Duchenne muscular dystrophy: lack of differences in the expression of endogenous carbohydrate- and heparin-binding proteins (lectins) in cultured fibroblasts.
Duchenne muscular dystrophy (DMD), the most severe form of inherited muscular dystrophies, is known to be caused by a deficiency of the protein "dystrophin", but the pathophysiologic consequences of this lack have not as yet been elucidated. Investigations with cultured fibroblasts point to altered adhesion mechanisms in cells from DMD patients. Because endogenous carbohydrate-binding proteins (lectins) play an important role in cell-cell and cell-matrix interactions and therefore might be involved in these alterations, we separated such proteins with specificities for beta-galactosyl-, alpha-mannosyl-, and alpha-glucosyl-residues, as well as heparin-binding proteins from cultured fibroblasts. Owing to enormous interindividual variations among DMD patients and among healthy controls our hypothesis could not be confirmed. In the course of the experiments we could detect a growth-related binding of heparin to nuclei of cells from DMD patients and healthy controls restricted to rapidly growing cultures and telophases of mitoses, but not in contact-inhibited monolayers. This finding suggested a possible involvement of the cellular ligand in growth control and coincided with the occurrence of basic fibroblast growth factor.
Stulnig
T
T
Institut für Medizinische Biologie und Humangenetik, Universität Innsbruck, Austria.
Schweiger
M
M
Hirsch-Kauffmann
M
M
D001665:Binding Sites; D050260:Carbohydrate Metabolism; D002352:Carrier Proteins / Q000096:biosynthesis* / Q000737:chemistry / Q000378:metabolism; D002478:Cells, Cultured; D016222:Fibroblast Growth Factor 2 / Q000378:metabolism; D005347:Fibroblasts / Q000378:metabolism; D006493:Heparin / Q000378:metabolism*; D006801:Humans; D037102:Lectins / Q000096:biosynthesis* / Q000737:chemistry / Q000378:metabolism; D008938:Mitosis; D008970:Molecular Weight; D009136:Muscular Dystrophies / Q000378:metabolism* / Q000473:pathology
D002352:Carrier Proteins; D037102:Lectins; D016222:Fibroblast Growth Factor 2; D006493:Heparin