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Factor IX
 
 
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Factor IX (or Christmas factor) is one of the 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 ....
s of the 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....
 system; it belongs to peptidase family S1. Deficiency of this protein causes hemophilia B
Haemophilia B

Haemophilia B is a coagulation disorder caused by a mutation of the Factor IX gene, leading to a deficiency of Factor IX. It is the least common form of haemophilia, rarer than haemophilia A....
. It was discovered after a young boy named Stephen Christmas
Stephen Christmas

Stephen Christmas was a 5 year old boy when he became the first patient described to have Christmas disease in 1952 by a group of British doctors....
 was found to be lacking this exact factor, leading to hemophilia, in 1952.

or IX is inactive unless activated by factor XI
Factor XI

Factor XI or plasma thromboplastin antecedent is the zymogen form of factor XIa, one of the enzymes of the coagulation. Like many other coagulation factors, it is a serine protease....
a (of the contact pathway) or factor VII
Factor VII

Factor VII is one of the central proteins in the coagulation. It is an enzyme of the serine protease class....
a (of the tissue factor pathway).






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Encyclopedia


Factor IX (or Christmas factor) is one of the 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 ....
s of the 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....
 system; it belongs to peptidase family S1. Deficiency of this protein causes hemophilia B
Haemophilia B

Haemophilia B is a coagulation disorder caused by a mutation of the Factor IX gene, leading to a deficiency of Factor IX. It is the least common form of haemophilia, rarer than haemophilia A....
. It was discovered after a young boy named Stephen Christmas
Stephen Christmas

Stephen Christmas was a 5 year old boy when he became the first patient described to have Christmas disease in 1952 by a group of British doctors....
 was found to be lacking this exact factor, leading to hemophilia, in 1952.

Physiology

Factor IX is inactive unless activated by factor XI
Factor XI

Factor XI or plasma thromboplastin antecedent is the zymogen form of factor XIa, one of the enzymes of the coagulation. Like many other coagulation factors, it is a serine protease....
a (of the contact pathway) or factor VII
Factor VII

Factor VII is one of the central proteins in the coagulation. It is an enzyme of the serine protease class....
a (of the tissue factor pathway). When activated into factor IXa, it acts by hydrolysing one arginine
Arginine

Arginine is an a-amino acid. The Optical isomerism is one of the 20 most common natural amino acids. Its codons are CGU, CGC, CGA, CGG, AGA, and AGG....
-isoleucine
Isoleucine

Isoleucine is an a-amino acid with the chemical formula HO2CCHCHCH2CH3. It is an essential amino acid, which means that humans cannot synthesize it, so it must be part of our diet....
 bond in factor X
Factor X

Factor X, also known by the eponym Stuart-Prower factor or as thrombokinase, is an enzyme of the coagulation. It is a serine protease ....
 to form factor Xa. It requires calcium
Calcium in biology

Calcium plays a pivotal role in the physiology and biochemistry of organisms and the cell . It plays an important role in signal transduction pathways, where it acts as a second messenger, in neurotransmitter release from neurons, contraction of all muscle cell types, and fertilization....
, membrane phospholipid
Phospholipid

File:Phospholipid.svgFile:phospholipid_structure.pngFile:Phosphatidyl-Choline.svgPhospholipids are a class of lipids and are a major component of all cell membranes....
s, 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....
 as 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...
s to do so.

Genetics

The gene
Gene

A gene is the basic unit of heredity in a living organism. All living things depend on genes. Genes hold the information to build and maintain their cell and pass genetic trait to offspring....
 for factor IX is located on the X chromosome
X chromosome

The X chromosome is one of the two sex determination system chromosomes in many animal species, including mammals . It is a part of the XY sex-determination system and X0 sex-determination system....
 (Xq27.1-q27.2). It was first cloned in 1982 by Kotoku Kurachi and Earl Davie.

Polly
Polly and Molly

Polly and Molly , two Domestic sheep, were the first mammals to have been successfully cloning from an adult somatic cell and to be transgenic animals at the same time....
, a transgenic cloned Poll Dorset
Poll Dorset

The Poll Dorset is a short wool, meat producing sheep that was developed in Australia during 1937-54 with the aim of breeding a true Dorset type sheep without horns....
 sheep carrying the gene for factor IX, was produced by Dr Ian Wilmut
Ian Wilmut

Sir Ian Wilmut, Order of the British Empire is an England embryologist and is currently one of the leaders of the Queen's Medical Research Institute at the University of Edinburgh....
 at the Roslin Institute
Roslin Institute

The Roslin Institute is a government research institute at, Roslin, Midlothian, a village in Midlothian, Scotland, that is sponsored by the Biotechnology and Biological Sciences Research Council....
 in 1997.

Role in disease

Deficiency of factor IX causes Christmas disease (hemophilia B). Over 100 mutations of factor IX have been described; some cause no symptoms, but many lead to a significant bleeding disorder.

Further reading