BBS5 Antibody (PACO61109)
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주문- SKU:
- PACO61109
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- IHC
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Frequently bought together:
Description
Antibody Name: | BBS5 Antibody (PACO61109) |
Antibody SKU: | PACO61109 |
Size: | 50ug |
Host Species: | Rabbit |
Tested Applications: | ELISA, IHC |
Recommended Dilutions: | ELISA:1:2000-1:10000, IHC:1:200-1:500 |
Species Reactivity: | Human |
Immunogen: | Recombinant Human Bardet-Biedl syndrome 5 protein (224-317AA) |
Form: | Liquid |
Storage Buffer: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, pH 7.4 |
Purification Method: | >95%, Protein G purified |
Clonality: | Polyclonal |
Isotype: | IgG |
Conjugate: | Non-conjugated |
IHC image of PACO61109 diluted at 1:200 and staining in paraffin-embedded human kidney tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system. |
Background: | 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 BBSome complex ciliary localization but not for the proper complex assembly. |
Synonyms: | Bardet-Biedl syndrome 5 protein, BBS5 |
UniProt Protein Function: | BBS5: 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. Defects in BBS5 are a cause of Bardet-Biedl syndrome type 5 (BBS5). Bardet-Biedl syndrome (BBS) is a genetically heterogeneous 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. Belongs to the BBS5 family. 2 isoforms of the human protein are produced by alternative splicing. |
UniProt Protein Details: | Chromosomal Location of Human Ortholog: 2q31.1 Cellular Component: cytosol; intracellular Molecular Function:phosphatidylinositol 3-phosphate binding; protein binding Biological Process: heart looping; intracellular transport; melanosome transport Disease: Bardet-biedl Syndrome 5 |
NCBI Summary: | This gene encodes a protein that has been directly linked to Bardet-Biedl syndrome. The primary features of this syndrome include retinal dystrophy, obesity, polydactyly, renal abnormalities and learning disabilities. Experimentation in non-human eukaryotes suggests that this gene is expressed in ciliated cells and that it is required for the formation of cilia. Alternate transcriptional splice variants have been observed but have not been fully characterized. [provided by RefSeq, Jul 2008] |
UniProt Code: | Q8N3I7 |
NCBI GenInfo Identifier: | 74750959 |
NCBI Gene ID: | 129880 |
NCBI Accession: | Q8N3I7.1 |
UniProt Secondary Accession: | Q8N3I7,Q6PKN0, D3DPC3, |
UniProt Related Accession: | Q8N3I7 |
Molecular Weight: | 39kDa |
NCBI Full Name: | Bardet-Biedl syndrome 5 protein |
NCBI Synonym Full Names: | Bardet-Biedl syndrome 5 |
NCBI Official Symbol: | BBS5 |
NCBI Protein Information: | Bardet-Biedl syndrome 5 protein |
UniProt Protein Name: | Bardet-Biedl syndrome 5 protein |
Protein Family: | Bardet-Biedl syndrome 5 protein |
UniProt Gene Name: | BBS5 |
Antibodies |
Anti-BBS5 Antibody (CAB12681) |
Secondary Antibody |
Anti-HRP Goat Anti-Rabbit IgG (H+L) Antibody (CABS014) |
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