BBS10 Antibody (A-3): sc-518239
- BBS10 Antibody (A-3) is a mouse monoclonal IgG1 κ provided at 200 µg/ml
- specific for an epitope mapping between amino acids 444-464 of BBS10 of human origin
- recommended for detection of BBS10 of human origin by WB, IP, IF and ELISA
- Anti-BBS10 Antibody (A-3) is available conjugated to agarose for IP; HRP for WB, IHC(P) and ELISA; and to either phycoerythrin or FITC for IF, IHC(P) and FCM
- also available conjugated to Alexa Fluor® 488, Alexa Fluor® 546, Alexa Fluor® 594 or Alexa Fluor® 647 for WB (RGB), IF, IHC(P) and FCM, and for use with RGB fluorescent imaging systems, such as iBright™ FL1000, FluorChem™, Typhoon, Azure and other comparable systems
- also available conjugated to Alexa Fluor® 680 or Alexa Fluor® 790 for WB (NIR), IF and FCM; for use with Near-Infrared (NIR) detection systems, such as LI-COR®Odyssey®, iBright™ FL1000, FluorChem™, Typhoon, Azure and other comparable systems
- m-IgG1 BP-HRP is the preferred secondary detection reagent for BBS10 Antibody (A-3) for WB applications. This reagent is now offered in a bundle with BBS10 Antibody (A-3) (see ordering information below).
BBS10 Antibody (A-3) is a mouse monoclonal IgG1 kappa light chain antibody specific for an epitope mapping between amino acids 444-464 of BBS10 of human origin by western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), and enzyme-linked immunosorbent assay (ELISA). BBS10 monoclonal antibody (A-3) is available in both non-conjugated and various conjugated forms, including agarose, horseradish peroxidase (HRP), phycoerythrin (PE), fluorescein isothiocyanate (FITC), and multiple Alexa Fluor® conjugates. Bardet-Biedl syndrome (BBS) is a complex genetic disorder that manifests through a range of symptoms, including obesity, photoreceptor degeneration, polydactyly, hypogenitalism, renal abnormalities, and developmental delays, with additional complications such as diabetes, hypertension, and congenital heart defects. Bardet-Biedl syndrome 10 protein (BBS10), also known as C12orf58, or FLJ23560, is a 723 amino acid protein that plays a crucial role in the assembly and function of cilia, which are essential for various cellular processes. BBS10 (A-3) antibody recognizes proteins that localize to the basal body of the primary cilium and are involved in proper protein folding through function as a member of the TCP-1 chaperonin family, utilizing ATP hydrolysis for energy. BBS10′s role in ciliogenesis is underscored by findings that inhibition can disrupt cilia formation, activate the glycogen synthase kinase 3 pathway, and lead to accumulation of peroxisome proliferator-activated receptor in the nucleus. The gene encoding BBS10 consists of two exons and is located on human chromosome 12q21.2, highlighting importance in the genetic landscape of Bardet-Biedl syndrome and associated phenotypes. These characteristics underscore anti-BBS10 antibody (A-3) as a valuable tool for studying molecular mechanisms of ciliogenesis and genetic underpinnings of Bardet-Biedl syndrome.
