MARFANS-SYNDROME - INVITRO STUDY OF COLLAGEN-METABOLISM IN TISSUE SPECIMENS OF AORTA

  • 1 January 1977
    • journal article
    • research article
    • Vol. 45  (3-4) , 207-221
Abstract
An aorta tissue specimen was obtained from a patient with Marfan''s syndrome after heart surgery. Media and adventitia were carefully separated and subsequently used for biochemical characterization. Aortas from an embryonic calf and a human adult were used as internal controls. An insufficient synthesis of type I collagen rather than a defect in the formation of cross-links may be the prime cause in this inherited connective tissue disorder. Such a disturbance in the regulation of the genes coding for the different collagen chains may explain the weakness and enhanced extensibility of the blood vessels finally resulting in an aneurysm.