BBS10 Antibody

中文名稱：

BBS10兔多克隆抗體
貨號：

CSB-PA823443LA01HU
規格：

￥440
圖片：
Immunohistochemistry of paraffin-embedded human liver cancer using CSB-PA823443LA01HU at dilution of 1:100
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

產品名稱：

Rabbit anti-Homo sapiens (Human) BBS10 Polyclonal antibody
Uniprot No.：

Q8TAM1
基因名：

BBS10
別名：

BBS10 antibody; C12orf58Bardet-Biedl syndrome 10 protein antibody
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human Bardet-Biedl syndrome 10 protein (435-552AA)
免疫原種屬：

Homo sapiens (Human)

標記方式：

Non-conjugated

本頁面中的產品，BBS10 Antibody (CSB-PA823443LA01HU)，的標記方式是Non-conjugated。對于BBS10 Antibody，我們還提供其他標記。見下表：

標記方式	貨號	產品名稱	應用
HRP	CSB-PA823443LB01HU	BBS10 Antibody, HRP conjugated	ELISA
FITC	CSB-PA823443LC01HU	BBS10 Antibody, FITC conjugated
Biotin	CSB-PA823443LD01HU	BBS10 Antibody, Biotin conjugated	ELISA

克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

>95%, Protein G purified
濃度：

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

Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
產品提供形式：

Liquid
應用范圍：

ELISA, IHC

推薦稀釋比：

Application	Recommended Dilution
IHC	1:20-1:200

Protocols：

ELISA 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：

Probable molecular chaperone that assists the folding of proteins upon ATP hydrolysis. Plays a role in the assembly of BBSome, a complex involved in ciliogenesis regulating transports vesicles to the cilia. Involved in adipogenic differentiation.
基因功能參考文獻：
1. In the 64 BBS patients (44 males, 20 females) were studied, mutations were predominant in BBS10 and ARL6 genes; the c.272T>C; p.(I91T) mutation in ARL6 gene was a recurrent mutation PMID: 29806606
2. Genetic analysis revealed compound heterozygous BBS10 mutations in the patient: a novel missense mutation c.98G>A PMID: 29666954
3. we report here, for the first time, in Indian population, a novel, different profile of mutations in BBS genes (BBS3, BBS9, BBS10 and BBS2) compared to worldwide (BBS1 and 10) reports. PMID: 24400638
4. novel BBS10 mutations in Bardet-Biedl syndrome patients in Spain PMID: 24611592
5. Novel mutation (c.1181_1182insGCATTTATACC) in BBS10 (p.S396Lfs*6) found in Tunisian families with Bardet-Biedl syndrome. PMID: 23432027
6. A rare variant (c.1189A>G [p.Ile397Val]; rs202042386) confers risk of type 2 diabetes in a recessive state. PMID: 25439097
7. We report two affected brothers from a consanguineous Pakistani Punjabi family, both the brothers were homozygous for c.1958_1967del, which is a novel deletion in BBS10 that is likely to be causing the Bardet-Biedl syndrome in this family. PMID: 23403234
8. Mutations identified in the present study extend the body of evidence implicating the genes ARL6 and BBS10 in causing Bardet-Biedl syndrome. PMID: 23219996
9. This study confirms the high frequency of BBS10 mutations, particularly of the p.Cys91LeufsX5 allele in Bardet-Biedl syndrome. PMID: 20805367
10. Mutation in BBS10 modulates Bardet-Biedl syndrome in a sibling. PMID: 20827784
11. Using sequence analysis, the role of BBS6, 10 and 12 was assessed in a Bardet-Biedl syndrome patient population comprising 93 cases from 74 families. PMID: 20472660
12. Detected in a family with high consanguinity and Bardet-Biedl syndrome. PMID: 17101080
13. the BBS10 and BBS12 proteins are located within the basal body of this primary cilium and inhibition of their expression impairs ciliogenesis, activates the GSK3 pathway, and induces PPAR nuclear accumulation, hence favoring adipogenesis PMID: 19190184
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相關疾病：

Bardet-Biedl syndrome 10 (BBS10)
亞細胞定位：

Cell projection, cilium.
蛋白家族：

TCP-1 chaperonin family
數據庫鏈接：

HGNC: 26291

OMIM: 610148

KEGG: hsa:79738

STRING: 9606.ENSP00000376946

UniGene: Hs.96322

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