Testing Data
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Rabbit BBS8 Polyclonal Antibody | anti-BBS8 antibody
BBS8 Rabbit Polyclonal
Immunogen Affinity Purified
Immunogen Affinity Purified
Testing Data
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Testing Data
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Western Blot (WB)
(mouse testis tissue were subjected to SDS PAGE followed by western blot with MBS767056 (BBS8 antibody) at dilution of 1:300)
Western Blot (WB)
(mouse testis tissue were subjected to SDS PAGE followed by western blot with MBS767056 (BBS8 antibody) at dilution of 1:300)
NCBI and Uniprot Product Information
NCBI Description
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 involved in the formation of cilia. A mutation in this gene has also been implicated in nonsyndromic retinitis pigmentosa. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jan 2014]
Uniprot Description
TTC8: 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 TTC8 are the cause of retinitis pigmentosa type 51 (RP51). 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). 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. 5 isoforms of the human protein are produced by alternative splicing.
Protein type: Cytoskeletal
Chromosomal Location of Human Ortholog: 14q31.3
Cellular Component: centrosome; cilium; cytosol
Molecular Function: protein binding
Biological Process: cilium biogenesis; establishment of anatomical structure orientation; protein transport; sensory processing
Disease: Bardet-biedl Syndrome 8; Retinitis Pigmentosa 51
Research Articles on BBS8
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Product Notes
The BBS8 ttc8 (Catalog #AAA767056) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The BBS8 Rabbit Polyclonal reacts with Human, Mouse, Rat and may cross-react with other species as described in the data sheet. AAA Biotech's BBS8 can be used in a range of immunoassay formats including, but not limited to, ELISA (EIA), Western Blot (WB), Immunoprecipitation (IP). Researchers should empirically determine the suitability of the BBS8 ttc8 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "BBS8, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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