Strain Data Sheet

RBRC11142

Strain Information

Image
BRC No.RBRC11142
TypeRecombinant Inbred
SpeciesMus musculus
Strain nameMXH36/Mo-lpr/lpr
Former Common nameMXH36/Mo/lpr
H-2 Haplotype
ES Cell line
Background strain
Appearance
Strain development1996年から東北大学大学院医学系研究科附属動物実験施設（東北大学動物実験施設）において、MRL/MpJ-lpr/lpr(MRL/lpr)マウスの雌とC3H/HeJ-lpr/lpr(C3H/lpr)マウスの雄を交配させて得られたF1同士を交配させ、当初、54系統のリコンビナント近交系マウス（MXH1/lpr～MXH54/lpr）マウスの樹立を試みましたが、2020年7月の時点で、8系統のRIマウスが維持されており、F86～F115世代になっております（F86の1系統のみ途中凍結胚から個体復元しました）。MXH36/Mo-lpr/lpr（MXH36/ lpr）はこのリコンビナント近交系マウスの中の1系統。Y染色体は、C3H/lpr由来。 2020年7月1日の時点でF114世代を維持している。リンパ節腫脹に関連するとされているlpr遺伝子を有し、全身のリンパ節腫脹を示す。血清学的所見としてdsDNA-IgGやIgG-RFが高値を示すが、MRL/lprマウスにみられるような致死性の腎炎は発症しない。　　第1～19染色体およびX染色体の対立遺伝子が、MRLあるいはC3H由来でホモ接合体であることを示すSDP（strain　distribution pattern）表はマイクロサテライトマーカーを用いて解析し作製済である。
Strain descriptionMRL/lprマウスは、4～5か月齢になると、リンパ節腫脹を来し、同一個体内で、全身性エリテマトーデスでみられる糸球体腎炎、結節性多発動脈炎でみられる血管炎、関節リウマチでみられる関節炎、およびシェーグレン症候群でみられる唾液腺炎に類似した病変を自然発症するマウスで膠原病の疾患モデルとして世界中で研究されてきました。lpr遺伝子は、アポトーシスに関わるFasタンパクを規定する遺伝子の機能の欠損遺伝子ですが、MRL/lprマウスの膠原病の発症にはMRLマウスの複数の疾患感受性遺伝子としての背景遺伝子が必要とされております。一方、C3H/lprマウスは、C3H/HeJマウスにlpr遺伝子を導入したマウスでリンパ節腫脹をきたしますが、MRL/lprマウスのような膠原病に類似した病変は発症しないことが知られています。MXH-lpr/lpr(MXH/lpr)マウスは、MRL/lprとC3H/lprマウスを始祖としたリコンビナント近交系(RI)マウスで、膠原病自然発症マウスでこれまで樹立された世界で唯一のRIマウスで、MXH/Mo/lpr系では、MXH36/lprマウスを含めて8系統樹立されております。これらのRIマウスは、系統毎に発症する病変や血中の自己抗体の産生が異なっている一方で、全ての対立遺伝子がMRLあるいはC3H由来のホモになっているため、疾患感受性遺伝子の同定や治療薬剤の作用部位の解析などに有効とされています。
Colony maintenance
References
Tissue Antigens, 64(4): 402-403 (2004).

Evaluating the role of rheumatoid factors for the development of rheumatoid arthritis in a mouse model with a newly established ELISA system.
Yuki Tanaka, Hiroaki Komori, Shiro Mori, Yoshiko Soga, Takahito Tsubaki, Miho Terada, Tatsuhiko Miyazaki, Takahiro Fujino, Satoshi Nakamura, Hiroyuki Kanno, Tatsuya Sawasaki, Yaeta Endo, Masato Nose
Tohoku J. Exp. Med., 220(3):199-206 (2010). 20208415

Genomics of vasculitis: lessons from mouse models.
Masato Nose, Hiroaki Komori, Tatsuhiko Miyazaki, Shiro Mori
Ann. Vasc. Dis., 6(1):16-21 (2013). 23641278

Health Report

Examination Date / Room / Rack2026/03/09Room:4-BRack:A囮検査
2026/01/05Room:4-BRack:A囮検査

Gene

Gene SymbolGene NameChr.Allele SymbolAllele NameCommon NamesPromoterDiseases Related to This Gene
Fas
MGI:95484		Fas cell surface death receptor19Fas<lpr>
MGI:1856334		lymphoproliferation
  • autoimmune lymphoproliferative syndrome type 1(DisGeNET, MedGEN)

    • Phenotype

    Phenotype annotation from literatures by Mammalian phenotype ontology
  • CNS inflammation (MP:0006082)

