Everything about Protein C totally explained
Protein C is a major physiological
anticoagulant. It is a
vitamin K-dependent
serine protease enzyme that's activated by
thrombin into
activated protein C (APC). The activated form (with
protein S and phospholipid as a
cofactor) degrades
Factor Va and
Factor VIIIa. It shouldn't be confused with
C peptide or
c-reactive protein or
protein kinase C.
The protein C pathway’s key enzyme, activated protein C, provides physiologic antithrombotic activity and exhibits both
anti-inflammatory and anti-
apoptotic activities. Its actions are related to development of
thrombosis and
ischemic stroke. The protein C pathway of the coagulation of the blood involves the influences of
lipids and
lipoproteins and the study of the strong epidemiologic association between
hyperlipidemia and
hypercoagulability.
» See: detailed diagram of
Role in disease
Protein C deficiency is a rare
genetic disorder that predisposes to
venous thrombosis and
habitual abortion. If
homozygous, this presents with a form of
disseminated intravascular coagulation in newborns termed
purpura fulminans; it's treated by replacing the defective protein C.
Activated protein C resistance is the inability of protein C to cleave factors V and/or VIII. This may be hereditary or acquired. The best known and most common hereditary form is
Factor V Leiden. Acquired forms occur in the presence of elevated Factor VIII concentrations.
Warfarin necrosis is acquired protein C deficiency due to treatment with the vitamin K inhibitor anticoagulant
warfarin. In initial stages of action, inhibition of protein C may be stronger than inhibition of the vitamin K-dependent coagulation factors (II, VII, IX and X), leading to paradoxical activation of coagulation and
necrosis of skin areas.
HDL and the effects of activated protein C (APC) on cells is very important.
Pharmacology
Drotrecogin alpha(activated) is
recombinant activated protein C from Ely Lilly Co, USA. It is used in the treatment of
severe sepsis,
septic shock and
disseminated intravascular coagulation.
Genetics
The
PROC gene is located on the second
chromosome (2q13-q14).
,
| Name = Protein C (inactivator of coagulation factors Va and VIIIa)
| HGNCid = 9451
| Symbol = PROC
| AltSymbols =; PROC1
| OMIM = 176860
| ECnumber =
| Homologene = 37288
| MGIid = 97771
| GeneAtlas_image1 = PBB_GE_PROC_206259_at_tn.png
| Function =
| Component =
| Process =
| Orthologs =
}}
Further Information
Get more info on 'Protein C'.
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