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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
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Publication History

Publication Date:
13 March 2007 (online)

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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-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

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