GONUTS has been updated to MW1.31 Most things seem to be working but be sure to report problems.
PMID:9151333
| Citation |
Bennett, MJ, Boriack, RL and Birch, DG (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 28:82-4 |
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| Abstract |
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. |
| Links |
PubMed Online version:10.1055/s-2007-973678 |
| Keywords |
Animals; Animals, Newborn; Brain/pathology; Disease Models, Animal; Fatty Acids, Unsaturated/administration & dosage; Female; Male; Mice; Mice, Neurologic Mutants; Microscopy, Electron; Neuronal Ceroid-Lipofuscinoses/diet therapy; Neuronal Ceroid-Lipofuscinoses/genetics; Neuronal Ceroid-Lipofuscinoses/pathology; Pregnancy |
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Significance
Annotations
| Gene product | Qualifier | GO ID | GO term name | Evidence Code | with/from | Aspect | Notes | Status |
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