| First Author | Lucas-Heron B | Year | 1997 |
| Journal | Biochem Biophys Res Commun | Volume | 232 |
| Issue | 2 | Pages | 559-61 |
| PubMed ID | 9125222 | Mgi Jnum | J:38962 |
| Mgi Id | MGI:86348 | Doi | 10.1006/bbrc.1997.6299 |
| Citation | Lucas-Heron B (1997) A drug inhibits the mitochondrial protease inducing calmitine deficiency in skeletal muscle of patients with Duchenne's muscular dystrophy and dy/dy dystrophic mice. Biochem Biophys Res Commun 232(2):559-61 |
| abstractText | This study demonstrates that the cause of calmitine deficiency in dy/dy dystrophic mice and patients with Duchenne's muscular dystrophy (DMD) is the same; i.e., the absence of an inhibitor of calmitine-specific mitochondrial protease. This inhibitor, which is present in control mice and control subjects, prevented degradation of the protein. It is also shown that a drug (IP96) was capable in vitro of inhibiting calmitine-specific mitochondrial protease from muscle of DMD patients and dy/dy mice. This drug was also active in vivo in an experimental model of myopathy created in the normal mouse by a single injection of chlorpromazine, a myotoxic drug, which induced temporary calmitine degradation. Thus, it seems quite likely that IP96 prevents calmitine degradation by inhibiting the specific protease. |
