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LMOD3 antibody (AA 45-558)

LMOD3 Reactivity: Human, Mouse, Rat WB, ELISA Host: Rabbit Polyclonal unconjugated
Catalog No. ABIN7601779
  • Target See all LMOD3 products
    LMOD3 (Leiomodin 3 (LMOD3))
    Binding Specificity
    • 1
    • 1
    AA 45-558
    Reactivity
    • 3
    • 1
    • 1
    • 1
    • 1
    • 1
    Human, Mouse, Rat
    Host
    • 3
    Rabbit
    Clonality
    • 3
    Polyclonal
    Conjugate
    • 3
    This LMOD3 antibody is un-conjugated
    Application
    • 3
    • 2
    • 2
    • 1
    Western Blotting (WB), ELISA
    Purpose
    Anti-LMOD3 Antibody Picoband®
    Cross-Reactivity (Details)
    No cross-reactivity with other proteins
    Characteristics
    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.
    Purification
    Immunogen affinity purified.
    Immunogen
    E.coli-derived human LMOD3 recombinant protein (Position: D45-E558). Human LMOD3 shares 72.2% amino acid (aa) sequence identity with mouse LMOD3.
    Isotype
    IgG
  • Application Notes
    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.
    Restrictions
    For Research Use only
  • Format
    Lyophilized
    Reconstitution
    Adding 0.2 mL of distilled water will yield a concentration of 500 μg/mL.
    Concentration
    500 μg/mL
    Buffer
    Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.
    Storage
    4 °C,-20 °C
    Storage Comment
    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.
  • Target
    LMOD3 (Leiomodin 3 (LMOD3))
    Alternative Name
    LMOD3 (LMOD3 Products)
    Background

    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.

    Molecular Weight
    70 kDa
    Gene ID
    56203
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