Key features and details
- Goat polyclonal to BBS8
- Suitable for: WB
- Reacts with: Human
- Isotype: IgG
Product nameAnti-BBS8 antibody
DescriptionGoat polyclonal to BBS8
Specificityab101731 is expected to recognize all reported isoforms (NP_653197.2; NP_938051.1; NP_938052.1).
Tested applicationsSuitable for: WBmore details
Species reactivityReacts with: Human
Predicted to work with: Mouse, Rat, Chicken, Cow, Dog, Pig
- Human Testis lysate
This product was previously labelled as TTC8
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Storage instructionsShipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid repeated freeze / thaw cycles.
Storage bufferpH: 7.30
Preservative: 0.02% Sodium azide
Constituents: 0.5% BSA, Tris buffered saline
Concentration information loading...
PurityImmunogen affinity purified
Purification notesab101731 was purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.
Our Abpromise guarantee covers the use of ab101731 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||Use a concentration of 0.2 - 0.6 µg/ml. Detects a band of approximately 60 kDa (predicted molecular weight: 62 kDa).
1 hour primary incubation is recommended for this product.
FunctionThe 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.
Tissue specificityWidely expressed.
Involvement in diseaseDefects in TTC8 are the cause of retinitis pigmentosa type 51 (RP51) [MIM:613464]. It is a retinal dystrophy belonging to the group of pigmentary retinopathies. Retinitis pigmentosa is characterized by retinal pigment deposits visible on fundus examination and primary loss of rod photoreceptor cells followed by secondary loss of cone photoreceptors. Patients typically have night vision blindness and loss of midperipheral visual field. As their condition progresses, they lose their far peripheral visual field and eventually central vision as well.
Defects in TTC8 are the cause of Bardet-Biedl syndrome type 8 (BBS8) [MIM:209900]. Bardet-Biedl syndrome (BBS) is a genetically heterogeneous, autosomal recessive 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.
Sequence similaritiesContains 8 TPR repeats.
Cellular localizationCytoplasm > cytoskeleton > centrosome. Cell projection > cilium membrane. Cytoplasm. Localizes to nonmembranous centriolar satellites in the cytoplasm.
- Information by UniProt
- Bardet Biedl syndrome 8 protein antibody
- Bardet Biedl syndrome type 8 antibody
- Bardet-Biedl syndrome 8 protein antibody
ab101731 has not yet been referenced specifically in any publications.