TABLE 36-1
Components of the Hemostatic System
(continued)
Associated Disease(s)
Concentration
Tissue
PDB
Common
Hemorrhagic/
Disease
in Plasma 3
Source/Half-life (hr)
Codes for
SwissProt
Name1
Thrombotic Risk
Prevalence
mg/L
jfiM
Proteins 4
Designations 4
Anticoagulant Subsystem Components
Cofactor Protein Inactivation
Protein C
Protein C deficiency, purpura fulminans
Unknown
3-5
0.06
Liver
1PCU, 2PCT
PRTCJHUMAN
(-2-5% in
patients with
thromboembolic
disease)
Protein S
Protein S deficiency
Unknown
20-25
0.25
Liver,
PRTS_HUMAN
(-2-5% in
megakaryocytes,
patients with
thromboembolic
endothelial cells
disease)
Thrombomodulin
NA
Endothelium
1ADX, 1EGT,
TRBM_HUMAN
1FGD, 1FGE,
1TMR,
1ZAQ, 2ADX
Thrombin
None
THRBJHUMAN
Protein C Inhibitor (PAI-3)
-4
0.07
IPSPJTUMAN
Proteinase Inactivation
Antithrombin
Deficiency-homozygous-only with
1 per 5,000 to 1
125
2.3
Liver
61-92
1ANT, 1ATH,
ANT 3 JHUM AN
(antithrombin III)
defective heparin binding
per 350
(90% oc-form,
1ATT, 1BR8,
Heterozygous, defective proteinase
(heparin-
1 0
% /3-form)
binding (low risk), defective heparin
binding (any risk?)
dependent)
Heparin Cofactor II
No clear link to risk for thrombosis
1
per
1 0 0 , 0 0 0
80
1 . 2
Liver
60
HEP2_HUMAN
(Leuserpin)
heterozygous
Tissue Factor Pathway
Decreased in massive DIC, no change
Unknown
0.1
0.0025
Endothelium
1ADZ, 1TFX
TFPIJHUMAN
inhibitor (TFPI,
LACI, EPI)
in moderate DIC
a-1 Proteinase Inhibitor
None linked directly to hemostasis,
—
-1,300
23.5
Liver
1ATU, 1KCT,
A1AT_HUMAN
(oc-1
PI)
a
-1 antitrypsin
a-
2 Macroglobulin
primarily linked to emphysema
2,500
3.5
Liver
2PSI
A2MG_HUMAN
00
СЛ
(continued)
