Application
Western blot analysis of BBS3 expression in WI 38 (A) and Hep G2 (B) whole cell lysates.| Product Name | BBS3 Antibody (H-120) |
|---|---|
| Antibody Type | Primary Antibodies |
| Antigen Alias | epitope corresponding to amino acids 67-186 mapping at the C-terminus of BBS3 of human origin |
| Modification Notes | Bardet-Biedl syndrome (BBS) is a pleiotropic genetic disorder characterized by obesity, photoreceptor degeneration, polydactyly, hypogenitalism, renal abnormalities, and developmental delay. BBS patients also have an increased risk of developing diabetes, hypertension, and congenital heart defects. BBS is a heterogeneous disorder; 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. Bardet-Biedl syndrome-3 (BBS3) results from a homozygous C-to-T transition in exon 7 of the ARL6 gene, resulting in an arg122-to-ter mutation with a premature truncation of the protein from 186 to 121 amino acids. Heterozygosity in a mutation of the BBS3 gene modifies the expression of the met390-to-arg mutation in the BBS1 gene. |
| Clonality | Polyclonal |
|---|---|
| Isotype | IgG |
| Host Species | Rabbit |
| Tested Applications | WBIPIFELISA |
| recommended for detection of BBS3 of mouse, rat and human origin by WB, IP, IF and ELISA also reactive with additional species, including equine, canine, bovine and porcine: |
|
| Species Reactivity | HumanMouseRat |
| Concentration | 1mg/ml |
| Purification | Affinity purified |
| Alternative Names | epitope corresponding to amino acids 67-186 mapping at the C-terminus of BBS3 of human origin |
|---|---|
| Tissue Specificity | epitope corresponding to amino acids 67-186 mapping at the C-terminus of BBS3 of human origin |
Application
Western blot analysis of BBS3 expression in WI 38 (A) and Hep G2 (B) whole cell lysates.| Application Notes | recommended for detection of BBS3 of mouse, rat and human origin by WB, IP, IF and ELISA also reactive with additional species, including equine, canine, bovine and porcine: |
|---|
| 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. |
