Myotilin Antibody (E-10): sc-393957
- Myotilin Antibody (E-10) is a mouse monoclonal IgG1 κ Myotilin antibody provided at 200 µg/ml
- specific for an epitope mapping between amino acids 321-420 within an internal region of Myotilin of human origin
- Myotilin Antibody (E-10) is recommended for detection of Myotilin of mouse, rat and human origin by WB, IP, IF and ELISA; also reactive with additional species, including canine
- Anti-Myotilin Antibody (E-10) is available conjugated to agarose for IP; HRP for WB, IHC(P) and ELISA; and to either phycoerythrin or FITC for IF, IHC(P) and FCM
- also available conjugated to Alexa Fluor® 488, Alexa Fluor® 546, Alexa Fluor® 594 or Alexa Fluor® 647 for WB (RGB), IF, IHC(P) and FCM, and for use with RGB fluorescent imaging systems, such as iBright™ FL1000, FluorChem™, Typhoon, Azure and other comparable systems
- also available conjugated to Alexa Fluor® 680 or Alexa Fluor® 790 for WB (NIR), IF and FCM; for use with Near-Infrared (NIR) detection systems, such as LI-COR®Odyssey®, iBright™ FL1000, FluorChem™, Typhoon, Azure and other comparable systems
- m-IgG Fc BP-HRP and m-IgG1 BP-HRP are the preferred secondary detection reagents for Myotilin Antibody (E-10) for WB applications. These reagents are now offered in bundles with Myotilin Antibody (E-10) (see ordering information below).
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Myotilin Antibody (E-10) is a mouse monoclonal IgG1 kappa light chain antibody that detects Myotilin protein of mouse, rat, and human origin by western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), and enzyme-linked immunosorbent assay (ELISA). Anti-Myotilin antibody (E-10) is available in both non-conjugated and various conjugated forms, including agarose, horseradish peroxidase (HRP), phycoerythrin (PE), fluorescein isothiocyanate (FITC), and multiple Alexa Fluor® conjugates. Myotilin is a crucial sarcomeric protein encoded by a gene located on human chromosome 5q31, playing a significant role in muscle structure and function. Myotilin is primarily localized in the Z-line of myofibrils, where Myotilin binds to α-actinin, facilitating the structural integrity of the sarcomere. This interaction is vital for maintaining muscle contraction and overall muscle health. Notably, defects in the myotilin gene are associated with autosomal dominant limb-girdle muscular dystrophy (LGMD), a condition characterized by progressive weakness in the hip and shoulder girdles, along with distinctive speech patterns. Affected individuals exhibit muscle degeneration, variations in fiber size, fiber splitting, centrally located myonuclei, and an increased number of autophagic vesicles, underscoring the importance of Myotilin in muscle integrity and function.
Alexa Fluor® is a trademark of Molecular Probes Inc., OR., USA
LI-COR® and Odyssey® are registered trademarks of LI-COR Biosciences
Myotilin Antibody (E-10) References:
- Myotilin, a novel sarcomeric protein with two Ig-like domains, is encoded by a candidate gene for limb-girdle muscular dystrophy. | Salmikangas, P., et al. 1999. Hum Mol Genet. 8: 1329-36. PMID: 10369880
- Myotilin is mutated in limb girdle muscular dystrophy 1A. | Hauser, MA., et al. 2000. Hum Mol Genet. 9: 2141-7. PMID: 10958653
- Indications for a novel muscular dystrophy pathway. gamma-filamin, the muscle-specific filamin isoform, interacts with myotilin. | van der Ven, PF., et al. 2000. J Cell Biol. 151: 235-48. PMID: 11038172
- Evidence for locus heterogeneity in autosomal dominant limb-girdle muscular dystrophy. | Speer, MC., et al. 1995. Am J Hum Genet. 57: 1371-6. PMID: 8533766
- Mutations in the caveolin-3 gene cause autosomal dominant limb-girdle muscular dystrophy. | Minetti, C., et al. 1998. Nat Genet. 18: 365-8. PMID: 9537420
Ordering Information
| Product Name | Catalog # | UNIT | Price | Qty | FAVORITES | |
Myotilin Antibody (E-10) | sc-393957 | 200 µg/ml | $322.00 | |||
Myotilin Antibody (E-10): m-IgG Fc BP-HRP Bundle | sc-527724 | 200 µg Ab; 10 µg BP | $361.00 | |||
Myotilin Antibody (E-10): m-IgG1 BP-HRP Bundle | sc-533097 | 200 µg Ab; 20 µg BP | $361.00 | |||
Myotilin Antibody (E-10) AC | sc-393957 AC | 500 µg/ml, 25% agarose | $424.00 | |||
Myotilin Antibody (E-10) HRP | sc-393957 HRP | 200 µg/ml | $322.00 | |||
Myotilin Antibody (E-10) FITC | sc-393957 FITC | 200 µg/ml | $336.00 | |||
Myotilin Antibody (E-10) PE | sc-393957 PE | 200 µg/ml | $349.00 | |||
Myotilin Antibody (E-10) Alexa Fluor® 488 | sc-393957 AF488 | 200 µg/ml | $364.00 | |||
Myotilin Antibody (E-10) Alexa Fluor® 546 | sc-393957 AF546 | 200 µg/ml | $364.00 | |||
Myotilin Antibody (E-10) Alexa Fluor® 594 | sc-393957 AF594 | 200 µg/ml | $364.00 | |||
Myotilin Antibody (E-10) Alexa Fluor® 647 | sc-393957 AF647 | 200 µg/ml | $364.00 | |||
Myotilin Antibody (E-10) Alexa Fluor® 680 | sc-393957 AF680 | 200 µg/ml | $364.00 | |||
Myotilin Antibody (E-10) Alexa Fluor® 790 | sc-393957 AF790 | 200 µg/ml | $364.00 | |||
Myotilin (E-10) Neutralizing Peptide | sc-393957 P | 100 µg/0.5 ml | $69.00 |