Product Name	Bestrophin
Chinese Name	卵黄状黄斑病蛋白抗体
Alias	BEST 1; BEST1; BEST-1; BEST; Best macular dystrophy; BEST1; BEST1_HUMAN; Bestrophin 1; Bestrophin-1; Bestrophin1; BMD; mBest1; TU15B; Vitelliform macular dystrophy 2; Vitelliform macular dystrophy; Vitelliform macular dystrophy protein 2; VMD 2; VMD2.
literatures	Specific References  (2)     |     SL11040R has been referenced in 2 publications. [IF=17.694] Liu, Haitao. et al. Reducing Akt2 in retinal pigment epithelial cells causes a compensatory increase in Akt1 and attenuates diabetic retinopathy. NAT COMMUN. 2022 Oct;13(1):1-19  IHC ;  Mouse.   PubMed:36229454 [IF=4.183] Amirkavei M et al. Induction of Heat Shock Protein 70 in Mouse RPE as an In Vivo Model of Transpupillary Thermal Stimulation. Int J Mol Sci. 2020 Mar 17;21(6). pii: E2063.  IF ;  mouse.   PubMed:32192227
Research Area	Neurobiology  Cell adhesion molecule
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species	(predicted: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, )
Applications	ELISA=1:5000-10000  not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Theoretical molecular weight	64kDa
Cellular localization	The cell membrane
Form	Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human Bestrophin: 251-350/585
Lsotype	IgG
Purification	affinity purified by Protein A
Buffer Solution	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage	Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Attention	This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed	PubMed
Product Detail	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. SWISS: O76090 Gene ID: 7439 Database links: Entrez Gene: 7439 Human Entrez Gene: 24115 Mouse Entrez Gene: 293735 Rat Omim: 607854 Human SwissProt: O76090 Human SwissProt: O88870 Mouse Unigene: 524910 Human Unigene: 712676 Human Unigene: 31577 Mouse

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