Proteomics

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Vulnerability of Progeroid Smooth Muscle Cells to biomechanical forces is mediated by MMP13

ABSTRACT: Hutchinson-Gilford Progeria Syndrome (HGPS) is a premature aging disease in children that leads to early death. Smooth muscle cells (SMCs) are the most affected cells in HGPS patients, although the reason for such vulnerability remains poorly understood. In this work, we developed a chip formed from HGPS-SMCs that were generated from induced pluripotent stem cells (iPSCs) to study their vulnerability to flow shear stress. HGPS-iPSC SMCs cultured under arterial flow conditions detached from the chip after a few days of culture; this process was mediated by the up-regulation of metalloprotease 13 (MMP13). Importantly, double mutant LmnaG609G/G609GMmp13-/- mice or LmnaG609G/G609GMmp13+/+ mice treated with a MMP inhibitor showed lower SMC loss in the aortic arch than controls. MMP13 up-regulation appears to be mediated, at least in part, by the up-regulation of glycocalyx. Our results offer a new platform for developing treatments for HGPS patients that may complement previous pre-clinical and clinical treatments.

INSTRUMENT(S): Orbitrap Fusion Lumos

ORGANISM(S): Homo Sapiens (human)

TISSUE(S): Cell Culture

SUBMITTER: Alessandro Ori  

LAB HEAD: Alessandro Ori

PROVIDER: PXD019316 | Pride | 2020-08-24

REPOSITORIES: Pride

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Dataset's files

Source:
Action DRS
190606_IH_PP_Portugal_TMT10_16frHpH_analysis_table_TargetPeptideSpectrumMatch.txt Txt
190606_IH_PP_Portugal_TMT10_16frHpH_fr01.msf Msf
190606_IH_PP_Portugal_TMT10_16frHpH_fr01.raw Raw
190606_IH_PP_Portugal_TMT10_16frHpH_fr02.raw Raw
190606_IH_PP_Portugal_TMT10_16frHpH_fr03.raw Raw
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Hutchinson-Gilford Progeria Syndrome (HGPS) is a premature aging disease in children that leads to early death. Smooth muscle cells (SMCs) are the most affected cells in HGPS individuals, although the reason for such vulnerability remains poorly understood. In this work, we develop a microfluidic chip formed by HGPS-SMCs generated from induced pluripotent stem cells (iPSCs), to study their vulnerability to flow shear stress. HGPS-iPSC SMCs cultured under arterial flow conditions detach from the  ...[more]

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