Strain Detail: ADAMTS13^L/L mice

ADAMTS13^L/Lis established to introduceAdamts13gene derived from 129/Sv strain into C57BL/6 strain by backcrossing. These mice express full-length ADAMTS13 (ADAMTS13L), whereas wildtype C57BL/6 mice express C-terminal domain deleted ADAMTS13 (ADAMTS13S) by the insertion of retrotransposon intoAdamts13gene.

It's known that ADAMTS13 is a plasma protease which cleaves von Willebrand factor (VWF) specifically and deletion of ADAMTS13 activity is caused thrombotic thrombocytopenic purpura (TTP) in human.

ADAMTS13^S/Smice (nbio135) which are introduced Adamts13 gene derived from C57BL/6 strain into 129/Sv strain by backcrossing and express C-terminal domain deleted ADAMTS13 (ADAMTS13S) is also available.

1. Banno F, Chauhan AK, Kokame K, Yang J, Miyata S, Wagner DD, Miyata T: The distal carboxyl-terminal domains of ADAMTS13 are required for regulation of in vivo thrombus formation.Blood, 113(21):5323-5329 (2009). [PMID:19109562]
2. Banno F, Kaminaka K, Soejima K, Kokame K, Miyata T: Identification of strain-specific variants of mouseAdamts13gene encoding von Willebrand factor-cleaving protease.J Biol Chem, 279, 30896-30903 (2004). [PMID:15136581]