Lysosomal Storage Diseases of Animals: An Essay in Comparative Pathology

R. D. Jolly, S. U. Walkley

Research output: Contribution to journalReview articlepeer-review

108 Scopus citations

Abstract

A wide variety of inherited lysosomal hydrolase deficiencies have been reported in animals and are characterized by accumulation of sphingolipids, glycolipids, oligosaccharides, or mucopolysaccharides within lysosomes. Inhibitors of a lysosomal hydrolase, e.g., swainsonine, may also induce storage disease. Another group of lysosomal storage diseases, the ceroid-lipofuscinoses, involve the accumulation of hydrophobic proteins but their pathogenesis is unclear. Some of these diseases are of veterinary importance, and those caused by a hydrolase deficiency can be controlled by detection of heterozygotes through the gene dosage phenomenon or by molecular genetic techniques. Other of these diseases are important to biomedical research either as models of the analogous human disease and/or through their ability to help elucidate specific aspects of cell biology. Some of these models have been used to explore possible therapeutic strategies and to define their limitations and expectations.

Original languageEnglish (US)
Pages (from-to)527-548
Number of pages22
JournalVeterinary Pathology
Volume34
Issue number6
DOIs
StatePublished - Nov 1997

Keywords

  • Animal models
  • Axons
  • Dendrites
  • Lysosomes
  • Neurodegenerative disease
  • Neurons
  • Storage diseases

ASJC Scopus subject areas

  • veterinary(all)

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