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Key Specifications Table
| Species Reactivity | Key Applications | Host | Format | Antibody Type |
|---|---|---|---|---|
| H, Mk, PO, B, M | WB, IHC | M | Purified | Monoclonal Antibody |
| Description | |
|---|---|
| Catalogue Number | MAB5466 |
| Brand Family | Chemicon® |
| Trade Name |
|
| Description | Anti-Bestrophin Antibody |
| Product Information | |
|---|---|
| Format | Purified |
| Presentation | Purified immunoglobulin. Liquid in PBS containing 0.08% sodium azide. |
| Quality Level | MQ100 |
| Applications | |
|---|---|
| Application | Detect Bestrophin using this Anti-Bestrophin Antibody validated for use in WB, IH. |
| Key Applications |
|
| Application Notes | Western blot: 5 μg/mL of this antibody detected Bestrophin in bovine retina cell lysate. Immunohistochemistry (Paraffin) Analysis: A 1:20 dilution from a representative lot detected Bestrophin in mouse retina tissue sections. Optimal working dilutions must be determined by end user. |
| Biological Information | |
|---|---|
| Immunogen | Recombinant human bestrophin protein. |
| Concentration | Please refer to the Certificate of Analysis for the lot-specific concentration. |
| Host | Mouse |
| Specificity | Reacts with bestrophin. |
| Isotype | IgG |
| Species Reactivity |
|
| Antibody Type | Monoclonal Antibody |
| Entrez Gene Number |
|
| Entrez Gene Summary | BEST1 belongs to the bestrophin family of anion channels, which includes BEST2 (MIM 607335), BEST3 (MIM 607337), and BEST4 (MIM 607336). Bestrophins are transmembrane (TM) proteins that share a homology region containing a high content of aromatic residues, including an invariant arg-phe-pro (RFP) motif. The bestrophin genes share a conserved gene structure, with almost identical sizes of the 8 RFP-TM domain-encoding exons and highly conserved exon-intron boundaries. Each of the 4 bestrophin genes has a unique 3-prime end of variable length (Stohr et Al., 2002 [PubMed 12032738]; Tsunenari et Al., 2003 [PubMed 12907679]).[supplied by OMIM] |
| Gene Symbol |
|
| UniProt Number |
|
| UniProt Summary | FUNCTION: SwissProt: O76090 # Forms calcium-sensitive chloride channels. May conduct other physiologically significant anions such as bicarbonate. SIZE: 585 amino acids; 67684 Da SUBUNIT: Tetramer or pentamers. May interact with PPP2CB and PPP2R1B (By similarity). SUBCELLULAR LOCATION: Cell membrane; Multi-pass membrane protein. TISSUE SPECIFICITY: Predominantly expressed in the basolateral membrane of the retinal pigment epithelium. PTM: Phosphorylated by PP2A (By similarity). DISEASE: SwissProt: O76090 # Defects in BEST1 are the cause of vitelliform macular dystrophy type 2 (VMD2) [MIM:153700]; also known as Best macular dystrophy (BMD). VMD2 is an autosomal dominant form of macular degeneration that usually begins in childhood or adolescence. VMD2 is characterized by typical egg-yolk macular lesions due to abnormal accumulation of lipofuscin within and beneath the retinal pigment epithelium cells. Progression of the disease leads to destruction of the retinal pigment epithelium and vision loss. & Defects in BEST1 are a cause of adult-onset vitelliform macular dystrophy (AVMD) [MIM:608161]. AVMD is a rare autosomal dominant disorder with incomplete penetrance and highly variable expression. Patients usually become symptomatic in the fourth or fifth decade of life with a protracted disease of decreased visual acuity. & Defects in BEST1 may be a cause of bulls eye maculopathy [MIM:153870]. SIMILARITY: SwissProt: O76090 ## Belongs to the bestrophin family. |
| Product Usage Statements | |
|---|---|
| Usage Statement |
|
| Storage and Shipping Information | |
|---|---|
| Storage Conditions | Maintain at -20°C in undiluted aliquots for up to 6 months after date of receipt. Avoid repeated freeze/thaw cycles. |
| Packaging Information | |
|---|---|
| Material Size | 100 µg |