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LMOD3 抗体 (AA 45-558)

LMOD3 适用: 人, 小鼠, 大鼠 WB, ELISA 宿主: 兔 Polyclonal unconjugated
产品编号 ABIN7601779
发货至: 中国
  • 抗原 See all LMOD3 products
    LMOD3 (Leiomodin 3 (LMOD3))
    抗原表位
    • 1
    • 1
    AA 45-558
    适用
    • 3
    • 1
    • 1
    • 1
    • 1
    • 1
    人, 小鼠, 大鼠
    宿主
    • 3
    克隆类型
    • 3
    多克隆
    标记
    • 3
    This LMOD3 antibody is un-conjugated
    应用范围
    • 3
    • 2
    • 2
    • 1
    Western Blotting (WB), ELISA
    原理
    Anti-LMOD3 Antibody Picoband®
    交叉反应 (详细)
    No cross-reactivity with other proteins
    产品特性
    Anti-LMOD3 Antibody Picoband® (ABIN7601779). Tested in WB, ELISA applications. This antibody reacts with Human, Mouse, Rat. The brand Picoband indicates this is a premium antibody that guarantees superior quality, high affinity, and strong signals with minimal background in Western blot applications. Only our best-performing antibodies are designated as Picoband, ensuring unmatched performance.
    纯化方法
    Immunogen affinity purified.
    免疫原
    E.coli-derived human LMOD3 recombinant protein (Position: D45-E558). Human LMOD3 shares 72.2% amino acid (aa) sequence identity with mouse LMOD3.
    亚型
    IgG
  • 应用备注
    Western blot, 0.25-0.5 μg/mL, Mouse, Rat
    ELISA, 0.1-0.5 μg/mL, -
    1. Abbott, M., Jain, M., Pferdehirt, R., Chen, Y., Tran, A., Duz, M. B., Seven, M., Gibs, R. A., Muzny, D., Lee, B., Marom, R., Burrage, L. C. Neonatal fractures as a presenting feature of LMOD3-associated congenital myopathy. Am. J. Med. Genet. 173A: 2789-2794, 2017. 2. Hartz, P. A. Personal Communication. Baltimore, Md. 12/1/2014. 3. Schatz, U. A, Weiss, S., Wenninger, S., Schoser, B., Muss, W. H., Bittner, R. E., Schmidt, W. M., Schossig, A. S., Rudnik-Schoneborn, S., Baumann, M. Evidence of mild founder LMOD3 mutations causing nemaline myopathy 10 in Germany and Austria. Neurology 91: e1690-e1694, 2018.
    限制
    仅限研究用
  • 状态
    Lyophilized
    溶解方式
    Adding 0.2 mL of distilled water will yield a concentration of 500 μg/mL.
    浓度
    500 μg/mL
    缓冲液
    Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.
    储存条件
    4 °C,-20 °C
    储存方法
    At -20°C for one year from date of receipt. After reconstitution, at 4°C for one month.
    It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freezing and thawing.
  • 抗原
    LMOD3 (Leiomodin 3 (LMOD3))
    别名
    LMOD3 (LMOD3 产品)
    背景

    Synonyms: LMOD3, Leiomodin-3, Leiomodin, fetal form

    Background: Leiomodin-3 is a protein that in humans is encoded by the LMOD3 gene. The protein encoded by this gene is a member of the leiomodin family of proteins. This protein contains three actin-binding domains, a tropomyosin domain, a leucine-rich repeat domain, and a Wiskott-Aldrich syndrome protein homology 2 domain (WH2). Localization of this protein to the pointed ends of thin filaments has been observed, and there is evidence that this protein acts as a catalyst of actin nucleation, and is important to the organization of sarcomeric thin filaments in skeletal muscles. Mutations in this gene have been associated as one cause of Nemaline myopathy, as other genes have also been linked to this disorder. Nemaline myopathy is a disorder characterized by nonprogressive generalized muscle weakness and protein inclusions (nemaline bodies) in skeletal myofibers. Patients with mutations in this gene often present with a severe congenital form of the disorder.

    分子量
    70 kDa
    基因ID
    56203
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