The Hageman factor, is a plasma protein now usually known as factor XII. It is part of the coagulation cascade and activates factor XI and prekallikrein. It is an enzyme (EC 3.4.21.38 http://www.expasy.org/cgi-bin/nicezyme.pl?3.4.21.38 ) of the serine protease (or serine endopeptidase) class.
Hageman factor deficiency is a rare hereditary disorder with a prevalence of about one in a million, although it is a little more common among Asians. Deficiency does not cause excessive haemorrhage as the other coagulation factors make up for the it. It may increase the risk of thrombosis, due to inadequate activation of the fibrinolytic pathway.
Hageman factor was first discovered in 1955 when a routine preoperative blood sample of the 37-year-old railroad brakeman John Hageman was found to have prolonged clotting time in test tubes, even though he had no haemorrhagic symptoms. Hageman was then examined by Dr. Oscar Ratnoff who found that Mr. Hageman lacked a previously unidentified clotting factor. Dr. Ratnoff later found that the Hageman factor deficiency is autosomal recessive disorder, when examining several related people which had the deficiency. Paradoxically, pulmonary embolism contributed to Hageman's death after an occupational accident.
Last updated: 02-06-2005 06:50:14
Last updated: 05-03-2005 17:50:55
