Ubiquitin expression in muscle from horses with polysaccharide storage myopathy.
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.
Publication Date: 2006-05-05 PubMed ID: 16672573DOI: 10.1354/vp.43-3-270Google Scholar: Lookup
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- Journal Article
- Research Support
- Non-U.S. Gov't
Summary
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This research paper studies the occurrence of ubiquitin expression in muscles of horses diagnosed with equine polysaccharide storage myopathy (EPSSM), a muscle disease. The study found that ubiquitin was generally present in all but one EPSSM horse and absent in non-EPSSM horses, suggesting that ubiquitin immunostaining may be useful for confirming an EPSSM diagnosis but is no more effective than the previous method using periodic acid-Schiff (PAS) staining.
Sample Collection and Treatment
- The researchers collected muscle biopsy specimens from a group of 28 horses across several breeds. These samples were formally fixed in formalin and embedded in paraffin.
- The specimens were then treated using a PAS stain, a common staining procedure used to detect polysaccharides like glycogen, and were also immunostained to identify ubiquitin expression.
Determination of Findings
- Of the 28 horse samples, 22 were diagnosed with EPSSM based on the results of the PAS-stained sections. These horses were aged between 2 and 23 years.
- The other six horses, which ranged from 0.5 to 15 years old, demonstrated a normal PAS staining pattern with no significant lesions, therefore they were deemed not affected by EPSSM.
- Ubiquitin expression was identified in all the EPSSM-affected horses barring one. On the contrary, it was not present in the non-EPSSM horses.
Ubiquitin Expression and Diagnosis of EPSSM
- The degree of ubiquitin expression was higher than the degree of PAS-positive, resistant material. Importantly, ubiquitin was detected in both amylase-sensitive glycogen aggregates and in amylase-resistant material.
- This suggests that glycogen aggregates develop and are marked by ubiquitin prior to the formation of amylase-resistant inclusions.
- The study concluded that ubiquitin immunostaining might be useful to confirm the diagnosis of EPSSM in horses, especially those with only amylase-sensitive glycogen and those with early amylase-resistant inclusions.
- However, the sensitivity of ubiquitin immunostaining for the diagnosis of EPSSM was not deemed higher than the sensitivity of PAS staining. This suggests that both diagnostic methods could be utilized with similar degrees of accuracy.
Cite This Article
APA
Valentine BA, Flint TH, Fischer KA.
(2006).
Ubiquitin expression in muscle from horses with polysaccharide storage myopathy.
Vet Pathol, 43(3), 270-275.
https://doi.org/10.1354/vp.43-3-270 Publication
Researcher Affiliations
- Department of Biomedical Sciences, College of Veterinary Medicine, Oregon State University, Magruder 142, Corvallis, OR 97331, USA. Beth.Valentine@oregonstate.edu
MeSH Terms
- Animals
- Gene Expression Regulation
- Glycogen Storage Disease / metabolism
- Glycogen Storage Disease / pathology
- Glycogen Storage Disease / veterinary
- Horse Diseases / metabolism
- Horse Diseases / pathology
- Horses
- Muscle, Skeletal / metabolism
- Muscle, Skeletal / pathology
- Muscular Diseases / metabolism
- Muscular Diseases / pathology
- Muscular Diseases / veterinary
- Polysaccharides / metabolism
- Ubiquitins / genetics
- Ubiquitins / metabolism
Citations
This article has been cited 1 times.- Naylor RJ, Livesey L, Schumacher J, Henke N, Massey C, Brock KV, Fernandez-Fuente M, Piercy RJ. Allele copy number and underlying pathology are associated with subclinical severity in equine type 1 polysaccharide storage myopathy (PSSM1).. PLoS One 2012;7(7):e42317.
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