| First Author | Weiss MD | Year | 2001 |
| Journal | Muscle Nerve | Volume | 24 |
| Issue | 10 | Pages | 1380-7 |
| PubMed ID | 11562920 | Mgi Jnum | J:116346 |
| Mgi Id | MGI:3694052 | Doi | 10.1002/mus.1159 |
| Citation | Weiss MD, et al. (2001) Nerve conduction abnormalities in aging mice deficient for myelin-associated glycoprotein. Muscle Nerve 24(10):1380-7 |
| abstractText | Ultrastructural, biochemical, and electrophysiological analyses were done on 12-14-month-old mice deficient for myelin-associated glycoprotein (MAG) to further characterize the neuropathy that develops as they age. Electron microscopy demonstrated normal myelin compaction and axonal degeneration in a large number of myelinated nerve fibers. Western blots showed that the proteins of compact myelin, P0 glycoprotein, and myelin basic protein were not significantly altered in the mutants; however, the Schwann cell protein, 2',3'-cyclic nucleotide 3'-phosphodiesterase, was reduced to less than half the control level. Also, both total and phosphorylated high-molecular-weight neurofilament proteins (TNFH and PNFH, respectively) were significantly decreased, as was the PNFH:TNFH ratio. Electrophysiological evaluation revealed a mild, but statistically significant, reduction of conduction velocity and a nonsignificant mild decrease in compound muscle action potential amplitudes. This constellation of findings in aging MAG-null mice is consistent with an axonopathy that resembles axonal Charcot-Marie-Tooth (CMT2) disease in many respects. Thus, mutation of a myelin-associated gene expressed by Schwann cells can induce axonal degeneration and cause a neuropathy with minimal signs of demyelination. |
