| First Author | Toursel T | Year | 2000 |
| Journal | Exp Neurol | Volume | 162 |
| Issue | 2 | Pages | 311-20 |
| PubMed ID | 10739637 | Mgi Jnum | J:115319 |
| Mgi Id | MGI:3691383 | Doi | 10.1006/exnr.1999.7349 |
| Citation | Toursel T, et al. (2000) Alterations in contractile properties and expression of myofibrillar proteins in wobbler mouse muscles. Exp Neurol 162(2):311-20 |
| abstractText | The purpose of this study was to characterize the alterations in muscle contractile (tension-pCa relationship) and biochemical (myosin heavy and light chains, troponin C content) properties in a hereditary motoneuron disease. The study was performed on wobbler mouse mutants which presented a neuronal degeneration. The time course of the disease was followed at 5 and 7 weeks in sternocleidomastoid (SCM) and soleus muscles. The wobbler disease was found to induce a shift from fast to slow myosin heavy-chain isoform expression in SCM and soleus muscles. The analysis of the myosin light-chain (MLC) composition revealed, for the SCM muscles, the appearance of the slow isoforms at 5 weeks and an increase in the regulatory MLC2 content at 7 weeks. A significant increase in the slow troponin C isoform content was found in both types of wobbler muscles at 7 weeks. The wobbler soleus and SCM muscles presented an age- and fiber-type-related atrophy, characterized by a decline in absolute maximal tension and fiber diameter. A decrease in calcium sensitivity was observed at 7 weeks for the soleus fibers and at both 5 and 7 weeks for the SCM. The results indicated fast-to-slow changes in contractile and biochemical properties of the wobbler soleus and SCM muscles, which occurred during the motoneuron degeneration process previously described in the wobbler pathology. |
