| Product Name | Mouse anti-Myotilin, clone RSO34 (monoclonal) |
|---|---|
| Description | Mouse anti-Myotilin, clone RSO34 (monoclonal) |
| Host | Mouse |
| Clonality | Monoclonal |
| Clone | RSO34 |
| Immunogen | Prokaryotic recombinant protein corresponding to a C-terminal region of 266 amino acids of the human myotilin molecule. |
| Isotype | IgG1, kappa |
| Reactivity | Human |
| Applications | IHC-Fr, IHC-P |
| Working Dilutions | IHC-P (1:40-1:80),IHC-fr (1:20) |
| Diluent Buffer | Lyophilized |
| Concentration | n/a |
| Storage | 2-8°C |
| Notes | Reconstitute with 1 mL or 0.1 mL of sterile distilled water as indicated on vial label. The myotilin gene on chromosome 5q31 encodes a 498 amino acid polypeptide with a molecular weight of 57kD. Myotilin is a structural protein of sarcomeric Z discs and sarcolemma in human skeletal and cardiac muscle. It is homologous to palladin and titin in the two C-terminal lg-domains and also to palladin in its unique serine-rich N-terminal region. Myotilin interacts with alpha-actinin, actin and gamma-filamin. Mutations in the myotilin gene are associated with limb-girdle muscular dystrophy 1 A (LGMD1A) and one form of Myofibrillar Myopathy. It is highly conserved between human and mouse with its expression being more widespread in the embryo than in the adult. Expression of myotilin has been reported in adult skeletal and cardiac muscle with variable expression reported in the peripheral nervous system, lung, liver and kidney. NCL-MYOTILIN will be of use in studies to determine the expression of myotilin in normal and pathological tissues. |
| References | Mologni L et al. Mechanisms of Development. 103: 121–125 (2001). Mykkänen OM et al. Mol. Biol. Cell. 12 (10): 3060–3073 (2001). Hauser MA et al. Human Molecular Genetics. 9 (14): 2141–2147 (2000). van der Ven PFM et al. Journal of Cell Biology. 151 (2): 235–247 (2000). Salmikangas P et al. Human Molecular Genetics. 8 (7): 1329–1336 (1999 |
| Supplier | MONOSAN |
All Research Products are sold for laboratory RESEARCH USE ONLY and ARE NOT TO BE USED FOR HUMAN OR ANIMAL THERAPEUTIC OR DIAGNOSTIC APPLICATIONS. The information presented is believed to be accurate; however, said information and products are offered without warranty or guarantee since the ultimate conditions of use and the variability of the materials treated are beyond our control. Nothing disclosed herein is to be construed as a recommendation to use our products in violation of any patents. ARP American Research Products, Inc. does not submit its products for regulatory review by any government body or other organization, and we do not validate them for clinical, therapeutic or diagnostic use, or for safety and effectiveness. You are solely responsible for making sure that the way you use the products complies with applicable laws, regulations and governmental policies and for obtaining all necessary approvals, intellectual property rights, licenses and permissions that you may need related to your use. Under no circumstances shall ARP American Research Products, Inc. be liable for damages, whether consequential, compensatory, incidental or special, strict liability or negligence, breach of warranty or any other theory arising out of the use of the products available from ARP American Research Products, Inc. Nothing contained herein warrants that the use of the products will not infringe on the claims of any patents covering the product itself or the use thereof in combination with other products or in the operation of any process. ARP American Research Products, Inc. disclaims any and all representations or warranties of any kind whatsoever, express or implied, including without limitation any implied warranties of merchantability or fitness for a particular purpose, of non-infringement, or regarding results obtained through the use of any product, whether arising from a statute or otherwise in law or from a course of performance, dealing or usage of trade.
