PXD036557-2

PXD036557 is an original dataset announced via ProteomeXchange.

Title	Modelling Premature Cardiac Aging with Induced Pluripotent Stem Cells From a Hutchinson-Gilford Progeria Syndrome Patient
Description	Hutchinson-Gilford Progeria Syndrome (HGPS) is a rare genetic disorder that causes accelerated aging and a high risk of cardiovascular complications. However, the underlying mechanisms of cardiac complications of this syndrome are not fully understood. This study modeled HGPS using cardiomyocytes (CM) derived from induced pluripotent stem cells (iPSC) derived from a patient with HGPS and characterized the biophysical, morphological, and molecular changes found in these CM compared to CM derived from a healthy donor. Electrophysiological recordings revealed that the HGPS CM was functional and had normal electrophysiological properties. Electron tomography showed nuclear morphology alteration and 3D reconstruction of electron tomography images indicated structural abnormalities in HGPS CM mitochondria. High-resolution respirometry with isolated CM derived from three distinct cellular differentiations suggests that HGPS-CM had a tendency of lower oxygen consumption capacity. However, there was no difference in mitochondrial content as measured by Mitotracker. Telomere length was measured using qRT-PCR, and no difference was found in either the iPSCs or the CM from HGPS when compared to the control. Proteomic analysis was carried out in a high-resolution system using Liquid Chromatography Tandem Mass Spectrometry (LC-MS/MS). The proteomics data show distinct group separations and protein expression differences between HGPS and control-CM, highlighting changes in ribosomal, TCA cycle, and amino acid biosynthesis, among other modifications. Our findings show that iPSC-derived cardiomyocytes from a Progeria Syndrome patient have significant changes in mitochondrial morphology and protein expression, implying novel mechanisms underlying premature cardiac aging
HostingRepository	PRIDE
AnnounceDate	2023-11-14
AnnouncementXML	Submission_2023-11-14_08:48:29.672.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Gustavo Monnerat
SpeciesList	scientific name: Homo sapiens (Human); NCBI TaxID: 9606;
ModificationList	iodoacetamide derivatized residue
Instrument	Q Exactive

Revision	Datetime	Status	ChangeLog Entry
0	2022-09-08 06:35:27	ID requested
1	2022-11-10 13:23:01	announced
⏵ 2	2023-11-14 08:48:30	announced	2023-11-14: Updated project metadata.

Dataset with its publication pending

submitter keyword: progeria,proteomics, ips

Gustavo Monnerat
contact affiliation	federal university of rio de janeiro
contact email	gustavo@lccm-ufrj.org
lab head
Gustavo Monnerat
contact affiliation	Federal University of Rio de Janeiro
contact email	gustavo@lccm-ufrj.org
dataset submitter

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PRIDE project URI

• PRIDE
  1. PXD036557
     1. Label: PRIDE project
     2. Name: Modelling Premature Cardiac Aging with Induced Pluripotent Stem Cells From a Hutchinson-Gilford Progeria Syndrome Patient