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Rabbit Anti-Factor XI light chain/FITC Conjugated antibody
background:
This gene encodes coagulation factor XI of the blood coagulation cascade. This protein is present in plasma as a zymogen, which is a unique plasma coagulation enzyme because it exists as a homodimer consisting of two identical polypeptide chains linked by disulfide bonds. During activation of the plasma factor XI, an internal peptide bond is cleaved by factor XIIa (or XII) in each of the two chains, resulting in activated factor XIa, a serine protease composed of two heavy and two light chains held together by disulfide bonds. This activated plasma factor XI triggers the middle phase of the intrisic pathway of blood coagulation by activating factor IX. Defects in this factor lead to Rosenthal syndrome, a blood coagulation abnormality. [provided by RefSeq, Jul 2008].
Function:
Factor XI triggers the middle phase of the intrinsic pathway of blood coagulation by activating factor IX.
Subunit:
Homodimer; disulfide-linked. Forms a heterodimer with SERPINA5. After activation the heavy and light chains are also linked by a disulfide bond.
Subcellular Location:
Secreted.
Tissue Specificity:
Isoform 2 is produced by platelets and megakaryocytes but absent from other blood cells.
Post-translational modifications:
Activated by factor XIIa (or XII), which cleaves each polypeptide after Arg-387 into the light chain, which contains the active site, and the heavy chain, which associates with high molecular weight (HMW) kininogen.
DISEASE:
Defects in F11 are the cause of factor XI deficiency (FA11D) [MIM:612416]; also known as plasma thromboplastin antecedent deficiency or Rosenthal syndrome. It is a hemorrhagic disease characterized by reduced levels and activity of factor XI resulting in moderate bleeding symptoms, usually occurring after trauma or surgery. Patients usually do not present spontaneous bleeding but women can present with menorrhagia. Hemorrhages are usually moderate.
Similarity:
Belongs to the peptidase S1 family. Plasma kallikrein subfamily.
Contains 4 apple domains.
Contains 1 peptidase S1 domain.
Database links:
Entrez Gene: 2160 Human
Entrez Gene: 290757 Rat
Omim: 26980 Human
SwissProt: P03951 Human
Unigene: 1430 Human
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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