huMSH3/huTFRC Mouse
製品名
huMSH3/huTFRC Mouse
製品ID
C002114
系統名
C57BL/6Cya-Msh3tm1(hMSH3)Tfrctm2(hTFRC)/Cya
背景情報
C57BL/6Cya
状況
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製品タイプ
年齢
遺伝子型
性別
数量
遺伝子別名
DUP, FAP4, MRP1, T9, TR, TFR, p90, CD71, TFR1, TRFR, IMD46
染色体
Chr 5, Chr 3
MGI ID
さらに
系統詳細
The MSH3 gene is a core component of the post‑replicative DNA mismatch repair (MMR) system, which is responsible for maintaining genomic stability. The MSH3 protein encoded by this gene is a housekeeping protein that is constitutively expressed at low levels in various human tissues, including the colon, rectum, small intestine, brain, and reproductive organs [1]. The primary function of MSH3 is to form a MutSβ heterodimer complex with MSH2, which specifically recognizes and initiates the repair of large insertion‑deletion loops (IDLs) as well as dinucleotide or longer microsatellite repeats. In addition to its canonical role in the MMR system, MSH3 also participates in homologous recombination and DNA double‑strand break repair, thereby making it an important factor influencing cellular tolerance to platinum‑based chemotherapeutic agents [2]. Furthermore, studies have confirmed that MSH3 is a key genetic modifier of repeat expansion disorders. In Huntington's disease and myotonic dystrophy type 1, for example, the MSH3 gene product can promote somatic expansion of cytotoxic CAG/CTG repeats, thereby influencing the onset and progression of these diseases [3].
Transferrin receptor (TFRC) exhibits a unique expression distribution profile in vivo and serves as an important receptor for the peripheral and central targeted delivery of nucleic acid therapeutics [4]. In the peripheral system, TFRC is highly expressed on the surface of skeletal muscle and cardiomyocytes, and has been used to develop antibody‑oligonucleotide conjugates for muscle‑targeted silencing. In the central nervous system, TFRC is expressed on brain capillary endothelial cells and serves as a core target for studying receptor‑mediated transcytosis (RMT) across the blood‑brain barrier (BBB).
The huMSH3/huTFRC mouse is a dual-gene humanized model obtained by crossing huMSH3 mice (Catalog No.: C001910) with huTFRC mice (Catalog No.: C001860). This model is applicable to mechanistic studies of Huntington's disease (HD) and myotonic dystrophy type 1 (DM1), as well as to the screening, development, and safety evaluation of therapeutic agents targeting MSH3/TFRC.
参考文献
Miao HK, Chen LP, Cai DP, Kong WJ, Xiao L, Lin J. MSH3 rs26279 polymorphism increases cancer risk: a meta-analysis. Int J Clin Exp Pathol. 2015 Sep 1;8(9):11060-7.
Tseng-Rogenski SS, Munakata K, Choi DY, Martin PK, Mehta S, Koi M, Zheng W, Zhang Y, Carethers JM. The Human DNA Mismatch Repair Protein MSH3 Contains Nuclear Localization and Export Signals That Enable Nuclear-Cytosolic Shuttling in Response to Inflammation. Mol Cell Biol. 2020 Jun 15;40(13):e00029-20.
Winquist RJ, Church B. Inhibiting Cytosine-Adenine-Guanine (CAG) repeat expansions as a therapeutic strategy for Huntington's disease. Biochem Pharmacol. 2025 Dec;242(Pt 4):117438.
Candelaria PV, Leoh LS, Penichet ML, Daniels-Wells TR. Antibodies Targeting the Transferrin Receptor 1 (TfR1) as Direct Anti-cancer Agents. Front Immunol. 2021 Mar 17;12:607692.
系統作製戦略
The huMSH3/huTFRC mouse is a dual-gene humanized model obtained by crossing the huMSH3 mouse (Catalog No.: C001910) with the huTFRC mouse (Catalog No.: C001860).
Figure 1. Gene editing strategy of huMSH3 mice. The exon 7 to downstream of exon 24 of mouse Msh3 was replaced with the entire human MSH3 gene plus human MSH3 promoter and downstream region, and the human sequence was inserted in reverse to prevent disruption of the Dhfr gene function.
Figure 2. Gene editing strategy of huTFRC mice. The mouse Tfrc endogenous extracellular domain was replaced with the human TFRC extracellular domain. The murine cytoplasmic and helical regions were kept.
適用分野
Mechanistic studies of repeat expansion disorders such as Huntington's disease (HD) and myotonic dystrophy type 1 (DM1);
Screening, development, and safety evaluation of therapeutic agents targeting MSH3/TFRC.
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