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| Primary information |
|---|
| ID | 14884 |
| Therapeutic ID | Th1549 |
| Protein Name | Factor XIII (human) |
| Sequence | >Th1549_Factor_XIII_(human)
GVNLQEFLNVTSVHLFKERWDTNKVDHHTDKYENNKLIVRRGQSFYVQIDFSRPYDPRRDLFRVEYVIGRYPQENKGTYIPVPIVSELQSGKWGAKIVMREDRSVRLSIQSSPKCIVGKFRMYVAVWTPYGVLRTSRNPETDTYILFNPWCEDDAVYLDNEKEREEYVLNDIGVIFYGEVNDIKTRSWSYGQFEDGILDTCLYVMDRAQMDLSGRGNPIKVSRVGSAMVNAKDDEGVLVGSWDNIYAYGVPPSAWTGSVDILLEYRSSENPVRYGQCWVFAGVFNTFLRCLGIPARIVTNYFSAHDNDANLQMDIFLEEDGNVNSKLTKDSVWNYHCWNEAWMTRPDLPVGFGGWQAVDSTPQENSDGMYRCGPASVQAIKHGHVCFQFDAPFVFAEVNSDLIYITAKKDGTHVVENVDATHIGKLIVTKQIGGDGMMDITDTYKFQEGQEEERLALETALMYGAKKPLNTEGVMKSRSNVDMDFEVENAVLGKDFKLSITFRNNSHNRYTITAYLSANITFYTGVPKAEFKKETFDVTLEPLSFKKEAVLIQAGEYMGQLLEQASLHFFVTARINETRDVLAKQKSTVLTIPEIIIKVRGTQVVGSDMTVTVEFTNPLKETLRNVWVHLDGPGVTRPMKKMFREIRPNSTVQWEEVCRPWVSGHRKLIASMSSDSLRHVYGELDVQIQRRPSM
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| Molecular Weight | NA |
| Chemical Formula | NA |
| Isoelectric Point | NA |
| Hydrophobicity | NA |
| Melting point | NA |
| Half-life | 6.6 ±2.29 days [FDA Label], 6.6 days [A32364] |
| Description | Factor XIII (human) is a heat-treated, lyophilized concentrate of coagulation factor XIII, an endogenous enzyme responsible for the crosslinking of fibrin and an essential component of the coagulation cascade [FDA Label]. For people with congenital deficiency or mutation of Factor XIII, a rare bleeding disorder, exogenous replacement of this key coagulation factor is essential for management and prevention of bleeding episodes. Also known as Fibrin Stabilizing Factor (FSF), Factor XIII is an endogenously produced coagulation factor and the final enzyme within the blood coagulation cascade. Within the body, FXIII circulates as a heterotetramer composed of 2 A-subunits and 2 B-subunits (A2B2)[A32363]. When activated by thrombin at the site of injury, the FXIII pro-enzyme is cleaved resulting in activation of the catalytic A-subunit and dissociation from its carrier B-subunit. As a result, the active transglutaminase from subunit A cross-links fibrin and other proteins resulting in increased mechanical strength and resistance to fibrinolysis of the fibrin clot. This contributes to enhanced platelet and clot adhesion to injured tissue, thereby improving blood coagulation and maintenance of hemostasis [A18581]. Other drug products with similar structure and function to Factor XIII (human) include [DB09310], which is a recombinant form of the A subunit of human coagulation factor XIII. Compared to Factor XIII (human), which is purified from pooled human plasma, [DB09310] is produced through recombinant DNA technology where the target protein is grown in yeast and then isolated [FDA Label]. Factor XIII (Human), available as the commercially available product Corifact, is approved by the Food and Drug Administration for routine prophylactic treatment and peri-operative management of surgical bleeding in adult and pediatric patients with congenital FXIII deficiency [FDA Label]. As the half-life of endogenous Factor XIII is long (5-11 days), prophylactic therapy with the replacement of FXIII can be given every 4-6 to maintain hemostasis[A32363]. |
| Indication/Disease | Factor XIII (Human), available as the commercially available product Corifact, is approved by the Food and Drug Administration for routine prophylactic treatment and peri-operative management of surgical bleeding in adult and pediatric patients with congenital FXIII deficiency [FDA Label]. |
| Pharmacodynamics | NA |
