Myofibrillar Myopathy.
Abstract: Myofibrillar myopathy (MFM) is characterized by segmental disarray of myofibrils and ectopic accumulation of a protein called desmin. Previously thought to be a glycogen storage disease, MFM is now recognized as a stand-alone myopathy. Endurance Arabians with MFM usually present with exertional rhabdomyolysis (MFM-ER) at the end of races, elevated serum muscle enzymes, and myoglobinuria. Warmblood horses with MFM (MFM-WB) usually present with pain-associated behaviors such as exercise intolerance, reluctance to engage hind quarter muscles, shifting lameness and normal serum muscle enzymes. Both forms have evidence of decreased cysteine-based antioxidants and, additionally, MFM-WB has molecular signatures of a maladaptive training response.
Copyright © 2024 Elsevier Inc. All rights reserved.
Publication Date: 2025-01-28 PubMed ID: 39880730DOI: 10.1016/j.cveq.2024.11.005Google Scholar: Lookup
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Summary
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The research article discusses Myofibrillar Myopathy (MFM), a condition characterized by disarray of muscle fibres and accumulation of a protein called desmin in Arabian and Warmblood horses. This could lead to muscle damage after exercise in Arabians and pain-related behaviors in Warmblood horses.
Understanding Myofibrillar Myopathy (MFM)
- Myofibrillar Myopathy, or MFM, is a condition in horses where the myofibrils (protein structures responsible for muscle contraction) are disarranged and thereby function abnormally. This condition is accompanied by the aberrant accumulation of a protein called desmin, which plays a crucial role in maintaining muscle structural integrity.
- Earlier considered to be a glycogen storage disease (a metabolic disorder), recent studies have established MFM as a unique muscular disorder.
Two Different Presentations of MFM
- MFM in Endurance Arabians (MFM-ER): Arabian horses affected with MFM usually show signs of exertional rhabdomyolysis, a condition causing muscle damage and pain, at the end of races. This is accompanied by increased levels of muscle enzymes in their blood, indicating muscle damage, and myoglobinuria, which is the presence of myoglobin in urine, a byproduct of muscle breakdown.
- MFM in Warmblood Horses (MFM-WB): In contrast, Warmblood horses suffering from MFM exhibit behaviors associated with discomfort, such as exercise intolerance and reluctance to engage their hindquarter muscles. Despite these outward signs, their serum muscle enzymes remain normal.
Underlying Biochemical Changes
- Decreased Antioxidants: Both forms of MFM, whether in Arabian or Warmblood horses, have been linked to reduced levels of cysteine-based antioxidants. Antioxidants play an essential role in fighting oxidative damage in cells, and a decrease could imply increased vulnerability to such damage, affecting muscle health.
- Maladaptive Response in Warmblood Horses: A unique feature to MFM-WB is the presence of molecular signatures indicating a maladaptive training response. This implies that when these horses are subjected to training, their body responds unfavorably, possibly exacerbating their symptoms and discomfort.
Cite This Article
APA
Valberg SJ, Williams ZJ.
(2025).
Myofibrillar Myopathy.
Vet Clin North Am Equine Pract, 41(1), 139-150.
https://doi.org/10.1016/j.cveq.2024.11.005 Publication
Researcher Affiliations
- Michigan State University, Large Animal Clinical Sciences, College of Veterinary Medicine, East Lansing, MI, USA.
- Department of Clinical Sciences, College of Veterinary Medicine and Biomedical Sciences, Colorado State University, Fort Collins, CO, USA. Electronic address: Zoe.Williams@colostate.edu.
MeSH Terms
- Animals
- Horse Diseases / diagnosis
- Horse Diseases / pathology
- Horse Diseases / therapy
- Horses
- Myopathies, Structural, Congenital / veterinary
- Myopathies, Structural, Congenital / diagnosis
- Myopathies, Structural, Congenital / therapy
- Myopathies, Structural, Congenital / pathology
- Physical Conditioning, Animal
Conflict of Interest Statement
Disclosure S.J. Valberg directs the Valberg Neuromuscular Disease laboratory (ValbergNMDL.com) and receives remuneration for analyzing muscle biopsy as well as royalties from the PSSM1 genetic test, the feed Re-Leve, and MFM pellet sold by Kentucky Equine Research. Z.J. Williams reports no conflict of interest.
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