The B6-hFBN1 mouse is a humanized model, generated by in situ replacement of the mouse Fbn1 gene sequence (including 3'UTR) with the corresponding human FBN1 sequence while retaining the mouse signal peptide. This model is suitable for research on the pathogenesis and therapeutic agents for Marfan syndrome (MFS), dominant Weill-Marchesani syndrome, scleroderma, and other related disorders. Additionally, leveraging its proprietary TurboKnockout fusion BAC recombination technology, Cyagen can provide popular mutation disease models based on this platform or offer customized services for different mutations to meet the experimental needs of researchers.