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Protein C
 
 
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Protein C is a major physiological anticoagulant
Anticoagulant

An anticoagulant is a substance that prevents blood coagulation; that is, it stops blood from clotting. A group of pharmaceuticals called anticoagulants can be used in vivo as a medication for thrombosis disorders....
. It is a vitamin K
Vitamin K

Vitamin K denotes a group of lipophilic, hydrophobic vitamins that are needed for the posttranslational modification of certain proteins, mostly required for blood coagulation....
-dependent serine protease
Serine protease

Serine proteases or serine endopeptidases are proteases in which one of the amino acids at the active site is serine.They are found in both single-cell and complex organisms, in both cells with nuclei and without nuclei ....
 enzyme
Enzyme

Enzymes are biomolecules that catalysis chemical reactions. Almost all enzymes are proteins. In enzymatic reactions, the molecules at the beginning of the process are called Substrate , and the enzyme converts them into different molecules, the products....
  that is activated by thrombin
Thrombin

Thrombin is a coagulation protein that has many effects in the coagulation#The_coagulation_cascade. It is a serine protease that converts soluble fibrinogen into insoluble strands of fibrin, as well as catalyzing many other coagulation-related reactions....
 into activated protein C (APC). The activated form (with protein S
Protein S

Protein S is a vitamin K-dependent plasma glycoprotein synthesized in the liver. In the circulation, Protein S exists in two forms: a free form and a complex form bound to complement system protein C4b....
 and phospholipid as a cofactor
Cofactor

Cofactor may refer to any of the following:* Cofactor The signed minor of a matrix* Minor as an alternative name for the determinant of a smaller Matrix than that which it describes...
) degrades Factor V
Factor V

Factor V is a protein of the coagulation system, rarely referred to as proaccelerin or labile factor. In contrast to most other coagulation factors, it is not enzymatically active but functions as a cofactor....
a and Factor VIII
Factor VIII

Factor VIII is an essential thrombusting factor. In humans, Factor VIII is encoded by the F8 gene. Defects in this gene results in hemophilia A, a common Dominance_#Recessive_trait X-linked coagulation disorder....
a. It should not be confused with C peptide or c-reactive protein
C-reactive protein

C-reactive protein is a protein found in the blood in response to inflammation .CRP is produced by the liver and by fat cells . It is a member of the pentraxin family of proteins....
 or protein kinase C
Protein kinase C

Protein kinase C is a family of protein kinases consisting of ~10 isozymes. They are divided into three subfamilies, based on their second messenger requirements: conventional , novel, and atypical....
.

The protein C pathway’s key enzyme, activated protein C, provides physiologic antithrombotic activity and exhibits both anti-inflammatory
Anti-inflammatory

Anti-inflammatory refers to the property of a substance or treatment that reduces inflammation. Anti-inflammatory drugs make up about half of analgesics, remedying pain by reducing inflammation as opposed to opioids which affect the brain....
 and anti-apoptotic activities.






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Encyclopedia


Protein C is a major physiological anticoagulant
Anticoagulant

An anticoagulant is a substance that prevents blood coagulation; that is, it stops blood from clotting. A group of pharmaceuticals called anticoagulants can be used in vivo as a medication for thrombosis disorders....
. It is a vitamin K
Vitamin K

Vitamin K denotes a group of lipophilic, hydrophobic vitamins that are needed for the posttranslational modification of certain proteins, mostly required for blood coagulation....
-dependent serine protease
Serine protease

Serine proteases or serine endopeptidases are proteases in which one of the amino acids at the active site is serine.They are found in both single-cell and complex organisms, in both cells with nuclei and without nuclei ....
 enzyme
Enzyme

Enzymes are biomolecules that catalysis chemical reactions. Almost all enzymes are proteins. In enzymatic reactions, the molecules at the beginning of the process are called Substrate , and the enzyme converts them into different molecules, the products....
  that is activated by thrombin
Thrombin

Thrombin is a coagulation protein that has many effects in the coagulation#The_coagulation_cascade. It is a serine protease that converts soluble fibrinogen into insoluble strands of fibrin, as well as catalyzing many other coagulation-related reactions....
 into activated protein C (APC). The activated form (with protein S
Protein S

