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Rat Models of Thrombotic Thrombocytopenic Purpura Reveal Crucial Role of Placental ADAMTS13 in Perinatal Survival - PubMed

6 hours ago
  • #Thrombotic Thrombocytopenic Purpura
  • #ADAMTS13
  • #Rat Models
  • Rat model of TTP created by CRISPR/Cas9 deletion of ADAMTS13 metalloprotease.
  • KO rats showed severe ADAMTS13 deficiency, increased VWF multimers, and spontaneous thrombocytopenia.
  • Elevated lactate dehydrogenase, urea nitrogen, and creatinine levels in KO rats.
  • Microvascular thrombi found in major organs of KO rats.
  • Pregnancy/postpartum did worsen thrombocytopenia but increased mortality risk in KO females bred with KO males.
  • KO females bred with KO males produced fewer live offspring compared to those bred with WT/Het males.
  • Model recapitulates congenital TTP and highlights fetal-placental ADAMTS13 role in perinatal survival.