| Mechanism of Action | Also known as Fibrin Stabilizing Factor (FSF), Factor XIII is an endogenously produced coagulation factor and the final enzyme within the blood coagulation cascade. Within the body, FXIII circulates as a heterotetramer composed of 2 A-subunits and 2 B-subunits (A2B2)[A32363]. When activated by thrombin at the site of injury, the FXIII pro-enzyme is cleaved resulting in activation of the catalytic A-subunit and dissociation from its carrier B-subunit. As a result, the active transglutaminase from subunit A cross-links fibrin and other proteins resulting in increased mechanical strength and resistance to fibrinolysis of the fibrin clot. This contributes to enhanced platelet and clot adhesion to injured tissue, thereby improving blood coagulation and maintenance of hemostasis [A18581]. Exogenous replacement of Factor XIII is a cornerstone of treatment for bleeding associated with congenital Factor XIII deficiency. |
| Toxicity | Corifact was studied in an acute toxicity study in mice and rats at doses up to 3550 units per kg and 1420 units per kg, respectively. Repeat dose toxicity was studied in rats at daily doses up to 350 units per kg for a period of 14 days. No signs of toxicity were observed in the single dose and repeat dose studies. A local tolerance study in rabbits demonstrated no clinical or histopathological changes at the injection site after intravenous, intra-arterial or para-venous administration of Corifact. A thrombogenicity test was performed in rabbits at doses up to 350 units per kg. Corifact showed no thrombogenic potential at the doses tested. |
| Metabolism | NA |
| Absorption | Tmax = 1.7 ±1.44 hr [FDA Label] Tmax = 1.72 hr [A32364] Cmax = 0.9 ±0.20 units/mL (peak concentration at steady state) [FDA Label] Cmax = 87.7% (peak concentration at steady state) [A32364] |
| Vss = 51.1 mL/kg (volume of distribution at steady state) [A32364] |
| Clearance | 0.25 ±0.09 mL/hr/kg [FDA Label] 0.25 mL/hr/kg [A32364] |
| Categories | Aminoacyltransferases |
| Patents Number | NA |
| Date of Issue | NA |
| Date of Expiry | NA |
| Drug Interaction | NA |
| Target | NA |
| Brand Name | Corifact 250 |
| Company | Csl Behring |
| Brand Description | Csl Behring |
| Prescribed For | Intravenous |
| Chemical Name | 250 unit |
| Formulation | CORIFACT is contraindicated in patients with known anaphylactic or severe systemic reactions to human plasma-derived products |
| Physical Appearance | hives, difficulty breathing, swelling of your face, lips, tongue, or throat, lightheadedness, new or worsening bleeding episodes, unusual bruising, sudden numbness or weakness (especially on one side of the body), sudden severe headache, slurred speech, problems with vision or balance, chest pain, sudden cough, wheezing, rapid breathing, coughing up blood, and pain, swelling, warmth, or redness in one or both legs |
| Route of Administration | Corifact, Factor XIII Concentrate (Human), is a heat-treated, lyophilized concentrate of coagulation factor XIII for reconstitution for intravenous use. Corifact (FXIII) consists of two A-subunits and two B-subunits, and is made from pooled human plasma. Each vial contains 1000-1600 units FXIII, 120 to 200 mg human albumin, 120 to 320 mg total protein, 80 to 120 mg glucose and 140 to 220 mg sodium chloride. Sodium hydroxide may have been used to adjust the pH. |
| Recommended Dosage | used as a prophylaxis and perioperative management of surgical bleeding in patient with Congenital Factor XIII Deficiency. |
| Contraindication | NA |
| Side Effects | NA |
| Useful Link 1 | Link |
| Useful Link 2 | Link |
| Remarks | NA |