Alexa Fluor® is a trademark of Molecular Probes Inc., OR., USA
LI-COR® and Odyssey® are registered trademarks of LI-COR Biosciences
BBS10 Antibody (A-3) References:
- BBS10 encodes a vertebrate-specific chaperonin-like protein and is a major BBS locus. | Stoetzel, C., et al. 2006. Nat Genet. 38: 521-4. PMID: 16582908
- Pitfalls of homozygosity mapping: an extended consanguineous Bardet-Biedl syndrome family with two mutant genes (BBS2, BBS10), three mutations, but no triallelism. | Laurier, V., et al. 2006. Eur J Hum Genet. 14: 1195-203. PMID: 16823392
- Identification of a novel BBS gene (BBS12) highlights the major role of a vertebrate-specific branch of chaperonin-related proteins in Bardet-Biedl syndrome. | Stoetzel, C., et al. 2007. Am J Hum Genet. 80: 1-11. PMID: 17160889
- Retinal morphology in patients with BBS1 and BBS10 related Bardet-Biedl Syndrome evaluated by Fourier-domain optical coherence tomography. | Gerth, C., et al. 2008. Vision Res. 48: 392-9. PMID: 17980398
- Transient ciliogenesis involving Bardet-Biedl syndrome proteins is a fundamental characteristic of adipogenic differentiation. | Marion, V., et al. 2009. Proc Natl Acad Sci U S A. 106: 1820-5. PMID: 19190184
- BBS10 mutations are common in 'Meckel'-type cystic kidneys. | Putoux, A., et al. 2010. J Med Genet. 47: 848-52. PMID: 20805367
- Comparing the Bbs10 complete knockout phenotype with a specific renal epithelial knockout one highlights the link between renal defects and systemic inactivation in mice. | Cognard, N., et al. 2015. Cilia. 4: 10. PMID: 26273430
- Bardet-Biedl Syndrome as a Chaperonopathy: Dissecting the Major Role of Chaperonin-Like BBS Proteins (BBS6-BBS10-BBS12). | Álvarez-Satta, M., et al. 2017. Front Mol Biosci. 4: 55. PMID: 28824921
- Multi-Omics Studies Unveil Extraciliary Functions of BBS10 and Show Metabolic Aberrations Underlying Renal Disease in Bardet-Biedl Syndrome. | Marchese, E., et al. 2022. Int J Mol Sci. 23: PMID: 36012682
- Computational and Structural Analysis to Assess the Pathogenicity of Bardet-Biedl Syndrome Related Missense Variants Identified in Bardet-Biedl Syndrome 10 Gene (BBS10). | Gupta, N., et al. 2022. ACS Omega. 7: 37654-37662. PMID: 36312387
- Prenatal diagnosis of Bardet‑Biedl syndrome due to novel variants in the BBS10 gene in a fetus with multiple anomalies: A case report. | Dong, X., et al. 2022. Exp Ther Med. 24: 721. PMID: 36340607
- Integration of multi-omics reveals the important role of the BBS10 gene in reproduction. | Zhang, G., et al. 2024. J Anim Sci. 102: PMID: 39315571
Ordering Information
| Product Name | Catalog # | UNIT | Price | Qty | FAVORITES | |
BBS10 Antibody (A-3) | sc-518239 | 200 µg/ml | $322.00 | |||
BBS10 Antibody (A-3): m-IgG1 BP-HRP Bundle | sc-551570 | 200 µg Ab; 20 µg BP | $361.00 | |||
BBS10 Antibody (A-3) AC | sc-518239 AC | 500 µg/ml, 25% agarose | $424.00 | |||
BBS10 Antibody (A-3) HRP | sc-518239 HRP | 200 µg/ml | $322.00 | |||
BBS10 Antibody (A-3) FITC | sc-518239 FITC | 200 µg/ml | $336.00 | |||
BBS10 Antibody (A-3) PE | sc-518239 PE | 200 µg/ml | $349.00 | |||
BBS10 Antibody (A-3) Alexa Fluor® 488 | sc-518239 AF488 | 200 µg/ml | $364.00 | |||
BBS10 Antibody (A-3) Alexa Fluor® 546 | sc-518239 AF546 | 200 µg/ml | $364.00 | |||
BBS10 Antibody (A-3) Alexa Fluor® 594 | sc-518239 AF594 | 200 µg/ml | $364.00 | |||
BBS10 Antibody (A-3) Alexa Fluor® 647 | sc-518239 AF647 | 200 µg/ml | $364.00 | |||
BBS10 Antibody (A-3) Alexa Fluor® 680 | sc-518239 AF680 | 200 µg/ml | $364.00 | |||
BBS10 Antibody (A-3) Alexa Fluor® 790 | sc-518239 AF790 | 200 µg/ml | $364.00 |