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Acta neuropathologica2001; 101(5); 509-517; doi: 10.1007/s004010000315

Tubular aggregates observed in spindle muscle fiber of horse lumbrical muscle.

Abstract: Tubular aggregates (TAs) originate from the sarcoplasmic reticulum (SR) and form polymorphic double (or single) -walled structures in cross section. TAs are involved in various human skeletal muscle disorders including periodic paralysis, congenital myasthenic syndromes, inflammatory myopathies, and malignant hyperthermias. Horse lumbrical muscle (LM) is a slender fusiform muscle that shows varying degrees of regression due to its limited activity in the limb. Double-walled TAs were found in degenerating spindle fibers and with a range of 80-116 nm (average 92 nm, n=135) for outer layer and 50-78 nm (average 59 nm, n=135) for the inner layer. TAs exhibit degradation of myofibrillar proteins, disruption of mitochondria with cristae lost, glycogen accumulation, electron-dense metabolic products, blebbing appearance of sarcolemma, and presence of various vacuoles. LM fibers also show a similarly degenerative state. The disassembly of the SR structure probably produces a large accumulation of SR proteins which remain as molecules without being further degraded and which could aggregate to form the orderly structure of TAs. We believe that TA formation may be an adaptation to store unbalanced extra proteins by forming ordered aggregates in degeneration caused by stress in cells.
Publication Date: 2001-08-04 PubMed ID: 11484823DOI: 10.1007/s004010000315Google Scholar: Lookup
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  • Journal Article
  • Research Support
  • Non-U.S. Gov't

Summary

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This research paper focuses on the identification and analysis of tubular aggregates (TAs) found in degenerating spindle fibers of horse lumbrical muscle, studying their origin, composition, and possible relation to various muscular disorders.

Origins and structure of Tubular Aggregates

  • Tubular aggregates (TAs) are structures emerging from the sarcoplasmic reticulum (SR), an essential component of muscle cells in humans and animals.
  • They can take different forms, appearing as single or double-walled structures when examined in cross-section.
  • In the horse lumbrical muscle under study, double-walled TAs were discovered in degenerating spindle fibers, muscle cells undergoing deterioration.
  • The average thickness of the outer and inner layers of these formations was measured to be 92 nm and 59 nm respectively.

Conditions related to Tubular Aggregates

  • TAs have been identified as factors involved in several human skeletal muscle disorders. These include periodic paralysis, congenital myasthenic syndromes, inflammatory myopathies, and malignant hyperthermias.
  • The horse lumbrical muscle shares similar degenerative conditions where TAs have been found, which may indicate a commonality in the role of TAs in such ailments.

Characteristics and implications of Tubular Aggregates

  • Where TAs were observed, several types of cellular degradation were noted. This included breakdown of myofibrillar proteins, disturbance of mitochondria resulting in loss of inner membrane folds known as cristae, and accumulation of glycogen and electron-dense metabolic by-products.
  • Other structural changes such as malformations in the muscle cell membrane (sarcolemma) and the presence of various vacuoles were also reported.
  • The researchers suggest that the disassembly of the SR structure, a crucial component in muscle cells, might lead to the accumulation of SR proteins. These proteins, unable to be further degraded or utilized, could aggregate to form TAs.
  • The formation of TAs might be an adaptive cellular response, serving to store excess proteins in an orderly manner during times of cellular stress and degeneration.

In conclusion, the research provides valuable insights into the nature of tubular aggregates, their involvement in muscle deterioration, and their possible role in certain muscle disorders. It hints at a broader part played by these structures, underlying adaptive cellular mechanisms in response to stress and damage.

Cite This Article

APA
Klomkleaw W, Kasashima Y, Kobayashi A, Fuller GA, Morimoto M, Nakade T, Muto M, Oba T, Hamlin RL, Yamaguchi M. (2001). Tubular aggregates observed in spindle muscle fiber of horse lumbrical muscle. Acta Neuropathol, 101(5), 509-517. https://doi.org/10.1007/s004010000315

Publication

ISSN: 0001-6322
NlmUniqueID: 0412041
Country: Germany
Language: English
Volume: 101
Issue: 5
Pages: 509-517

Researcher Affiliations

Klomkleaw, W
  • Department of Veterinary Biosciences, College of Veterinary Medicine, The Ohio State University, Columbus 43210, USA.
Kasashima, Y
    Kobayashi, A
      Fuller, G A
        Morimoto, M
          Nakade, T
            Muto, M
              Oba, T
                Hamlin, R L
                  Yamaguchi, M

                    MeSH Terms

                    • Animals
                    • Disease Models, Animal
                    • Forelimb / pathology
                    • Forelimb / physiopathology
                    • Forelimb / ultrastructure
                    • Horses
                    • Microscopy, Electron
                    • Muscle Fibers, Skeletal / metabolism
                    • Muscle Fibers, Skeletal / pathology
                    • Muscle Fibers, Skeletal / ultrastructure
                    • Muscle Proteins / metabolism
                    • Muscle Spindles / metabolism
                    • Muscle Spindles / pathology
                    • Muscle Spindles / ultrastructure
                    • Muscle, Skeletal / pathology
                    • Muscle, Skeletal / physiopathology
                    • Muscle, Skeletal / ultrastructure
                    • Muscular Atrophy / pathology
                    • Muscular Atrophy / physiopathology
                    • Muscular Atrophy / veterinary
                    • Myopathies, Structural, Congenital / pathology
                    • Myopathies, Structural, Congenital / physiopathology
                    • Myopathies, Structural, Congenital / veterinary
                    • Sarcolemma / metabolism
                    • Sarcolemma / pathology
                    • Sarcolemma / ultrastructure
                    • Sarcoplasmic Reticulum / metabolism
                    • Sarcoplasmic Reticulum / pathology
                    • Sarcoplasmic Reticulum / ultrastructure

                    Citations

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