In-utero and post-delivery supplementation of motor neuron degeneration mutant mice with polyunsaturated fatty acids does not alter the clinical or pathological course.
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.
Bennett
M J
MJ
Department of Pathology and Pediatrics, University of Texas Southwestern Medical Center, Children's Medical Center of Dallas 75235, USA.
Boriack
R L
RL
Birch
D G
DG
D000818:Animals; D000831:Animals, Newborn; D001921:Brain / Q000473:pathology; D004195:Disease Models, Animal; D005231:Fatty Acids, Unsaturated / Q000008:administration & dosage*; D005260:Female; D008297:Male; D051379:Mice; D008818:Mice, Neurologic Mutants; D008854:Microscopy, Electron; D009472:Neuronal Ceroid-Lipofuscinoses / Q000178:diet therapy* / Q000235:genetics / Q000473:pathology; D011247:Pregnancy
D005231:Fatty Acids, Unsaturated