B6-huMSH3 Mouse
製品名
B6-huMSH3 Mouse
製品ID
C001910
系統名
C57BL/6NCya-Msh3tm1(hMSH3)/Cya
背景情報
C57BL/6NCya
状況
このマウス系統を論文で使用する場合は、「B6-huMSH3 Mouse（カタログ番号C001910）はサイアジェンから購入しました。」と引用してください。
製品タイプ
年齢
遺伝子型
性別
数量
遺伝子名
遺伝子別名
DUP, FAP4, MRP1
NCBI ID
染色体
Chr 5
MGI ID
さらに
系統詳細
The MSH3 gene is a critical component of the post-replicative DNA mismatch repair (MMR) system that maintains genomic stability. It encodes the MSH3 protein, which serves as a housekeeping protein and is ubiquitously expressed at low levels across a wide range of human tissues, including the colon, rectum, small intestine, brain, and reproductive organs [1]. The protein primarily functions by forming a heterodimer with MSH2 to create the MutSβ complex, which is specialized in recognizing and initiating the repair of large insertion-deletion loops (IDLs) and dinucleotide or longer microsatellite repeats. Beyond its canonical MMR role, MSH3 is involved in homologous recombination and the repair of DNA double-strand breaks, contributing to cellular resistance against platinum-based chemotherapeutics [2]. Furthermore, MSH3 has been identified as a key genetic modifier of repeat expansion diseases, such as Huntington’s disease and myotonic dystrophy type 1, where its activity paradoxically promotes the somatic expansion of toxic CAG/CTG repeats, thereby influencing disease onset and progression [3].
The B6-huMSH3 mouse is a humanized model constructed through gene-editing technology, in which the exon 7 to downstream of exon 24 of mouse Msh3 is replaced with the entire human MSH3 gene plus human MSH3 promoter and downstream region, and the human sequence is inserted in reverse to prevent disruption of the Dhfr gene function. This model can be used for research on Huntington’s disease (HD) and myotonic dystrophy type 1 (DM1), as well as for screening, development, and preclinical evaluation of MSH3-targeted therapeutics.
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.
系統作製戦略
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 1. Diagram of the gene editing strategy for the generation of B6-huMSH3 mice.
適用分野
Screening, development, and preclinical evaluation of MSH3-targeted drugs;
Research on the pathogenic mechanism and relevant treatment methods of Huntington's disease (HD);
Research on the pathogenic mechanism and relevant treatment methods of myotonic dystrophy type 1 (DM1).
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