title: [Pathophysiology and metabolism in osteogenesis imperfecta (author's transl)].
abstract: The tissue changes in osteogenesis imperfecta apparently lie in the collagen and in the glycosaminoglycans. The collagen shows structural development disturbance and incomplete calcification. The tissue glycosaminoglycans are increased. The basis probably lies in a disturbed ATP metabolism (Solomons and Millar, 1973). An increased pyrophosphate concentration explains the decreased calcification and the structural changes. The increased osteolysis could be explained by increased quantities of cAMP.
mesh_terms: D001862:Bone Resorption; D002463:Cell Membrane Permeability; D003094:Collagen / Q000378:metabolism; D000242:Cyclic AMP / Q000378:metabolism; D011756:Diphosphates / Q000378:metabolism; D004734:Energy Metabolism; D006025:Glycosaminoglycans / Q000378:metabolism; D006801:Humans; D010013:Osteogenesis Imperfecta / Q000378:metabolism / Q000503:physiopathology*
chemical_list: D011756:Diphosphates; D006025:Glycosaminoglycans; D003094:Collagen; D000242:Cyclic AMP
vernacular_title: Zur Pathophysiologie und über Stoffwechselstörungen bei der Osteogenesis imperfecta.