Application
Immunoperoxidase staining of formalin fixed, paraffin-embedded human pancreas tissue showing cytoplasmic staining of exocrine glandular cells and Islets of Langerhans.Application
Western blot analysis of BBS4 expression in mouse heart (A), rat heart (B) and rat kidney (C) tissue extracts.| Product Name | BBS4 Antibody (H-304) |
|---|---|
| Antibody Type | Primary Antibodies |
| Antigen Alias | epitope corresponding to amino acids 33-336 mapping near the N-terminus of BBS4 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. Other associated clinical findings in BBS patients include 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. BBS4 is expressed in the olfactory epithelium and localizes to the centriolar satellites of centrosomes and basal bodies of primary cilia. BBS4 regulates the p150 subunit of the dynein transport machinery (DCTN1) to attract pericentriolar material-1 protein (PCM1) and its associated components to the satellites. Loss of BBS4 is correlated with obesity caused by abnormal lipid profiles, liver dysfunction, elevated insulin, and abnormal leptin levels. |
| Clonality | Polyclonal |
|---|---|
| Isotype | IgG |
| Host Species | Rabbit |
| Tested Applications | WBIPIFIHCELISA |
| recommended for detection of BBS4 of mouse, rat and human origin by WB, IP, IF, IHC(P) 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 33-336 mapping near the N-terminus of BBS4 of human origin |
|---|---|
| Tissue Specificity | epitope corresponding to amino acids 33-336 mapping near the N-terminus of BBS4 of human origin |
| Entrez Gene | 585 |
|---|
Application
Immunoperoxidase staining of formalin fixed, paraffin-embedded human pancreas tissue showing cytoplasmic staining of exocrine glandular cells and Islets of Langerhans.
Application
Western blot analysis of BBS4 expression in mouse heart (A), rat heart (B) and rat kidney (C) tissue extracts.| Application Notes | recommended for detection of BBS4 of mouse, rat and human origin by WB, IP, IF, IHC(P) 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. |
