title: In-utero and post-delivery supplementation of motor neuron degeneration mutant mice with polyunsaturated fatty acids does not alter the clinical or pathological course.
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.
authors.affiliation: Department of Pathology and Pediatrics, University of Texas Southwestern Medical Center, Children's Medical Center of Dallas 75235, USA.
authors.forename: M J
authors.initials: MJ
authors.lastname: Bennett
authors.forename: R L
authors.initials: RL
authors.lastname: Boriack
authors.forename: D G
authors.initials: DG
authors.lastname: Birch
mesh_terms: 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
chemical_list: D005231:Fatty Acids, Unsaturated