Technical Data
B1080-25B
Bestrophin (BEST, Best Macular Dystrophy, BMD, Vitelliform Macular Dystrophy, VMD2)
Description:
Best macular dystrophy (BMD) or vitelliform macular dystrophy (VMD2), is an autosomal form of macular degeneration, characterized by a depressed light peak in the electrooculogram (EOG). It is inherited and has an early onset. Bestrophin is a 68kD basolateral plasma membrane protein encoded by the VMD2 gene. Bestrophin's function is still unknown, but data suggests that it is a chloride channel that plays a role in generating the altered EOG in Best disease patients. In addition, Bestrophin is a useful biochemical and histological marker of RPE (retinal pigment epithelial cells) cells.

Positive Control: Pig RPE (Retinal Pigment Epithelium) whole cell extract

Applications:
Suitable for use in Immunofluorescence and Western Blot. Other applications not tested.

Recommended Dilution:
Optimal dilutions to be determined by the researcher.

Storage and Stability:
May be stored at 4°C for short-term only. For long-term storage and to avoid repeated freezing and thawing, aliquot and store at -20°C. Aliquots are stable for at least 12 months at -20°C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.
TypeIsotypeCloneGrade
MabIgG1,k8J321
SizeStorageShippingSourceHost
100ul-20°CBlue IceMouse
Concentration:
Not determined
Immunogen:
Partial recombinant protein [KDHMDPYWALENRDEAHS].
Purity:
Ascites
Form
Supplied as a liquid (ascites) containing 0.01% sodium azide
Specificity:
Species Crossreactivity: Recognizes human, porcine, and monkey. This antibody does not work in rat. Other species have not been tested.
Intended for research use only. Not for use in human, therapeutic, or diagnostic applications.
2. Petrushkin, K., et al. Identification of the gene responsible for Best macular dystrophy. Nature Genetics. 19: 241-247 (1998)
3. Caldwell, G.M., et al. Bestrophin gene mutations in patients with Best Vitelliform Macular Dystrophy. Genomics. 58: 98-100 (1999)
4. Marmorstein, L.Y., et al. Bestrophin physically and functionally interacts with protein phosphatase 2A. JBC. 277(34): 30591-30597 (2002)