Protein S is a vitamin K-dependent plasma glycoprotein synthesized in the liver. In the circulation, Protein S exists in two forms: a free form and a complex form bound to complement system protein C4b....
 and phospholipid as a cofactor
Cofactor

Cofactor may refer to any of the following:* Cofactor The signed minor of a matrix* Minor as an alternative name for the determinant of a smaller Matrix than that which it describes...
) degrades Factor V
Factor V

Factor V is a protein of the coagulation system, rarely referred to as proaccelerin or labile factor. In contrast to most other coagulation factors, it is not enzymatically active but functions as a cofactor....
a and Factor VIII
Factor VIII

Factor VIII is an essential thrombusting factor. In humans, Factor VIII is encoded by the F8 gene. Defects in this gene results in hemophilia A, a common Dominance_#Recessive_trait X-linked coagulation disorder....
a. It should not be confused with C peptide or c-reactive protein
C-reactive protein

C-reactive protein is a protein found in the blood in response to inflammation .CRP is produced by the liver and by fat cells . It is a member of the pentraxin family of proteins....
 or protein kinase C
Protein kinase C

Protein kinase C is a family of protein kinases consisting of ~10 isozymes. They are divided into three subfamilies, based on their second messenger requirements: conventional , novel, and atypical....
.

The protein C pathway’s key enzyme, activated protein C, provides physiologic antithrombotic activity and exhibits both anti-inflammatory
Anti-inflammatory

Anti-inflammatory refers to the property of a substance or treatment that reduces inflammation. Anti-inflammatory drugs make up about half of analgesics, remedying pain by reducing inflammation as opposed to opioids which affect the brain....
 and anti-apoptotic activities. Its actions are related to development of thrombosis
Thrombosis

Thrombosis is the formation of a blood clot inside a blood vessel, obstructing the flow of blood through the circulatory system. When a blood vessel is injured, the body uses platelets and fibrin to form a blood clot, because the first step in repairing it is to prevent loss of blood....
 and ischemic stroke. The protein C pathway of the coagulation of the blood involves the influences of lipid
Lipid

Lipids are broadly defined as any fat-soluble , naturally-occurring molecule, such as fats, oils, waxes, cholesterol, sterols, fat-soluble vitamins , monoglycerides, diglycerides, phospholipids, and others....
s and lipoprotein
Lipoprotein

A lipoprotein is a biochemistry assembly that contains both proteins and lipids. The lipids or their derivatives may be covalently or non-covalently bound to the proteins....
s and the study of the strong epidemiologic association between hyperlipidemia
Hyperlipidemia

Hyperlipidemia, hyperlipoproteinemia or dyslipidemia is the presence of raised or abnormal levels of lipids and/or lipoproteins in the blood....
 and hypercoagulability.

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


Role in disease

Protein C Anticoagulant
Protein C deficiency
Protein C deficiency

Protein C deficiency is a rare genetic trait that predisposes to thrombosis.The first person ever to be prescribed with this is Jane angwin - giving birth to Helena Ajne Angwin, Nurse - Helena Daily....
 is a rare genetic disorder
Genetic disorder

A genetic disorder is an illness caused by abnormalities in genes or chromosomes. While some diseases, such as cancer, are due in part to a genetic disorders, they can also be caused by Environment factors....
 that predisposes to venous thrombosis
Thrombosis

Thrombosis is the formation of a blood clot inside a blood vessel, obstructing the flow of blood through the circulatory system. When a blood vessel is injured, the body uses platelets and fibrin to form a blood clot, because the first step in repairing it is to prevent loss of blood....
 and habitual abortion
Habitual abortion

Habitual abortion, recurrent miscarriage or recurrent pregnancy loss is the occurrence of repeated pregnancy that end in miscarriage of the fetus, usually before 20 weeks of gestation....
. If homozygous, this presents with a form of disseminated intravascular coagulation
Disseminated intravascular coagulation

Disseminated intravascular coagulation , also known as consumptive coagulopathy, is a pathological activation of coagulation mechanisms that happens in response to a variety of diseases....
 in newborns termed purpura fulminans
Purpura fulminans

Purpura fulminans is a haemorrhagic condition usually associated with sepsis or previous infection. It occurs mainly in babies and small children....
; it is treated by replacing the defective protein C.

