{"id":5266,"date":"2013-06-27T11:19:09","date_gmt":"2013-06-27T02:19:09","guid":{"rendered":"http:\/\/mus.brc.riken.jp:7301\/?page_id=5266"},"modified":"2021-04-07T15:28:03","modified_gmt":"2021-04-07T06:28:03","slug":"jul_2011_mm","status":"publish","type":"page","link":"http:\/\/mus.brc.riken.jp\/ja\/mouse_of_month\/jul_2011_mm","title":{"rendered":"Jul 2011 A Systemic Inflammation Model: Unc93B1 with a D34A mutation<\/em>"},"content":{"rendered":"

\"mouse_of_month_201107\"<\/a><\/p>\n\n\n\n
\n

A Systemic Inflammation Model:
\nUnc93B1 with a D34A mutation<\/em><\/h2>\n
B6;129P2(129)-Unc93b1tm1.1(D34A)Kmiy<\/sup>\u00a0RBRC04929<\/a>
\nB6.129P2(129)-Unc93b1tm1.1(D34A)Kmiy<\/sup>\u00a0RBRC04930<\/a>
\nC.129P2(Cg)-Unc93b1tm1.1(D34A)Kmiy<\/sup>\u00a0RBRC04931<\/a><\/h5>\n
\"mn1107_0101\"<\/h5>\n<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n

 <\/p>\n

Unc93B1 is a multitransmembrane endoplasmic reticulum (ER)-resident protein that controls the transportation of\u00a0TLR7<\/a>\u00a0and\u00a0TLR9<\/a>\u00a0from the ER to the endolysosomes. To clarify the role of Unc93B1 in controlling the balance of\u00a0TLR7<\/a>\u00a0and\u00a0TLR9<\/a>\u00a0in vivo<\/em>, this strain has been generated by the targeted replacement of Unc93B1 with a mutated gene carrying D34A. Homozygous\u00a0Unc93b1D34A\/D34A<\/sup><\/em>\u00a0mutant mice exhibited progressive splenomegaly, multilobular hepatic necrosis, glomerulonephritis, thrombocytopenia, and myeloproliferative disorders. Fifty percent of homozygous\u00a0Unc93b1D34A\/D34A<\/sup><\/em>\u00a0mutant 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<\/sup><\/em>\u00a0strain has been crossed to C57BL\/6NCrSlc and BALB\/cCrSlc strains to establish congenic strains with different genetic backgrounds.<\/strong>\n<\/div>\n

 <\/p>\n\n\n\n\n\n
Other related strains<\/td>\n:<\/td>\nD1;129P2(Cg)-Unc93b1tm1.1(D34A)Kmiy<\/sup>\u00a0RBRC04932<\/a>
\nC3;129P2(Cg)-Unc93b1tm1.1(D34A)Kmiy<\/sup>\u00a0RBRC04933<\/a>
\nNOD;129P2(Cg)-Unc93b1tm1.1(D34A)Kmiy<\/sup>\u00a0RBRC04934<\/a><\/td>\n<\/tr>\n
Depositor<\/td>\n:<\/td>\nDr. Kensuke Miyake, The Institute of Medical Science, The University of Tokyo<\/td>\n<\/tr>\n
Reference<\/td>\n:<\/td>\nRyutaro 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.\u00a0Immunity;<\/em>35(1):69-81, 2011<\/a><\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n","protected":false},"excerpt":{"rendered":"

A Systemic Inflammation Model: Unc93B1 with a D34A mutation B6;129P2(129)-Unc93b1tm1.1(D34A)Kmiy\u00a0RBRC04929 B6.129P2(129)-Unc93b1tm1.1(D34A)Kmiy\u00a0RBRC04930 C.129P2(Cg)-Unc93b1tm1.1(D34A)Kmiy\u00a0RBRC04931 & […]<\/p>\n","protected":false},"author":8,"featured_media":0,"parent":198,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","meta":{"_seopress_titles_title":"","_seopress_titles_desc":"","_seopress_robots_index":"","_seopress_robots_follow":"","_seopress_robots_imageindex":"","_seopress_robots_snippet":"","_seopress_robots_primary_cat":"","_seopress_robots_breadcrumbs":"","_seopress_robots_freeze_modified_date":"","_seopress_robots_custom_modified_date":"","_seopress_robots_canonical":"","_seopress_social_fb_title":"","_seopress_social_fb_desc":"","_seopress_social_fb_img":"","_seopress_social_fb_img_attachment_id":0,"_seopress_social_fb_img_width":0,"_seopress_social_fb_img_height":0,"_seopress_social_twitter_title":"","_seopress_social_twitter_desc":"","_seopress_social_twitter_img":"","_seopress_social_twitter_img_attachment_id":0,"_seopress_social_twitter_img_width":0,"_seopress_social_twitter_img_height":0,"_seopress_redirections_value":"","_seopress_redirections_enabled":"","_seopress_redirections_enabled_regex":"","_seopress_redirections_logged_status":"","_seopress_redirections_param":"","_seopress_redirections_type":301,"_seopress_analysis_target_kw":"","footnotes":"","_wp_rev_ctl_limit":""},"class_list":["post-5266","page","type-page","status-publish","hentry"],"_links":{"self":[{"href":"http:\/\/mus.brc.riken.jp\/ja\/wp-json\/wp\/v2\/pages\/5266","targetHints":{"allow":["GET"]}}],"collection":[{"href":"http:\/\/mus.brc.riken.jp\/ja\/wp-json\/wp\/v2\/pages"}],"about":[{"href":"http:\/\/mus.brc.riken.jp\/ja\/wp-json\/wp\/v2\/types\/page"}],"author":[{"embeddable":true,"href":"http:\/\/mus.brc.riken.jp\/ja\/wp-json\/wp\/v2\/users\/8"}],"replies":[{"embeddable":true,"href":"http:\/\/mus.brc.riken.jp\/ja\/wp-json\/wp\/v2\/comments?post=5266"}],"version-history":[{"count":9,"href":"http:\/\/mus.brc.riken.jp\/ja\/wp-json\/wp\/v2\/pages\/5266\/revisions"}],"predecessor-version":[{"id":18197,"href":"http:\/\/mus.brc.riken.jp\/ja\/wp-json\/wp\/v2\/pages\/5266\/revisions\/18197"}],"up":[{"embeddable":true,"href":"http:\/\/mus.brc.riken.jp\/ja\/wp-json\/wp\/v2\/pages\/198"}],"wp:attachment":[{"href":"http:\/\/mus.brc.riken.jp\/ja\/wp-json\/wp\/v2\/media?parent=5266"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}