Download PDF
Neuropediatrics 1997; 28(1): 82-84
DOI: 10.1055/s-2007-973678
Original articles

© Hippokrates Verlag GmbH Stuttgart

In-Utero and Post-Delivery Supplementation of Motor Neuron Degeneration Mutant Mice with Polyunsaturated Fatty Acids Does Not Alter the Clinical or Pathological Course

M. J. Bennett1 , R. L. Boriack1 , D. G. Birch2
  • 1Department of Pathology and Pediatrics, University of Texas Southwestern Medical Center, Children's Medical Center of Dallas, 1935 Motor Street, Dallas, TX 75235,
  • 2Retina Foundation of the Southwest, 9900 N. Central Expressway, Dallas, TX 75231, USA
Further Information

Publication History

Publication Date:
13 March 2007 (online)

Also available at
  PDF Download  Permissions and Reprints

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-itro studies in humans with CLN-3, and in-vitro studies in CLN-3 lympho-blasts, 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.

Key words

Juvenile neuronal ceroid lipofuscinosis - Batten disease - Mnd mutant mice - Polyunsaturated fatty acids -Electroretinography - Electron microscopy