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Molekulargenetik hereditärer Hämostasedefekte

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Herz-Kreislauf-Erkrankungen

Part of the book series: Handbuch der Molekularen Medizin ((HDBMOLEK,volume 3))

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Zusammenfassung

Die Hämostase zählt zu den wichtigsten Abwehrmechanismen des Organismus. Ein komplexes Zusammenspiel von zellularen, vaskulären und humoralen Mechanismen gewährleistet einerseits, daß das Blut innerhalb des intakten Gefäßsystems flüssig bleibt, andererseits wird nach einer Gefäßverletzung der Blutverlust durch die Ausbildung spezifischer Strukturen (primärer hämostatischer Pfropf, definitives Gerinnsel, Narbe) minimiert. Im Mittelpunkt der Hämostaseprozesse steht die Blutgerinnung, d. h. die Umwandlung des im Plasma gelösten Fibrinogens in das unlösliche Fibrin. Es handelt sich bei der Fibrinogen-Fibrin-Umwandlung um einen proteolytischen Prozeß. Das Gerinnungsenzym Thrombin spaltet vom Fibrinogenmolekül die Fibrinpeptide A und B ab. Die entstehenden Fibrinmonomere weisen eine elektrische Ladung auf, die zu einer zunächst reversiblen Polymerisation der Monomere [Fibrins (s=solubilis)] führt.

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Authors
  1. Falko H. Herrmann
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  2. Günter Vogel
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Herrmann, F.H., Vogel, G. (1998). Molekulargenetik hereditärer Hämostasedefekte. In: Ganten, D., Ruckpaul, K. (eds) Herz-Kreislauf-Erkrankungen. Handbuch der Molekularen Medizin, vol 3. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-80360-4_6

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