This gene is a member of the Bardet-Biedl syndrome (BBS) gene family.
Bardet-Biedl syndrome is an autosomal recessive disorder characterized by progressive retinal degeneration, obesity, polydactyly, renal malformation and mental retardation.
The proteins encoded by BBS gene family members are structurally diverse and the similar phenotypes exhibited by mutations in BBS gene family members is likely due to their shared roles in cilia formation and function.
Many BBS proteins localize to the basal bodies, ciliary axonemes, and pericentriolar regions of cells.
BBS proteins may also be involved in intracellular trafficking via microtubule-related transport.
The protein encoded by this gene is likely not a ciliary protein but rather has distant sequence homology to type II chaperonins.
As a molecular chaperone, this protein may affect the folding or stability of other ciliary or basal body proteins.
Inhibition of this protein's expression impairs ciliogenesis in preadipocytes.
Mutations in this gene cause Bardet-Biedl syndrome type 10.
Applications:Suitable for use in Western Blot.
Other applications not tested.
Recommended Dilution:Optimal dilutions to be determined by the researcher.
Storage and Stability:May be stored at 4°C for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20°C.
Aliquots are stable for 12 months after receipt.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size:100ul
Host:rabbit
Source Antibody:human
Grade:Affinity Purified
Purity:Purified by affinity chromatography.
Form:Supplied as a liquid in PBS, 2% sucrose, 0.09% sodium azide.
Specificity:Recognizes human BBS10.
Isotype:IgG
Calc Applications Abbrev:WB
Calc Crossreactivity:Hu
Immunogen:Synthetic peptide corresponding to the C-terminal region of human BBS10