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

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      In review
      research-article
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      ScienceOpen Preprints
      ScienceOpen
      HGPS, stem cell, iPSCs, disease models, aging.
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            Abstract

            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.

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            Author and article information

            Journal
            ScienceOpen Preprints
            ScienceOpen
            30 January 2022
            Affiliations
            [1 ] Guangdong Nephrotic Drug Engineering Technology Research Center, The R&D Center of Drug for Renal Diseases, Consun Pharmaceutical Group, Dongpeng Avenue, No.71, Guangzhou, Guangdong 510000, China.
            Author notes
            Article
            10.14293/S2199-1006.1.SOR-.PPYLHY8.v1
            661ceae4-f781-4c99-9966-08ca405a843e

            This work has been published open access under Creative Commons Attribution License CC BY 4.0 , which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Conditions, terms of use and publishing policy can be found at www.scienceopen.com .


            The datasets generated during and/or analysed during the current study are available from the corresponding author on reasonable request.
            Medicine,Life sciences
            HGPS, stem cell, iPSCs, disease models, aging.

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