PMID: 8960313Dec 1, 1996Paper

Neuropathology of murine mucopolysaccharidosis type VII

Acta Neuropathologica
B LevyW S Sly

Abstract

We describe the neuropathology in mucopolysaccharidosis type VII (MPS VII) mice with a recessively inherited deficiency of the lysosomal enzyme beta-glucuronidase. Affected animals have a shortened life span, are dysmorphic, dwarfed and have clinical evidence of behavioral and memory deficiencies. Widespread lysosomal distention with glycosaminoglycan accumulation affects most viscera. In the central nervous system there is progressive accumulation of lysosomal storage in neurons, glia and mesenchymal tissue. The morphological character and the amount of lysosomal storage varies among neuronal groups. In the hippocampus, regional variation in the abundance of lysosomal storage in the MPS VII mice correlates with regional variation in the amount of beta-glucuronidase activity in normal mice. The MPS VII mouse provides a well-defined genetic system for the analysis of the neuropathology of MPS VII and is an attractive model on which to test the effects of potential therapies for lysosomal storage disease on the central nervous system.

Citations

Nov 26, 2002·Annals of Neurology·Gregory G HeuerJohn H Wolfe
Jan 20, 2007·Molecular Therapy : the Journal of the American Society of Gene Therapy·Gumei LiuBeverly L Davidson
Sep 16, 2005·Proceedings of the National Academy of Sciences of the United States of America·Carole VoglerWilliam S Sly
Apr 8, 2016·Molecular Genetics and Metabolism·Michael K ParenteJohn H Wolfe
Jul 14, 2011·APMIS : Acta Pathologica, Microbiologica, Et Immunologica Scandinavica·Agnes ChenPatricia Dickson
Mar 7, 2006·Molecular Therapy : the Journal of the American Society of Gene Therapy·Bradford K BergesNigel W Fraser
Feb 25, 2006·Molecular Therapy : the Journal of the American Society of Gene Therapy·Iryna A IsakovaDonald G Phinney
Mar 29, 2001·Molecular Therapy : the Journal of the American Society of Gene Therapy·W A FrisellaM S Sands
Aug 27, 2005·Molecular Therapy : the Journal of the American Society of Gene Therapy·B K BergesN W Fraser
Feb 8, 2003·Molecular Therapy : the Journal of the American Society of Gene Therapy·Pampee P YoungMark S Sands
Nov 6, 2003·Molecular Therapy : the Journal of the American Society of Gene Therapy·Arihiko KanajiTorayuki Okuyama
Jan 18, 2006·Molecular Therapy : the Journal of the American Society of Gene Therapy·Cassia N Cearley, John H Wolfe
Oct 14, 2005·Pediatric and Developmental Pathology : the Official Journal of the Society for Pediatric Pathology and the Paediatric Pathology Society·Carole VoglerJane Barker
Jun 1, 2000·Pediatric Research·M L Casal, J H Wolfe
Jun 22, 1999·The Journal of Histochemistry and Cytochemistry : Official Journal of the Histochemistry Society·G C KopenD G Phinney
Oct 31, 2000·Veterinary Pathology·P C SchultheissM A Thrall
Aug 3, 2020·Molecular Genetics and Metabolism·Ainslie Derrick-RobertsSharon Byers
Jul 28, 2021·Proceedings of the National Academy of Sciences of the United States of America·Dhananjay YellajoshyulaWilliam T Dauer

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