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  • merck millipore,默克密理博,MAB5466,Anti-Bestrophin Antibody

  • merck millipore,默克密理博,MAB5466,Anti-Bestrophin Antibody

    产品名称：Anti-Bestrophin Antibody
    产品型号：MAB5466
    Detect Bestrophin using this Anti-Bestrophin Antibody validated for use in WB, IH.

    merck millipore,默克密理博,MAB5466,Anti-Bestrophin Antibody

  • 产品介绍
  • merck millipore,默克密理博,MAB5466,Anti-Bestrophin Antibody

    重要规格表

    品种反应性 主要应用 宿主 格式 抗体类型
    Po, H, MkIHC, WBMPurifiedMonoclonal Antibody
    描述
    产品目录编号 MAB5466
    品牌系列 Chemicon®
    商名
    • Chemicon
    描述 Anti-Bestrophin Antibody
    产品信息
    格式 Purified
    演示 Purified immunoglobulin. Liquid in PBS containing 0.08% sodium azide.
    应用
    应用 Detect Bestrophin using this Anti-Bestrophin Antibody validated for use in WB, IH.
    主要应用
    • Immunohistochemistry
    • Western Blotting
    应用说明 Western blot: 5-10 μg/mL. The antibody recognizes the ~68 kDa bestrophin protein.

    Immunohistochemistry on paraformaldehyde fixed tissue sections.

    Optimal working dilutions must be determined by end user.
    生物信息
    免疫原品种 Recombinant human bestrophin protein.
    浓缩 Please refer to the Certificate of Analysis for the lot-specific concentration.
    宿主 Mouse
    特异性 Reacts with bestrophin.
    同种型 IgG
    品种反应性 PigHumanMonkey
    抗体类型 Monoclonal Antibody
    Entrez基因编号
    • NM_004183.2
    Entrez基因汇总 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]
    基因符号
    • BMD
    • BEST1
    • BEST
    • VMD2
    • bestrophin
    • Bestrophin-1
    • TU15B
    UniProt编号
    • O76090
    UniProt汇总 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 bull's eye maculopathy [MIM:153870].
    SIMILARITY: SwissProt: O76090 ## Belongs to the bestrophin family.
    产品使用声明
    使用声明
    • Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
    储存和货运信息
    存储条件 Maintain at -20°C in undiluted aliquots for up to 6 months after date of receipt. Avoid repeated freeze/thaw cycles.
    包装信息
    数量 100 µg

    merck millipore,默克密理博,MAB5466,Anti-Bestrophin Antibody

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