital hypoplasia, and cardiovascular disease. PMID: 12365916
8. A novel Frameshift Mutation between the splice donor site and exon 5 of BBS4 in a Bardet-Biedl syndrome patient and a novel heterozygous base substitution in both an affected mother and her affected daughter. PMID: 12872256
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相關疾?。?/div>
Bardet-Biedl syndrome 4 (BBS4)
亞細胞定位：

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.
蛋白家族：

BBS4 family
組織特異性：

Ubiquitously expressed. The highest level of expression is found in the kidney.
數據庫鏈接：

HGNC: 969

OMIM: 600374

KEGG: hsa:585

STRING: 9606.ENSP00000268057

UniGene: Hs.208681

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