Mouse Genome Informatics
hm1
    F13a1tm1Gdi/F13a1tm1Gdi
involves: 129P2/OlaHsd
Key:
phenotype observed in females
phenotype observed in males
N normal phenotype

Altered response to myocardial infraction in F13a1tm1Gdi/F13a1tm1Gdi mice

mortality/aging
• all die within 5 days after myocardial infarction from left ventricular rupture (J:121502)
• daily intravenous replenishment of F13a1 for 5 days restores survival (J:121502)

cardiovascular system
• observed in some male mice
• following myocardial infarction, intrathoracic hematoma is seen adjacent to the experimental anterolateral infarction (J:129358)
• intrauterine bleeding (J:86919)
• mutants subjected to coronary ligation die within 5 days and exhibit hemothorax, myocardial rupture of the left ventricular wall, and reduced inflammatory response in the infarct (J:121502)

reproductive system
• following myocardial infarction, intrathoracic hematoma is seen adjacent to the experimental anterolateral infarction (J:129358)
• intrauterine bleeding (J:86919)
• intrauterine fetal death due to placental hemorrhage

respiratory system
• observed in some male mice

homeostasis/metabolism
• mutants subjected to coronary ligation die within 5 days and exhibit hemothorax, myocardial rupture of the left ventricular wall, and reduced inflammatory response in the infarct (J:121502)

Mouse Models of Human Disease
OMIM IDRef(s)
Factor XIII, A Subunit, Deficiency of 613225 J:86919


Mouse Genome Informatics
hm2
    F13a1tm1Gdi/F13a1tm1Gdi
involves: 129P2/OlaHsd * CBA/Ca
Key:
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• ~30% of homozygotes die within 12 months after birth
• in these mice, bleeding time is only modestly prolonged by a factor of 2, suggesting that impaired clot integrity and stability, rather than delayed clot formation, may contrubute to increased mortality
• an increase in maternal lethality (~50%) is observed in female homozygotes during gestation

cardiovascular system
• homozygotes display spontaneous fatal hemorrhage, including hemothorax, hemoperitoneum, and subcutaneous bleeding

homeostasis/metabolism
N
• homozygotes show no significant differences in the extent of platelet aggregation induced by thrombin, ADP, or collagen relative to wild-type control mice
• in vitro, the global coagulation properties of blood from homozygous mutant mice, including activated partial thromboplastin time (aPTT), prothrombin time (PT) and thrombin time (TT) are indistinguishable from those of control mice
• in vitro, thrombelastography indicates impaired clot strength and rapid degradation of the mechanical properties of the clot in blood from mutant mice relative to 129 or CBA control mice
• however, replacement of mutant mice with human plasma F13A1 restores the mechanical properties of the clot to near normal values in a dose-dependent manner
• mean tail-tip bleeding time is significantly increased in homozygous mutant mice relative to 129 or CBA control mice
• however, replacement of mutant mice with human plasma F13A1 (200 U/kg i.v.) increases plasma activity to normal levels of ~135% and restores tail-tip bleeding time to within normal range

reproductive system
• female homozygotes are fertile; however, pregnancy is severely impaired

respiratory system

hematopoietic system
N
• homozygotes display no significant differences in platelet number, total white blood cell count, or erythrocyte number relative to wild-type mice

Mouse Models of Human Disease
OMIM IDRef(s)
Factor XIII, A Subunit, Deficiency of 613225 J:87293


Mouse Genome Informatics
ht3
    F13a1tm1Gdi/F13a1+
involves: 129P2/OlaHsd
Key:
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• all die within 5 days after myocardial infarction from left ventricular rupture

cardiovascular system
• mutants subjected to coronary ligation die within 5 days

homeostasis/metabolism
• mutants subjected to coronary ligation die within 5 days