Development of polyglucosan inclusions in skeletal muscle.
Abstract: Muscle samples from 24 horses with polysaccharide storage myopathy were stained with periodic acid-Schiff (PAS) stain and were immunostained for ubiquitin. Abnormalities detected with PAS stain were coarse granular cytoplasmic aggregates of amylase sensitive glycogen, subsarcolemmal aggregates of glycogen, central amylase sensitive bodies, and a variety of subsarcolemmal to intracytoplasmic amylase resistant polyglucosan inclusions. All amylase resistant inclusions were positive for ubiquitin. Ubiquitin was also detected in many amylase sensitive inclusions. Based on morphologic findings and pattern of ubiquitin staining, a sequence of events, beginning with abnormal glycogen storage followed by ubiquitination and eventual development of amylase resistance, is proposed.
Publication Date: 2006-08-21 PubMed ID: 16919952DOI: 10.1016/j.nmd.2006.05.259Google Scholar: Lookup
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- Journal Article
- Research Support
- Non-U.S. Gov't
Summary
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The research paper discusses the formation of polyglucosan inclusions in the muscle tissues of horses suffering from polysaccharide storage myopathy, suggesting that the development of these inclusions starts with abnormal glycogen accumulation, progresses with ubiquitination, and ends with resistance to amylase.
Methodology
- The researchers used muscle samples from 24 horses diagnosed with polysaccharide storage myopathy.
- These samples were stained with periodic acid-Schiff (PAS) stain, a method commonly used in pathology to detect polysaccharides such as glycogen in tissues.
- Further to this, the samples were also immunostained for ubiquitin, a small protein that helps in protein degradation and is thought to play a role in the development of various diseases.
Findings
- The PAS staining revealed a range of abnormalities. This included coarse granular cytoplasmic aggregates of glycogen that can be broken down by amylase, an enzyme that digests carbohydrates.
- Also observed were subsarcolemmal aggregates of glycogen, central bodies sensitive to amylase, and various polyglucosan inclusions resistant to amylase from the subsarcolemmal to the intracytoplasmic level.
- Polyglucosan bodies are abnormal structures in muscle cells that contain branched sugars. Their presence could point to certain metabolic disorders like polysaccharide storage myopathy.
- All the amylase-resistant inclusions tested positive for ubiquitin. Additionally, many amylase-sensitive inclusions also showed the presence of ubiquitin.
Conclusion
- The presence of ubiquitin in both amylase-resistant and sensitive inclusions, and based on morphological findings, the researchers proposed a sequence of events in the development of these polyglucosan inclusions.
- This sequence starts off with abnormal glycogen storage, followed by ubiquitination (where ubiquitin tags the glycogen for degradation), leading eventually to the development of resistance to amylase, and the formation of amylase-resistant polyglucosan inclusions.
Cite This Article
APA
Valentine BA, Cooper BJ.
(2006).
Development of polyglucosan inclusions in skeletal muscle.
Neuromuscul Disord, 16(9-10), 603-607.
https://doi.org/10.1016/j.nmd.2006.05.259 Publication
Researcher Affiliations
- Veterinary Diagnostic Laboratory, Department of Biomedical Sciences, College of Veterinary Medicine, Oregon State University, 30th and Washington Way, Corvallis OR 97331, USA. Beth.Valentine@oregonstate.edu
MeSH Terms
- Amylases / analysis
- Amylases / metabolism
- Animals
- Glucans / analysis
- Glucans / metabolism
- Glycogen / analysis
- Glycogen / metabolism
- Glycogen Storage Disease / diagnosis
- Glycogen Storage Disease / metabolism
- Glycogen Storage Disease / physiopathology
- Horse Diseases / diagnosis
- Horse Diseases / metabolism
- Horse Diseases / physiopathology
- Horses
- Immunohistochemistry
- Inclusion Bodies / metabolism
- Inclusion Bodies / pathology
- Muscle Fibers, Skeletal / metabolism
- Muscle Fibers, Skeletal / pathology
- Muscle, Skeletal / metabolism
- Muscle, Skeletal / pathology
- Muscle, Skeletal / physiopathology
- Muscular Diseases / diagnosis
- Muscular Diseases / metabolism
- Muscular Diseases / physiopathology
- Periodic Acid-Schiff Reaction
- Species Specificity
- Ubiquitin / metabolism
Citations
This article has been cited 2 times.- Cenacchi G, Papa V, Costa R, Pegoraro V, Marozzo R, Fanin M, Angelini C. Update on polyglucosan storage diseases.. Virchows Arch 2019 Dec;475(6):671-686.
- 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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