Ubiquitin expression in muscle from horses with polysaccharide storage myopathy.

TitleUbiquitin expression in muscle from horses with polysaccharide storage myopathy.
Publication TypeJournal Article
Year of Publication2006
AuthorsValentine BA, Flint TH, Fischer KA
JournalVeterinary pathology
Volume43
Issue3
Pagination270-5
Date Published2006 May
ISSN0300-9858
KeywordsAnimals, Gene Expression Regulation, Glycogen Storage Disease, Horse Diseases, Horses, Muscle, Skeletal, Muscular Diseases, Polysaccharides, Ubiquitins
Abstract

Serial sections of formalin-fixed, paraffin-embedded muscle biopsy specimens from 28 Quarter Horse, Paint, and draft-related breeds, aged 0.5-23 years, were treated with periodic acid-Schiff (PAS) stain for glycogen and were immunostained to detect ubiquitin expression. On the basis of findings in PAS-stained sections, a diagnosis of equine polysaccharide storage myopathy (EPSSM) was made in 22 horses aged 2-23 years (mean, 9.4 years); samples from 6 horses aged 0.5-15 years (mean, 7.3 years) had a normal PAS staining pattern, with no relevant lesions. Ubiquitin expression was detected in all but a 2-year-old EPSSM-affected horse and was not detected in the non-EPSSM-affected horses. Ubiquitin expression was greater than the degree of PAS-positive, amylase-resistant material, and ubiquitin was detected in aggregates of amylase-sensitive glycogen as well as in aggregates of amylase-resistant material. Results suggest that glycogen aggregates develop and are ubiquitinated prior to development of amylase-resistant inclusions. Ubiquitin immunostaining may be most useful for confirming the diagnosis of EPSSM in horses with only amylase-sensitive glycogen aggregates and in horses with early amylase-resistant inclusions. However, ubiquitin immunostaining is no more sensitive than is PAS staining for diagnosis of EPSSM.

Alternate JournalVet. Pathol.