title: Osteogenesis imperfecta: morphological, histochemical and biochemical aspects. Modifications induced by (+)-catechin.
abstract: Two patients affected with two different forms of Osteogenesis Imperfecta were examined in order to study collagen and glycosaminoglycans (GAGs) in skin and iliac crest cartilage. A sharp decrease of the galactosamine to glucosamine ratio due to a reduced content of chondroitin sulfate was evidenced in both patients. Moreover the structure of proteoglycans appeared altered, this being more evident in the severe form of the disease. Morphological examination in light and electron microscopy of cartilage of the less severely diseased patient showed that GAGs in the extracellular matrix did not present regular connection with collagen fibers. Chondrocytes, elongated and disorderly scattered, showed large lipidic inclusions and, on histochemical basis, were devoid of UDPG dehydrogenase activity. Treatment with (+)-catechin produced an improvement, in both patients, of the biochemical pattern of collagen and GAGs. Similarly a shift of the cellular activity and of the matrix morphology towards normality was observed in the investigated cartilage of the less severely affected patient.
mesh_terms: D000293:Adolescent; D001578:Benzopyrans / Q000627:therapeutic use*; D002356:Cartilage / Q000378:metabolism / Q000473:pathology*; D002392:Catechin / Q000627:therapeutic use*; D003094:Collagen / Q000032:analysis; D005260:Female; D005688:Galactosamine / Q000032:analysis; D006025:Glycosaminoglycans / Q000032:analysis; D006801:Humans; D007223:Infant; D007770:L-Lactate Dehydrogenase / Q000378:metabolism; D010013:Osteogenesis Imperfecta / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology*; D014533:Uridine Diphosphate Glucose Dehydrogenase / Q000378:metabolism
chemical_list: D001578:Benzopyrans; D006025:Glycosaminoglycans; D005688:Galactosamine; D002392:Catechin; D003094:Collagen; D014533:Uridine Diphosphate Glucose Dehydrogenase; D007770:L-Lactate Dehydrogenase