concanavalin-a and Muscular-Dystrophies

Differences in glycosylation between molecular forms of acetylcholinesterase (AChE) and butyrylcholinesterase (BuChE) in muscle and serum of normal and dystrophic mice have been studied by means of their adsorption to immobilized lectins. Application of a two-step extraction procedure, first with saline buffer, and second with saline buffer and Triton X-100, brought into solution most of the muscle AChE and BuChE activities. The AChE activity was five times greater than that of BuChE in normal (NM) and dystrophic muscle (DM). The AChE activity in the serum of dystrophic mice was twice that measured in control animals, but the BuChE activity remained almost unchanged. Both AChE and BuChE in muscle and serum bound completely to concanavalin A (Con A) and Lens culinaris agglutinin (LCA). A12, A8 and G4 AChE, but not the light G2 and G1 AChE forms, in NM and DM were completely adsorbed to wheat germ agglutinin (WGA). Similarly, G4 BuChE, but not the G2 and G1 forms, were associated to WGA. A high proportion of G4 and G1 AChE and G4 BuChE forms in mouse serum were fixed to WGA. Asymmetric AChE in NM and DM reacted with Ricinus communis agglutinin (RCA) but the light AChE and BuChE forms in muscle and serum did not bind to the lectin. G4 AChE and G4 BuChE in NM were not recognized by RCA, but the isoforms in DM bound fully to the lectin. Serum G4 AChE from control or dystrophic mice did not react with RCA, but G4 BuChE was fixed to the lectin. Since RCA is specific for galactose, the results suggest that in dystrophic muscle galactose is incorporated early in G4 AChE and this affects the level of the functional tetramers destined for insertion in the plasma membrane.

Topics: Acetylcholinesterase; Animals; Butyrylcholinesterase; Centrifugation, Density Gradient; Concanavalin A; Glycosylation; Isoenzymes; Lectins; Mice; Muscles; Muscular Dystrophies; Plant Lectins; Wheat Germ Agglutinins

Topics: Adolescent; Cell Survival; Cells, Cultured; Child; Child, Preschool; Concanavalin A; Female; Fibroblasts; Humans; Male; Muscular Dystrophies; Myotonic Dystrophy; Skin; Wheat Germ Agglutinins

There have been conflicting studies of lymphocyte capping from patients with Duchenne Muscular Dystrophy. We have evaluated the proportion of capped fibroblasts of 10 patients with Duchenne Muscular Dystrophy. The results, compared with 15 normal controls, showed that the reduction of fibroblast capping is correlated to age patients. Additional studies, decreased number of retracted fibroblasts after colchicine incubation and ultrastructural observations, suggest that capping deficit would be the consequence of microtubular system alteration. This late phenomenon may be secondary to the primary membrane defect.

Topics: Adolescent; Adult; Aged; Aging; Cells, Cultured; Child; Child, Preschool; Colchicine; Concanavalin A; Fibroblasts; Humans; Infant; Lysosomes; Microscopy, Electron; Microtubules; Middle Aged; Muscular Dystrophies

An investigation of [125I]Con A binding to skin fibroblasts from Duchenne muscular dystrophy patients and age-matched controls was carried out. The age groups examined were 5-6 years, 11-12 years, and 15-17 years. Only small differences in binding abilities were observed between dystrophic cells and matched controls. When data was examined as micrograms Con A bound/micrograms protein, dystrophic fibroblasts bound slightly more lectin compared to controls with the 5-6 and 11-12 year age groups, whereas the 15-17 years age group bound slightly less Con A compared to normal controls. However, analysis of binding data as lectin bound/cell showed slightly reduced binding of Con A to dystrophic cells from all age groups when compared to matched controls. It was also found that the amount of Con A bound by both normal and dystrophic fibroblasts markedly increased with the age of the donor. Obviously several factors must be taken into account when analyzing lectin binding data obtained with human fibroblasts. Taken as a whole, our studies do not provide evidence for significant modification of cell surface Con A receptors on fibroblasts from Duchenne muscular dystrophy patients.

Topics: Adolescent; Cells, Cultured; Child; Child, Preschool; Concanavalin A; Fibroblasts; Glycoproteins; Humans; Kinetics; Muscular Dystrophies; Receptors, Concanavalin A; Skin

Topics: Adult; Cells, Cultured; Concanavalin A; Fibroblasts; Humans; Male; Muscular Dystrophies; Staining and Labeling

Topics: Christianity; Concanavalin A; Consanguinity; Female; Gene Frequency; Genes, Recessive; Genetic Carrier Screening; Humans; Immunologic Capping; Indiana; Lymphocytes; Male; Muscular Dystrophies; Pedigree; Receptors, Antigen, B-Cell; Risk

Topics: Acetylcholine; Cells, Cultured; Concanavalin A; Epinephrine; Fibroblasts; Humans; Kinetics; Muscular Dystrophies; Phosphatidic Acids; Phosphatidylinositols; Phosphorus Radioisotopes; Skin