elastin and Polychondritis--Relapsing

The cells of the connective tissues contain lysosomes with enzymes capable of degrading intercellular substances (collagen, elastin, proteoglycans) and release their enzymes in membrane-bound or in free form into their intercellular substance. In this way extracellularly located lysosomes (= matrix lysosomes) can be detected by morphological and enzymatic means as well as by their metal concentrating property. This function of such matrix-lysosomes is in connection with the two step-degradation of the connective tissue and is thought to be the main part for the fibrocytic fibrolysis, chondrocytic chondrolysis, osteocytic osteolysis and myocytic mediolyses in the vessel wall. The cells of the mesenchymal tissues thus control the turnover and transformation of their own ground substance. Inflammatory and immunologic processes are suggested to be superimposed on this lysosomal action. If the lysosomal enzyme system in the connective tissues and the vessel wall gets out of control, the consequences can be dangerous as e.g. in case of relapsing polychondritis and arterial aneurysms. In this enzyme system proteolytic activators as well as proteolytic decomposable inhibitors are present. The corresponding proteolytic processes are of lysosomal nature and are subordinated to other regulatory mechanisms.

Topics: Aneurysm; Animals; Arthritis, Rheumatoid; Collagen Diseases; Connective Tissue; Elastin; Female; Humans; Lysosomes; Male; Microscopy, Electron; Middle Aged; Polychondritis, Relapsing; Proteoglycans; Rats; Vascular Diseases

Topics: Adult; Amino Acids; Aorta, Thoracic; Aortic Valve; Aortic Valve Insufficiency; Collagen; Deafness; Elastin; Humans; Lipids; Male; Mitral Valve Insufficiency; Myocardium; Nose Deformities, Acquired; Phospholipids; Polychondritis, Relapsing