Tubular aggregates observed in spindle muscle fiber of horse lumbrical muscle.
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.
Klomkleaw
W
W
Department of Veterinary Biosciences, College of Veterinary Medicine, The Ohio State University, Columbus 43210, USA.
Kasashima
Y
Y
Kobayashi
A
A
Fuller
G A
GA
Morimoto
M
M
Nakade
T
T
Muto
M
M
Oba
T
T
Hamlin
R L
RL
Yamaguchi
M
M
D000818:Animals; D004195:Disease Models, Animal; D005552:Forelimb / Q000473:pathology* / Q000503:physiopathology / Q000648:ultrastructure; D006736:Horses; D008854:Microscopy, Electron; D018485:Muscle Fibers, Skeletal / Q000378:metabolism / Q000473:pathology* / Q000648:ultrastructure; D009124:Muscle Proteins / Q000378:metabolism; D009470:Muscle Spindles / Q000378:metabolism / Q000473:pathology* / Q000648:ultrastructure; D018482:Muscle, Skeletal / Q000473:pathology* / Q000503:physiopathology / Q000648:ultrastructure; D009133:Muscular Atrophy / Q000473:pathology* / Q000503:physiopathology / Q000662:veterinary; D020914:Myopathies, Structural, Congenital / Q000473:pathology* / Q000503:physiopathology / Q000662:veterinary; D012508:Sarcolemma / Q000378:metabolism / Q000473:pathology / Q000648:ultrastructure; D012519:Sarcoplasmic Reticulum / Q000378:metabolism / Q000473:pathology* / Q000648:ultrastructure
D009124:Muscle Proteins