PXD044407

PXD044407 is an original dataset announced via ProteomeXchange.

Title	The effect of utrophin on the electrophysiological properties of DMD hiPSC-derived cardiomyocytes
Description	DMD is a genetic disease, which leads to muscle weakness and cardiomyopathy. The latter remains incurable, being the main cause of death in DMD, therefore new therapeutic strategies are being sought to provide effective treatment. One of them considers upregulation of utrophin, a protein structurally and functionally homologous to dystrophin. In this study proteomic analysis of dystrophin-deficient and both dystrophin- and utrophin-deficient hiPSC-CM indicated on considerable differences in terms of contraction-related mechanisms. We thus investigated the role of utrophin in the maintenance of electrophysiological properties of DMD hiPSC-CM using the cells with additional utrophin deficiency and with utrophin upregulation. Obtained results indicated on disturbance of calcium handling in DMD hiPSC-CM, even more pronounced in DMD/UTRN KO hiPSC-CM and increased values of AHP in DMD hiPSC-CM. Utrophin upregulation improved both calcium oscillations and AHP values. Our findings highlight utrophin as important in the maintenance of the electrophysiological properties of DMD hiPSC-CM.
HostingRepository	PRIDE
AnnounceDate	2024-06-20
AnnouncementXML	Submission_2024-06-19_23:51:49.381.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Luisa Marie Schmidt
SpeciesList	scientific name: Homo sapiens (Human); NCBI TaxID: 9606;
ModificationList	No PTMs are included in the dataset
Instrument	Orbitrap Eclipse

Revision	Datetime	Status	ChangeLog Entry
0	2023-08-07 04:26:45	ID requested
⏵ 1	2024-06-19 23:51:49	announced

10.1016/J.OMTN.2024.102247;

submitter keyword: cardiomyocytes, DMD,utrophin

Józef Dulak
contact affiliation	Department of Medical Biotechnology; Faculty of Biochemistry, Biophysics and Biotechnology, Jagiellonian University, Kraków, Poland
contact email	jozef.dulak@uj.edu.pl
lab head
Luisa Marie Schmidt
contact affiliation	CECAD research center, Institue of Genetics, University of Cologne
contact email	luisa.schmidt@uni-koeln.de
dataset submitter

Dataset FTP location NOTE: Most web browsers have now discontinued native support for FTP access within the browser window. But you can usually install another FTP app (we recommend FileZilla) and configure your browser to launch the external application when you click on this FTP link. Or otherwise, launch an app that supports FTP (like FileZilla) and use this address: ftp://ftp.pride.ebi.ac.uk/pride/data/archive/2024/06/PXD044407

PRIDE project URI

• PRIDE
  1. PXD044407
     1. Label: PRIDE project
     2. Name: The effect of utrophin on the electrophysiological properties of DMD hiPSC-derived cardiomyocytes