  • abnormal B cell activation (MP:0008217)

  • abnormal B cell morphology (MP:0004939)

  • abnormal B cell receptor editing (MP:0008766)

  • abnormal NK cell physiology (MP:0010766)
    • more 145 phenotypes
  • abnormal T cell morphology (MP:0008037)

  • abnormal T cell physiology (MP:0002444)

  • abnormal T cell proliferation (MP:0005094)

  • abnormal T-helper 2 physiology (MP:0005466)

  • abnormal airway responsiveness (MP:0002334)

  • abnormal cardiovascular system physiology (MP:0001544)

  • abnormal circulating protein level (MP:0005416)

  • abnormal conjunctival epithelium morphology (MP:0004481)

  • abnormal coronary artery morphology (MP:0004111)

  • abnormal cytotoxic T cell physiology (MP:0005078)

  • abnormal enzyme/coenzyme activity (MP:0005584)

  • abnormal gland morphology (MP:0002163)

  • abnormal glomerular capillary endothelium morphology (MP:0011453)

  • abnormal glomerular capillary morphology (MP:0011320)

  • abnormal hepatocyte morphology (MP:0000607)

  • abnormal immune system morphology (MP:0000685)

  • abnormal immune system organ morphology (MP:0002722)

  • abnormal immune system physiology (MP:0001790)

  • abnormal immunoglobulin level (MP:0002490)

  • abnormal interleukin level (MP:0008751)

  • abnormal kidney morphology (MP:0002135)

  • abnormal kidney physiology (MP:0002136)

  • abnormal leukocyte adhesion (MP:0003628)

  • abnormal leukocyte morphology (MP:0008246)

  • abnormal liver physiology (MP:0000609)

  • abnormal lymph node cell ratio (MP:0008828)

  • abnormal lymph node morphology (MP:0002339)

  • abnormal marginal zone B cell morphology (MP:0008180)

  • abnormal myocardium layer morphology (MP:0005329)

  • abnormal renal glomerulus basement membrane morphology (MP:0011348)

  • abnormal renal glomerulus morphology (MP:0005325)

  • abnormal skin condition (MP:0001191)

  • abnormal spatial learning (MP:0001463)

  • abnormal splenic cell ratio (MP:0008826)

  • abnormal stria vascularis morphology (MP:0000048)

  • abnormal stria vascularis vasculature morphology (MP:0004368)

  • abnormal strial intermediate cell morphology (MP:0004367)

  • abnormal submandibular gland morphology (MP:0003793)

  • abnormal thymus medulla morphology (MP:0002375)

  • abnormal thyroid follicle morphology (MP:0004696)

  • abnormal thyroid gland morphology (MP:0000681)

  • abnormal type III hypersensitivity reaction (MP:0002533)

  • abnormal vagina weight (MP:0008978)

  • albuminuria (MP:0002871)

  • autoimmune response (MP:0001844)

  • blood vessel inflammation (MP:0001863)

  • brain inflammation (MP:0001847)

  • conjunctivitis (MP:0001852)

  • cortical renal glomerulopathies (MP:0000523)

  • decreased B cell apoptosis (MP:0008783)

  • decreased B cell number (MP:0005017)

  • decreased CD4-positive, alpha-beta T cell number (MP:0008075)

  • decreased CD8-positive, alpha-beta T cell number (MP:0008079)

  • decreased IgG level (MP:0001805)

  • decreased activated T cell number (MP:0001830)

  • decreased apoptosis (MP:0006043)

  • decreased eosinophil cell number (MP:0005012)

  • decreased hepatocyte apoptosis (MP:0008891)

  • decreased immature B cell number (MP:0008215)

  • decreased interleukin-2 secretion (MP:0008688)

  • decreased macrophage apoptosis (MP:0011081)

  • decreased marginal zone B cell number (MP:0008182)

  • decreased mature B cell number (MP:0008211)

  • decreased neuron apoptosis (MP:0003204)

  • decreased spleen germinal center number (MP:0008482)

  • decreased susceptibility to autoimmune diabetes (MP:0004804)

  • decreased transitional stage B cell number (MP:0008190)

  • demyelination (MP:0000921)

  • dilated renal glomerular capsule (MP:0003670)

  • enlarged Peyer's patches (MP:0008136)

  • enlarged lymph nodes (MP:0000702)

  • enlarged spleen (MP:0000691)

  • enlarged thymus (MP:0000709)

  • epidermal necrosis (MP:0001220)

  • expanded mesangial matrix (MP:0011353)

  • focal hepatic necrosis (MP:0001656)

  • glomerular crescent (MP:0011506)

  • glomerulonephritis (MP:0002743)

