LMOD3 Antikörper (AA 45-558)

Produktnummer ABIN7601779

Produktdetails anti-LMOD3 Antikörper

Target: LMOD3 (Leiomodin 3 (LMOD3))

Bindungsspezifität: AA 45-558

Reaktivität: Human, Maus, Ratte

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser LMOD3 Antikörper ist unkonjugiert

Applikation: Western Blotting (WB), ELISA

Verwendungszweck: Anti-LMOD3 Antibody Picoband®

Kreuzreaktivität (Details): No cross-reactivity with other proteins

Produktmerkmale: 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.

Aufreinigung: 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.

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: 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.

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Lyophilized

Rekonstitution: Adding 0.2 mL of distilled water will yield a concentration of 500 μg/mL.

Konzentration: 500 μg/mL

Buffer: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.

Lagerung: 4 °C,-20 °C

Informationen zur Lagerung: 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.

Details zu LMOD3

Target: LMOD3 (Leiomodin 3 (LMOD3))

Andere Bezeichnung: LMOD3 (LMOD3 Produkte)

Hintergrund:

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.

Molekulargewicht: 70 kDa

Gen-ID: 56203