| Product Name | BBS8 Antibody (H-300) |
|---|---|
| Antibody Type | Primary Antibodies |
| Antigen Alias | epitope corresponding to amino acids 232-531 mapping at the C-terminus of BBS8 of human origin |
| Modification Notes | 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. |
| Clonality | Polyclonal |
|---|---|
| Isotype | IgG |
| Host Species | Rabbit |
| Tested Applications | WBIPIFELISA |
| recommended for detection of BBS8 of mouse, rat and human origin by WB, IP, IF and ELISA also reactive with additional species, including equine, canine, bovine, porcine and avian: |
|
| Species Reactivity | HumanMouseRat |
| Concentration | 1mg/ml |
| Purification | Affinity purified |
| Alternative Names | epitope corresponding to amino acids 232-531 mapping at the C-terminus of BBS8 of human origin |
|---|---|
| Tissue Specificity | epitope corresponding to amino acids 232-531 mapping at the C-terminus of BBS8 of human origin |
| Entrez Gene | 123016 |
|---|
| Application Notes | recommended for detection of BBS8 of mouse, rat and human origin by WB, IP, IF and ELISA also reactive with additional species, including equine, canine, bovine, porcine and avian: |
|---|
| Form | Liquid |
|---|---|
| Storage Instructions | For short-term storage, store at 4° C. For long-term storage, aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles. |
| Storage Buffer | phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol. |