  • impaired coordination (MP:0001405)

  • impaired leukocyte tethering or rolling (MP:0020332)

  • increased IgA level (MP:0002495)

  • increased IgG level (MP:0002493)

  • increased IgG1 level (MP:0008499)

  • increased IgG2a level (MP:0008500)

  • increased IgG2b level (MP:0008501)

  • increased IgG3 level (MP:0008502)

  • increased IgM level (MP:0002494)

  • increased anti-double stranded DNA antibody level (MP:0004762)

  • increased anti-erythrocyte antigen antibody level (MP:0004797)

  • increased anti-histone antibody level (MP:0004796)

  • increased anti-nuclear antigen antibody level (MP:0004794)

  • increased anti-single stranded DNA antibody level (MP:0004771)

  • increased apoptosis (MP:0006042)

  • increased autoantibody level (MP:0003725)

  • increased blood urea nitrogen level (MP:0005565)

  • increased body weight (MP:0001260)

  • increased circulating serum albumin level (MP:0002965)

  • increased circulating total protein level (MP:0005568)

  • increased double-negative T cell number (MP:0005090)

  • increased follicular B cell number (MP:0008173)

  • increased gamma-delta T cell number (MP:0008346)

  • increased immature B cell number (MP:0008214)

  • increased immunoglobulin level (MP:0002461)

  • increased kidney cell proliferation (MP:0011440)

  • increased marginal zone B cell number (MP:0008181)

  • increased mesangial cell number (MP:0011427)

  • increased neutrophil cell number (MP:0000219)

  • increased or absent threshold for auditory brainstem response (MP:0011967)

  • increased plasma cell number (MP:0008097)

  • increased renal glomerulus apoptosis (MP:0011369)

  • increased renal glomerulus basement membrane thickness (MP:0011409)

  • increased spleen weight (MP:0004952)

  • increased splenocyte number (MP:0009338)

  • increased susceptibility to Picornaviridae infection (MP:0020937)

  • increased susceptibility to autoimmune disorder (MP:0005350)

  • increased susceptibility to bacterial infection (MP:0002412)

  • increased susceptibility to induced morbidity/mortality (MP:0009763)

  • increased susceptibility to systemic lupus erythematosus (MP:0004801)

  • increased susceptibility to type III hypersensitivity reaction (MP:0005615)

  • increased thymus weight (MP:0004955)

  • increased transitional stage B cell number (MP:0008189)

  • increased urine protein level (MP:0002962)

  • joint swelling (MP:0002936)

  • kidney failure (MP:0003606)

  • kidney inflammation (MP:0001859)

  • lacrimal gland atrophy (MP:0013455)

  • lacrimal gland inflammation (MP:0008880)

  • lymph node hemorrhage (MP:0031051)

  • lymph node hyperplasia (MP:0008102)

  • lymph node necrosis (MP:0031052)

  • mortality/aging (MP:0010768)

  • podocyte foot process effacement (MP:0008140)

  • premature death (MP:0002083)

  • renal cast (MP:0011402)

  • renal glomerular immunoglobulin deposits (MP:0020519)

  • renal glomerular protein deposits (MP:0020518)

  • renal glomerulus hypertrophy (MP:0011294)

  • ruffled hair (MP:0000420)

  • salivary gland inflammation (MP:0001870)

  • skin lesions (MP:0001212)

  • submandibular gland inflammation (MP:0008879)

  • thymus atrophy (MP:0003644)

  • thymus cortex atrophy (MP:0013585)

  • thyroid gland inflammation (MP:0003504)

  • vascular inflammation (MP:0001864)
    • Detailed phenotype data

    Ordering Information

    供与核酸
    Research applicationApoptosis Research
    Immunology and Inflammation Research
    Mouse Models for Human Disease
    提供条件利用者は提供承諾書を用いて、事前に寄託者の承諾を得る。研究成果の公表にあたって寄託者の指定する文献を引用する。Tissue Antigens, 64(4): 402-403 (2004). Tohoku J. Exp. Med., 220(3):199-206 (2010). Ann. Vasc. Dis., 6(1):16-21 (2013).
    Depositor小玉　哲也（国立大学法人東北大学）
    Strain Status凍結胚のアイコン凍結胚
    凍結精子のアイコン凍結精子
    Strain Availability凍結胚を1ヶ月以内に提供可能
    生体マウスを1~3ヶ月以内に提供可能
    Additional Info.Necessary documents for ordering:
    1. Approval form (Japanese / English)
    2. Order form (Japanese / English)
    3. Category I MTA: MTA for distribution with RIKEN BRC (Japanese / English)

    BRC mice in Publications

    No Data