Strain Data Sheet

RBRC11160

Strain Information

Image
BRC No.RBRC11160
TypeRecombinant Congenic
SpeciesMus musculus
Strain nameMcH/Mo-lpr/lpr-RA1
Former Common nameMcH/Mo/lpr-RA1
H-2 Haplotype
ES Cell line
Background strain
Appearance
Strain development早期に破壊性関節炎、唾液腺炎、血管炎を自然発症するリコンビナントコンジェニックマウス McH/Mo-lpr/lpr-RAl(McH/lpr-RA1)は、東北大学大学院医工学研究科 腫瘍医工学分野 森士朗が樹立し、McH/lpr-RA1マウスの開発年は2001年です。McH/lpr-RAl由来となった系統は、MRL/MpJ-lpr/lprマウスおよびC3H/HeJ-lpr/lprマウスで米国ジャクソン研究所から導入しました。McH/lpr-RA1マウスは次に示すように交配し樹立しました：　MRL/MpJ-/pr/lpr x (MRL/MpJ-/pr/lpr x C3H/HeJ/pr/lpr)F1→N2→N2F1→N2F2→ N2F54。Y染色体の由来系統はC3H/HeJ-lpr/lprマウスです。病理組織学的に関節炎、関節強直、唾液腺炎、関節炎を発症。血清学的にはMRL/lprマウスに比較し、IgG-RF、anti-dsDNA IgG、anti-dsDNA IgM、血清IgGは低値です。
Strain description膠原病におきましては、関節リウマチ(RA)、全身性エリテマトーデス(SLE)、シェーグレン症候群(SjS)、結節性多発性動脈炎(PN)などにみられる複数の病変が、同一個体に併発する症例がしばしばみられます。これらの膠原病の発症機を解明するため、我々は、同一個体に自己免疫性の腎炎、血管炎、唾液腺炎、血管炎などを自然発症するMRL/MpJ-lpr/lpr(MRL/lpr)マウスを用いたゲノム解析を行ってまいりました。その結果、膠原病の発症には複数の疾患感受性遺伝子が関与することが明らかとなりました。我々が用いた解析手法は、上記MRL/lprマウスとC3H/HeJ-lpr/lpr(C3H/lpr)マウスのF2やN2世代のマウスを対象にしたマイクロサテライトマーカーを用いたゲノム連鎖解析でした。この研究の過程で我々は、足関節に著明な関節腫脹を示す個体を見出し、このマウスを50世代以上に渡り関節炎発症個体との選択的交配を繰り返すことにより、早期に関節強直を自然発症するリコンビナントコンジェニックマウスMcH/Mo-lpr/lpr-RA1(McH/lpr-RA1)の樹立することができました。McH/lpr-RA1マウスは、3.5か月齢以降の雄で高率に、MRL/lprマウスではみられないような、ヒトのRAに類似したパンヌス形成を伴う骨破壊や関節強直を自然発症する疾患モデルマウスです。また、SjSやPNにみられるような唾液腺炎や血管炎も関節炎の発症と同時期に、MRL/lprマウスに比較し、高頻度で重度に発症します。一方、McH/lpr-RA1マウスにおきましては、MRL/lprマウスにみられるような全身のリンパ節腫脹や重篤な腎炎の発症はみられませんので、繁殖・維持が容易で、長期の薬剤投与実験も可能です。血清学的所見も添付資料に示しますようにMRL/lprマウスとは異なり、新規の膠原病疾患モデルマウスとして期待できます。
Colony maintenance
References
Novel recombinant congenic mouse strain developing arthritis with enthesopathy.
Shiro Mori, Naoko Tanda, Mitsuko R Ito, Hisashi Oishi, Takahito Tsubaki, Hiroaki Komori, Ming-Cai Zhang, Masao Ono, Masahiko Nishimura, Masato Nose
Pathol. Int., 58(7):407-14 (2008). 18577108

McH-lpr/lpr-RA1 mice: A novel spontaneous mouse model of autoimmune sialadenitis.
Keiichi Saito, Shiro Mori, Tetsuya Kodama
Immunol. Lett., 237:3-10 (2021). 34174253

Health Report

Examination Date / Room / Rack

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)

  • cardiovascular system phenotype (MP:0005385)

  • 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)

  • 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。Pathol. Int., 58(7):407-14 (2008). Immunol. Lett., 237:3-10 (2021).
    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凍結胚より作出したマウスを2~4ヶ月以内に提供可能
    凍結胚を1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