BBS9 Antibody

中文名稱：

BBS9兔多克隆抗體
貨號：

CSB-PA659641LA01HU
規格：

￥440
圖片：
Immunohistochemistry of paraffin-embedded human liver tissue using CSB-PA659641LA01HU at dilution of 1:100

Immunofluorescent analysis of Hela cells using CSB-PA659641LA01HU at dilution of 1:100 and Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L)
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) BBS9 Polyclonal antibody
Uniprot No.：

Q3SYG4
基因名：

BBS9
別名：

B1 antibody; Bardet Biedl syndrome 9 antibody; Bardet-Biedl syndrome 9 protein antibody; bbs9 antibody; C18 antibody; D1 antibody; MGC118917 antibody; OTTHUMP00000158833 antibody; OTTHUMP00000202918 antibody; OTTHUMP00000202919 antibody; OTTHUMP00000202920 antibody; Parathyroid hormone-responsive B1 gene protein antibody; Protein PTHB1 antibody; PTH-responsive osteosarcoma B1 protein antibody; PTHB1 antibody; PTHB1_HUMAN antibody
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human Protein PTHB1 protein (8-185AA)
免疫原種屬：

Homo sapiens (Human)

標記方式：

Non-conjugated

本頁面中的產品，BBS9 Antibody (CSB-PA659641LA01HU)，的標記方式是Non-conjugated。對于BBS9 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-PA659641LB01HU	BBS9 Antibody, HRP conjugated	ELISA
FITC	CSB-PA659641LC01HU	BBS9 Antibody, FITC conjugated
Biotin	CSB-PA659641LD01HU	BBS9 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, IF

推薦稀釋比：

Application	Recommended Dilution
IHC	1:20-1:200
IF	1:50-1:200

Protocols：

ELISA Protocol
Immunohistochemistry (IHC) Protocol
Immunofluorescence (IF) 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. Required for proper BBSome complex assembly and its ciliary localization.
基因功能參考文獻：
1. Our results provide evidence for new loci influencing abdominal visceral (BBS9, ADCY8, KCNK9) and subcutaneous (MLLT10/DNAJC1/EBLN1) fat, and confirmed a locus (THNSL2) previously reported to be associated with abdominal fat in women PMID: 26480920
2. BBS9/PTHB1 gene mutations have been shown to be associated with Bardet Biedl syndrome and to the best of our knowledge this study reports the first Pakistani family linked to the BBS9 gene. PMID: 26846096
3. BBS9 has four folded domains, based on structure prediction; the N-terminal domain is a beta-propeller. PMID: 26085087
4. 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
5. The endoplasmic reticulum membrane J protein C18 executes a distinct role in promoting simian virus 40 membrane penetration. PMID: 25631089
6. Robust associations with nonsyndromic sagittal craniosynostosis were found in a 120-kb region downstream of BMP2 flanked by rs1884302 and rs6140226 and within a 167-kb region of BBS9 between rs10262453 and rs17724206. PMID: 23160099
7. Human BBS9 mRNA rescues bbs9 knockdown phenotype in the zebrafish. PMID: 22479622
8. gene is interrupted by a t(1;7)(q42;p15) breakpoint associated with Wilms' tumour; new alternately spliced isoforms were found in a wide range of adult and foetal tissues PMID: 12618763
9. Comparative genomics and gene expression analysis identifies PHTB1 protein as BBS9, a new Bardet-Biedl syndrome gene. PMID: 16380913
10. PTHB1 is strongly associated with POF, and ht1 confers susceptibility to POF PMID: 18349106
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相關疾病：

Bardet-Biedl syndrome 9 (BBS9)
亞細胞定位：

Cytoplasm, cytoskeleton, microtubule organizing center, centrosome. Cell projection, cilium membrane. Cytoplasm. Cytoplasm, cytoskeleton, microtubule organizing center, centrosome, centriolar satellite.
組織特異性：

Widely expressed. Expressed in adult heart, skeletal muscle, lung, liver, kidney, placenta and brain, and in fetal kidney, lung, liver and brain.
數據庫鏈接：

HGNC: 30000

OMIM: 607968

KEGG: hsa:27241

STRING: 9606.ENSP00000242067

UniGene: Hs.372360

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