Activated protein C resistance
Activated protein C resistance

Activated protein C resistance is a hemostasis characterized by a poor anticoagulant response to activated protein C . This results in an increased risk of venous thrombosis, which can cause myocardial infarction, strokes, and other problems with circulation....
 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
Factor V Leiden

Factor V Leiden is the name given to a variant of human factor V that causes a thrombophilia disorder. In this disorder the Leiden variant of factor V, cannot be inactivated by activated protein C....
. Acquired forms occur in the presence of elevated Factor VIII concentrations.

Warfarin necrosis
Warfarin necrosis

Warfarin-induced skin necrosis is a condition in which skin and subcutaneous tissue necrosis occurs due to acquired protein C deficiency following treatment with Vitamin K antagonist anticoagulants ....
 is acquired protein C deficiency due to treatment with the vitamin K inhibitor anticoagulant warfarin
Warfarin

Warfarin is an anticoagulant. It was initially marketed as a pesticide against rats and mice, and is still popular for this purpose, although more potent poisons such as brodifacoum have since been developed....
. 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
Necrosis

Necrosis is the name given to premature death of cell s and living biological tissue. Necrosis is caused by external factors, such as infection, toxins, or trauma....
 of skin areas.

HDL
High density lipoprotein

High-density lipoproteins is one of the 5 major groups of lipoproteins which enable lipids like cholesterol and triglycerides to be transported within the water based blood stream....
 and the effects of activated protein C (APC) on cells is very important.

Pharmacology

Drotrecogin alpha(activated) is recombinant
Recombinant

Recombinant may refer to :* Recombinant DNA - a form of artificial DNA* , offers healthcare providers and academic medical centers proven, leading-edge data warehousing and clinical intelligence solutions to deliver higher quality outcomes, accelerate personalized medicine, and lower costs....
 activated protein C from Eli Lilly Co, USA. It is used in the treatment of severe sepsis
Sepsis

Sepsis, is a serious medicine condition characterized by a whole-body Inflammation state and the presence of a known or suspected infection., septic shock
Septic shock

Septic shock is a serious medicine condition caused by decreased tissue perfusion and oxygen delivery as a result of infection and sepsis, though the microbe may be systemic or localized to a particular site....
 and disseminated intravascular coagulation
Disseminated intravascular coagulation

Disseminated intravascular coagulation , also known as consumptive coagulopathy, is a pathological activation of coagulation mechanisms that happens in response to a variety of diseases....
.

Genetics

The PROC gene is located on the second chromosome
Chromosome

A chromosome is an organized structure of DNA and protein that is found in Cell . A chromosome is a single piece of DNA that contains many genes, regulatory sequence and other genetic sequence....
 (2q13-q14).


See also

  • Activated protein C resistance
    Activated protein C resistance

    Activated protein C resistance is a hemostasis characterized by a poor anticoagulant response to activated protein C . This results in an increased risk of venous thrombosis, which can cause myocardial infarction, strokes, and other problems with circulation....
  • Protein C inhibitor
    Protein C inhibitor

    Protein C inhibitor is a serpin which limits the expression of protein C.See also* SerpinFurther readingExternal links...
  • Hemostasis
    Hemostasis

    Hemostasis is a complex process which causes the bleeding process to stop. Most time this includes the changing of blood from a fluid to a solid state....
  • Thrombosis
    Thrombosis

    Thrombosis is the formation of a blood clot inside a blood vessel, obstructing the flow of blood through the circulatory system. When a blood vessel is injured, the body uses platelets and fibrin to form a blood clot, because the first step in repairing it is to prevent loss of blood....
  • Coagulation
    Coagulation

    Coagulation is a complex process by which blood forms clots. It is an important part of hemostasis , wherein a damaged blood vessel wall is covered by a platelet and fibrin-containing clot to stop hemorrhage and begin repair of the damaged vessel....
  • Anticoagulant
    Anticoagulant

    An anticoagulant is a substance that prevents blood coagulation; that is, it stops blood from clotting. A group of pharmaceuticals called anticoagulants can be used in vivo as a medication for thrombosis disorders....
    s


External links

  • , Gruber A, Marzec UM, Bush L, Di Cera E, Fernandez JA, Berny MA, Tucker EI, McCarty OJ, Griffin JH, Hanson SR., Blood (2007) - 310 articles by Griffin, et al. are represented at this link* - John H. Griffin, retired, TSRI, La Jolla, California
  • Diagram of