Jul 2011 A Systemic Inflammation Model: Unc93B1 with a D34A mutation


A Systemic Inflammation Model:
Unc93B1 with a D34A mutation

B6;129P2(129)-Unc93b1tm1.1(D34A)Kmiy RBRC04929
B6.129P2(129)-Unc93b1tm1.1(D34A)Kmiy RBRC04930
C.129P2(Cg)-Unc93b1tm1.1(D34A)Kmiy RBRC04931


Unc93B1 is a multitransmembrane endoplasmic reticulum (ER)-resident protein that controls the transportation of TLR7 and TLR9 from the ER to the endolysosomes. To clarify the role of Unc93B1 in controlling the balance of TLR7 and TLR9 in vivo, this strain has been generated by the targeted replacement of Unc93B1 with a mutated gene carrying D34A. Homozygous Unc93b1D34A/D34A mutant mice exhibited progressive splenomegaly, multilobular hepatic necrosis, glomerulonephritis, thrombocytopenia, and myeloproliferative disorders. Fifty percent of homozygous Unc93b1D34A/D34A mutant mice die within 6 months and 70% die within 1 year, probably because of acute hepatitis. This strain provides a unique model to study the molecular basis for inflammatory diseases in mice and humans. The original B6;129P2(129)-Unc93b1tm1.1(D34A)Kmiy strain has been crossed to C57BL/6NCrSlc and BALB/cCrSlc strains to establish congenic strains with different genetic backgrounds.


Other related strains : D1;129P2(Cg)-Unc93b1tm1.1(D34A)Kmiy RBRC04932
C3;129P2(Cg)-Unc93b1tm1.1(D34A)Kmiy RBRC04933
NOD;129P2(Cg)-Unc93b1tm1.1(D34A)Kmiy RBRC04934
Depositor : Dr. Kensuke Miyake, The Institute of Medical Science, The University of Tokyo
Reference : Ryutaro Fukui, Shin-Ichiroh Saitoh, Atsuo Kanno, Masahiro Onji, Takuma Shibata, Akihiko Ito, Morikazu Onji, Mitsuru Matsumoto, Shizuo Akira, Nobuaki Yoshida, and Kensuke Miyake. Unc93B1 restricts systemic lethal inflammation by orchestrating TLR7- and TLR9-trafficking. Immunity;35(1):69-81, 2011

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