BBS7 Antibody

中文名稱：

BBS7兔多克隆抗體
貨號：

CSB-PA814219LA01HU
規格：

￥440
圖片：
Immunofluorescence staining of Hela cells with CSB-PA814219LA01HU at 1:133, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).
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) BBS7 Polyclonal antibody
Uniprot No.：

Q8IWZ6
基因名：

BBS7
別名：

Bardet Biedl syndrome 7 protein antibody; Bardet-Biedl syndrome 7 antibody; Bardet-Biedl syndrome 7 protein antibody; BBS2 like 1 antibody; BBS2 like protein 1 antibody; BBS2-like protein 1 antibody; BBS2L1 antibody; BBS7 antibody; BBS7_HUMAN antibody; FLJ10715 antibody
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human Bardet-Biedl syndrome 7 protein (289-393AA)
免疫原種屬：

Homo sapiens (Human)

標記方式：

Non-conjugated

本頁面中的產品，BBS7 Antibody (CSB-PA814219LA01HU)，的標記方式是Non-conjugated。對于BBS7 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-PA814219LB01HU	BBS7 Antibody, HRP conjugated	ELISA
FITC	CSB-PA814219LC01HU	BBS7 Antibody, FITC conjugated
Biotin	CSB-PA814219LD01HU	BBS7 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, IF

推薦稀釋比：

Application	Recommended Dilution
IF	1:50-1:200

Protocols：

ELISA 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. 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.
基因功能參考文獻：
1. Two novel mutations and three previously reported variants, identified in the present study, further extend the body of evidence implicating BBS6, BBS7, BBS8, and BBS10 in causing Bardet-Biedl Syndrome. PMID: 28761321
2. Sequence variants in BBS7 were identified in families with CRB2-related syndrome. PMID: 27004616
3. BBS7 gene was a novel variant (c.103-1G>A) in the consensus splice acceptor site, which altered the splicing recognition site of 'AG' to 'AA' at the BBS7 gene intron 2 and exon 3 boundary. PMID: 25553308
4. A novel Bardet-Biedl syndrome protein is identified anad characterized. PMID: 12567324
5. This study describes a novel mutation in BBS7 causing Bardet-Biedl syndrome in a Chinese family. PMID: 19093007
6. small role of BBS7 and TTC8 in the overall mutational load of Bardet-Biedl syndrome patients PMID: 19402160
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相關疾病：

Bardet-Biedl syndrome 7 (BBS7)
亞細胞定位：

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

Isoform 2 is ubiquitously expressed. Isoform 1 is expressed in retina, lung, liver, testis, ovary, prostate, small intestine, liver, brain, heart and pancreas.
數據庫鏈接：

HGNC: 18758

OMIM: 607590

KEGG: hsa:55212

STRING: 9606.ENSP00000264499

UniGene: Hs.591694

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