|Other Names||Bestrophin-3, Vitelliform macular dystrophy 2-like protein 3, BEST3, VMD2L3|
|Format||Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|Function||Forms calcium-sensitive chloride channels. Permeable to bicarbonate.|
|Cellular Location||Cell membrane; Multi-pass membrane protein.|
|Tissue Location||Present in skeletal muscle and weakly in brain, spinal cord, bone marrow and retina|
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BEST3 belongs to the bestrophin family of anion channels,which includes BEST1 (MIM 607854), the gene mutant in vitelliformmacular dystrophy (VMD; MIM 153700), and 2 other BEST1-like genes,BEST2 (MIM 607335) and BEST4 (MIM 607336). Bestrophins aretransmembrane (TM) proteins that share a homology region containinga high content of aromatic residues, including an invariantarg-phe-pro (RFP) motif. The bestrophin genes share a conservedgene structure, with almost identical sizes of the 8 RFP-TMdomain-encoding exons and highly conserved exon-intron boundaries.Each of the 4 bestrophin genes has a unique 3-prime end of variablelength (Stohr et al., 2002 [PubMed 12032738]; Tsunenari et al.,2003 [PubMed 12907679]).
Marsey, L.L., et al. J. Physiol. (Lond.) 587 (PT 10), 2211-2224 (2009) :Qu, Z.Q., et al. J. Biol. Chem. 282(24):17460-17467(2007)Tsunenari, T., et al. J. Biol. Chem. 278(42):41114-41125(2003)Stohr, H., et al. Eur. J. Hum. Genet. 10(4):281-284(2002)Marquardt, A., et al. Hum. Mol. Genet. 7(9):1517-1525(1998)
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