PXD025523

PXD025523 is an original dataset announced via ProteomeXchange.

Title	arrythmogenic right ventricular acrdiomayopathy (PKP2-cko in mice and Human biopsies)
Description	Summary: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiac disorder. It is classified as the second most common cause of unexpected sudden death by cardiac arrest in the young. ARVC has devastating psychosocial consequences, especially as many patients are young adults, and current therapy is limited to extreme exercise restriction and defibrillator implantation. More than 40% of the reported genetic variants linked to ARVC reside in a gene called PKP2 , which encodes for the plakophilin-2 (PKP2) protein. The pathogenic mechanisms linking this protein to ARVC remain to be elucidated, which is the focus of this project. I aim to identify exactly which proteins in the heart are dysregulated and to identify why exercise is detrimental for carriers of PKP2-deficient hearts. Our approach is based on state-of-the-art mass spectrometry (MS) technologies that allow us to measure all proteins in the heart simultaneously. We used both human heart biopsy material as well as a murine disease model I anticipate to elucidate novel roles of PKP2 that are of utmost importance for understanding ARVC pathogenesis.
HostingRepository	PRIDE
AnnounceDate	2023-11-14
AnnouncementXML	Submission_2023-11-14_07:40:32.076.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Navratan Bagwan
SpeciesList	scientific name: Mus musculus (Mouse); NCBI TaxID: 10090; scientific name: Homo sapiens (Human); NCBI TaxID: 9606;
ModificationList	No PTMs are included in the dataset
Instrument	Q Exactive HF

Revision	Datetime	Status	ChangeLog Entry
0	2021-04-21 02:49:24	ID requested
1	2022-11-04 10:29:09	announced
⏵ 2	2023-11-14 07:40:34	announced	2023-11-14: Updated project metadata.

P, é, rez-Hern, á, ndez M, van Opbergen CJM, Bagwan N, Vissing CR, Marr, ó, n-Li, ñ, ares GM, Zhang M, Torres Vega E, Sorrentino A, Drici L, Sulek K, Zhai R, Hansen FB, Christensen AH, Boesgaard S, Gustafsson F, Rossing K, Small EM, Davies MJ, Rothenberg E, Sato PY, Cerrone M, Jensen THL, Qvortrup K, Bundgaard H, Delmar M, Lundby A, Loss of Nuclear Envelope Integrity and Increased Oxidant Production Cause DNA Damage in Adult Hearts Deficient in PKP2: A Molecular Substrate of ARVC. Circulation, 146(11):851-867(2022) [pubmed]

submitter keyword: cardiovascular protemics, TMT, heart failure, ARVC

Alicia Lundby
contact affiliation	Associate Professor, Department of Biomedical Sciences,Affiliated with The Novo Nordisk Foundation Center for Protein Research, Faculty of Health and Medical Sciences, University of Copenhagen.
contact email	alicia.lundby@sund.ku.dk
lab head
Navratan Bagwan
contact affiliation	University of Copenhagen
contact email	navratan.bagwan@sund.ku.dk
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/2022/11/PXD025523

PRIDE project URI

• PRIDE
  1. PXD025523
     1. Label: PRIDE project
     2. Name: arrythmogenic right ventricular acrdiomayopathy (PKP2-cko in mice and Human biopsies)