Altered secretion of chondroitin sulfate proteoglycan in Duchenne muscular dystrophy cultures.
We have characterised the CSPG secreted into the nutrient medium of human skeletal muscle cultures from normal, Duchenne muscular dystrophy (DMD) and other myopathic muscle, using radiolabelling techniques and molecular sieve chromatography. Results from these studies show alterations in the accumulation and sulphation of a high molecular weight chondroitin sulphate proteoglycan, in the nutrient medium of skeletal muscle cultures obtained from DMD but not other myopathic sources. These include a significant reduction in the accumulation of such molecules in pre-fusion cultures, and in the accumulation of an undersulphated molecule in post-fusion cultures.
D000328:Adult; D002478:Cells, Cultured; D011508:Chondroitin Sulfate Proteoglycans / Q000378:metabolism*; D006801:Humans; D008970:Molecular Weight; D009132:Muscles / Q000378:metabolism*; D009136:Muscular Dystrophies / Q000378:metabolism*; D011509:Proteoglycans / Q000378:metabolism*
D011508:Chondroitin Sulfate Proteoglycans; D011509:Proteoglycans