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- mouse monoclonal IgG2a, 100 µg/ml
- raised against recombinant BBS8 of human origin
- recommended for detection of BBS8 of mouse, rat and human origin by WB, IP and ELISA
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BBS8 Background Information Bardet-Biedl syndrome (BBS) is a heterogeneous pleiotropic genetic disorder characterized by obesity, photoreceptor degeneration, polydactyly, hypogenitalism, renal abnormalities, developmental delay, diabetes, hypertension and congenital heart defects. BBS genes map to eight genetic loci and encode eight proteins, BBS1-BBS8. Five BBS genes encode basal body or cilia proteins, suggesting that BBS is a ciliary dysfunction disorder. Mutations in BBS8, also designated tetratricopeptide repeat protein (TTC8), probably account for only a minority (2%) of BBS families, underlining the difficulty of genotyping heterogeneous conditions. The identification of BBS8 provides the key to the pathogenesis of the condition as a primary ciliary disorder. |
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BBS8 (Q30)
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BBS8 (Q30): sc-100682. Western blot analysis of BBS8 expression in NIH/3T3 whole cell lysate.
BBS8 (Q30): sc-100682. Western blot analysis of BBS8 expression in non-transfected: sc-117752 (A) and mouse BBS8 transfected: sc-118690 (B) 293T whole cell lysates.
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