PXD019316-1

PXD019316 is an original dataset announced via ProteomeXchange.

Title	Vulnerability of Progeroid Smooth Muscle Cells to biomechanical forces is mediated by MMP13
Description	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.
HostingRepository	PRIDE
AnnounceDate	2020-08-24
AnnouncementXML	Submission_2020-08-23_23:18:56.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Alessandro Ori
SpeciesList	scientific name: Homo sapiens (Human); NCBI TaxID: 9606;
ModificationList	monohydroxylated residue; acetylated residue; iodoacetamide derivatized residue
Instrument	Orbitrap Fusion Lumos

Revision	Datetime	Status	ChangeLog Entry
0	2020-05-20 17:25:26	ID requested
⏵ 1	2020-08-23 23:18:57	announced
2	2020-09-11 03:24:51	announced	2020-09-11: Updated publication reference for PubMed record(s): 32807790.
3	2024-10-22 05:11:19	announced	2024-10-22: Updated project metadata.

Pitrez PR, Estronca L, Monteiro LM, Colell G, Vaz, ã, o H, Santinha D, Harhouri K, Thornton D, Navarro C, Egesipe AL, Carvalho T, Dos Santos RL, L, é, vy N, Smith JC, de Magalh, ã, es JP, Ori A, Bernardo A, De Sandre-Giovannoli A, Nissan X, Rosell A, Ferreira L, Vulnerability of progeroid smooth muscle cells to biomechanical forces is mediated by MMP13. Nat Commun, 11(1):4110(2020) [pubmed]

submitter keyword: Progeria, aging, disease modelling, induced pluripotent stem cells, therapies

Alessandro Ori
contact affiliation	Leibniz Institute on Aging – Fritz Lipmann Institute (FLI) Beutenbergstrasse 11 07745 Jena, Germany
contact email	alessandro.ori@leibniz-fli.de
lab head
Alessandro Ori
contact affiliation	Leibniz Institute on Aging
contact email	aleori@gmail.com
dataset submitter

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PRIDE project URI

• PRIDE
  1. PXD019316
     1. Label: PRIDE project
     2. Name: Vulnerability of Progeroid Smooth Muscle Cells to biomechanical forces is mediated by MMP13