Specific binding of Ulex europaeus agglutinin I lectin to sarcolemma of distal myopathy with rimmed vacuole formation.
Ulex europaeus agglutinin I (UEA I) binding was studied in 83 patients with various neuromuscular disorders. UEA I labelled endomysial capillaries and endothelial cells of perimysial blood vessels in all the examined muscles. There was no UEA I binding to muscle fibres except for all (9) cases of distal myopathy with rimmed vacuole formation (DMRV), 1 of 5 cases of inclusion body myositis and 1 of 36 cases of inflammatory myopathies. The UEA I binding was completely eliminated by preincubation of UEA I solution with L-fucose. Using electron microscopy, the UEA I binding was localized to sarcolemma and intrasarco-plasmic membranous organelles other than mitochondria. Myosatellite cells were not labelled. These findings revealed the existence of fucosylated proteins or lipids in a subset of skeletal muscles suffering from DMRV. Biochemical identification of the fucosylated substance and further detailed study on subcellular localization of UEA I binding may yield important clues to the unknown pathogenesis of DMRV.
Yatabe
K
K
Department of Neurology, Tokyo Women's Medical College, Japan.
Kawai
M
M
D006651:Histocytochemistry; D006801:Humans; D037102:Lectins / Q000378:metabolism*; D008854:Microscopy, Electron; D009135:Muscular Diseases / Q000378:metabolism* / Q000473:pathology*; D037121:Plant Lectins; D012508:Sarcolemma / Q000378:metabolism*; D014617:Vacuoles / Q000473:pathology*
D037102:Lectins; D037121:Plant Lectins; C036850:Ulex europaeus lectins