Scripps VIVO scripps research logo

  • Index
  • Log in
  • Home
  • People
  • Organizations
  • Research
  • Events
Search form

Deficiency of protein-C inhibitor in combined factor-V-VIII deficiency disease

Academic Article
uri icon
  • Overview
  • Identity
  • Additional Document Info
  • View All
scroll to property group menus

Overview

authors

  • Marlar, R. A.
  • Griffin, John

publication date

  • 1980

journal

  • Journal of Clinical Investigation  Journal

abstract

  • Activated protein C is an anticoagulant plasma protease enzyme that inactivates Factors V and VIII in plasma. Normal plasma contains a protein that inhibits activated protein C and that is distinct from previously described plasma protease inhibitors. Protein C inhibitory activity is not detectable in plasmas from four unrelated patients with combined Factor V/VIII deficiency but is present in normal amounts in plasmas from patients with simple factor V deficiency or Factor VIII deficiency. It is suggested that the molecular basis for combined Factor V/VIII deficiency that exhibits simple autosomal recessive inheritance is a deficiency of protein C inhibitor.

subject areas

  • Blood Proteins
  • Chromatography, Ion Exchange
  • Factor V Deficiency
  • Hemophilia A
  • Protein C Inhibitor
scroll to property group menus

Identity

International Standard Serial Number (ISSN)

  • 0021-9738

Digital Object Identifier (DOI)

  • 10.1172/jci109952

PubMed ID

  • 6253526
scroll to property group menus

Additional Document Info

start page

  • 1186

end page

  • 1189

volume

  • 66

issue

  • 5

©2021 The Scripps Research Institute | Terms of Use | Powered by VIVO

  • About
  • Contact Us
  • Support