本公司提供科研
Bestrophin卵黄状黄斑病蛋白抗体，抗体质量可靠，订购
Bestrophin卵黄状黄斑病蛋白抗体请联系在线客服或者销售人员。
抗体参数如下>>>>
中文名称：
卵黄状黄斑病蛋白抗体英文名称：
Anti-Bestrophin货号：bs-11040R
抗体来源：兔
克隆类型：多克隆
蛋白分子量：predicted molecular weight: 64kDa
纯化方法：affinity purified by Protein A
交叉反应：hu, mo, rat, hrs, pig, dog, cow, Rb
测试应用：ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500
（石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
产品背景介绍：Best vitelliform macular dystrophy, known as Best disease, is an early-onset autosomal dominant condition in which accumulation of lipofuscin-like material within and beneath the RPE leads to progressive loss of central vision. Best disease is frequently a reflection of mutations in the Bestrophin gene, which encodes a protein containing four putative transmembrane domains and localizes to the basolateral plasma membrane of RPE cells. Human Bestrophin forms oligomeric chloride channels that are sensitive to intracellular calcium. Missense mutations at the Bestrophin locus reduces or abolishes Bestrophin protein mediated membrane current. Bestrophin Bestrophin 2,Bestrophin 3, and Bestrophin 4 are transmembrane proteins that contain a high percentage of aromatic residues, a conserved RFP (Arg-Phe-Pro) motif and they function as anion channels.Function : Forms calcium-sensitive chloride channels. Highly permeable to bicarbonate.Subunit : Tetramer or pentamers. May interact with PPP2CB and PPP2R1B.Subcellular Location : Cell membrane. Basolateral cell membrane.Tissue Specificity : Predominantly expressed in the basolateral membrane of the retinal pigment epithelium.Post-translational modifications : Phosphorylated by PP2A.DISEASE : Defects in BEST1 are the cause of vitelliform macular dystrophy type 2 (VMD2) ; 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 the cause of retinitis pigmentosa type 50 (RP50) . A retinal dystrophy belonging to the group of pigmentary retinopathies. RP is characterized by retinal pigment deposits visible on fundus examination and primary loss of rod photoreceptor cells followed by secondary loss of cone photoreceptors. Patients typically have night vision blindness and loss of midperipheral visual field. As their condition progresses, they lose their far peripheral visual field and eventually central vision as well.Similarity : Belongs to the bestrophin family.
