| First Author | Bennett MJ | Year | 1997 |
| Journal | Neuropediatrics | Volume | 28 |
| Issue | 1 | Pages | 82-4 |
| PubMed ID | 9151333 | Mgi Jnum | J:42641 |
| Mgi Id | MGI:1096072 | Doi | 10.1055/s-2007-973678 |
| Citation | Bennett MJ, et al. (1997) In-utero and post-delivery supplementation of motor neuron degeneration mutant mice with polyunsaturated fatty acids does not alter the clinical or pathological course. Neuropediatrics 28(1):82-4 |
| abstractText | We have studied the effects of polyunsaturated fatty acid (PUFA) supplementation in utero and throughout life in mnd mutant mice, a proposed model for juvenile neuronal ceroid lipofuscinosis (CLN-3). Unlike our earlier in-vitro studies in humans with CLN-3, and in-vitro studies in CLN-3 lymphoblasts, we saw no beneficial effects in electroretinographic, electron microscopic or clinical studies in the mnd mice. Electron microscopy of brain revealed a pattern which was not consistent with the characteristic ceroid patterns in CLN-3. Our data suggest that the mnd mouse is not responsive to PUFA supplementation and may not be an appropriate animal model for CLN-3. |
