Recent research advances in disease models of Hutchinson-Gilford Progeria Syndrome

Aging can cause many diseases, and aging research may help to elucidate the pathogenesis and identify suitable therapies for attenuating aging or alleviating aging-related diseases such as Hutchinson-Gilford progeria syndrome (HGPS). HGPS is a sporadic genetic premature disorder caused by a de novo point mutation in the LMNA gene encoding the intermediate filament proteins lamins A and C, typically resulting in a short lifespan (average age of 14.6 years). To date, there has been no effective therapy to cure it. In the past few decades, many scientists have focused on the development of disease models (such as iPSC, adult stem cell, and animal models) to uncover the underlying pathogenesis and find an effective therapy for HGPS. In this review, we summarize the recent advances in the disease models of HGPS, compare their advantages and disadvantages, and discuss the perspectives and challenges for these models, which may provide some clues for HGPS therapy.