| Product Name | BBS9 Antibody (H-284) |
|---|---|
| Antibody Type | Primary Antibodies |
| Modification Notes | BBS9 (Bardet-Biedl syndrome 9), also known as B1, D1, C18 or PTHB1, is an 887 amino acid protein that localizes to both the cytoplasm and the centrosome and exists as six alternatively spliced isoforms. Expressed in a wide variety of tissues, including liver, lung, heart, brain and skeletal muscle, BBS9 functions as a component of the multi-protein BBSome complex which is required for ciliogenesis and is regulated by GDP/GTP exchange factors. Defects in the gene encoding BBS9 are associated with the pathogenesis of Bardet-Biedl syndrome type 9 (BBS9), an autosomal recessive disorder that is characterized by severe pigmentary retinopathy, early onset obesity, polydactyly, hypogenitalism, renal malformation and mental retardation. Additionally, chromosomal aberrations involving the BBS9 gene may play a role in the formation of Wilms tumor 5 (WT5). |
| Clonality | Polyclonal |
|---|---|
| Isotype | IgG |
| Host Species | Rabbit |
| Tested Applications | WBIPIFELISA |
| recommended for detection of BBS9 of mouse, rat and human origin by WB, IP, IF and ELISA non cross-reactive with other BBS family members 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 47-330 mapping near the N-terminus of BBS9 of human origin |
|---|---|
| Tissue Specificity | epitope corresponding to amino acids 47-330 mapping near the N-terminus of BBS9 of human origin |
| Entrez Gene | 27241 |
|---|
| Application Notes | recommended for detection of BBS9 of mouse, rat and human origin by WB, IP, IF and ELISA non cross-reactive with other BBS family members 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. |
