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Protein C



 


Protein C is a major physiological anticoagulant. It is a vitamin K-dependent serine protease enzyme (EC 3.4.21.69) that is 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 should not 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.[1]

See: detailed diagram of Blood Coagulation (Thrombin) and Protein C Pathways


Additional recommended knowledge

Contents

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 is 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.[3]

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).


Protein C (inactivator of coagulation factors Va and VIIIa)
PDB rendering based on 1aut.
Available structures: 1aut, 1lqv
Identifiers
Symbol(s) PROC; PROC1
External IDs OMIM: 176860 MGI: 97771 Homologene: 37288
RNA expression pattern

More reference expression data

Orthologs
Human Mouse
Entrez 5624 19123
Ensembl ENSG00000115718 ENSMUSG00000024386
Uniprot P04070 P33587
Refseq NM_000312 (mRNA)
NP_000303 (protein)
XM_984063 (mRNA)
XP_989157 (protein)
Location Chr 2: 127.89 - 127.9 Mb Chr 18: 32.27 - 32.28 Mb
Pubmed search [1] [2]

See also

  • Activated protein C resistance
  • Protein C inhibitor
  • Hemostasis

Notes

  1. ^ Thrombosis, Blood Coagulation and the Antithrombotic Protein C Pathway - John H. Griffin, TSRI
  2. ^ Activated protein C resistance
  3. ^ Blood review by Mosnier, Zlokovic and Griffin 2006 ePub



 
This article is licensed under the GNU Free Documentation License. It uses material from the Wikipedia article "Protein_C". A list of authors is available in Wikipedia.
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