PXD039613-1

PXD039613 is an original dataset announced via ProteomeXchange.

Title	Multi-omics profiling of hypertrophic cardiomyopathy reveals altered mechanisms in mitochondrial dynamics and excitation-contraction coupling
Description	Hypertrophic cardiomyopathy is one of the most common inherited cardiomyopathies, and a leading cause of sudden cardiac death in young adults. Despite profound insights into the genetics, there is imperfect correlation between mutation and clinical prognosis, suggesting complex molecular cascades driving pathogenesis. To investigate this, we performed an integrated quantitative multi-omics (proteomic, phosphoproteomic, metabolomic) analysis to illuminate the early and direct consequences of mutations in myosin heavy chain in engineered human induced pluripotent stem cell-derived cardiomyocytes relative to late-stage disease using patient myectomies. We captured hundreds of differential features which map to distinct molecular mechanisms modulating mitochondrial homeostasis at the earliest stages of pathobiology as well as stage-specific metabolic and excitation-coupling maladaptation. Collectively, this study fills in gaps from previous studies by expanding knowledge of the initial responses to mutations that protect cells against the early stress prior to contractile dysfunction and overt disease.
HostingRepository	PRIDE
AnnounceDate	2023-05-10
AnnouncementXML	Submission_2023-05-10_15:05:39.340.xml
DigitalObjectIdentifier	https://dx.doi.org/10.6019/PXD039613
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Supported dataset by repository
PrimarySubmitter	JarrodMoore
SpeciesList	scientific name: Homo sapiens (Human); NCBI TaxID: 9606;
ModificationList	phosphorylated residue
Instrument	Orbitrap Eclipse

Revision	Datetime	Status	ChangeLog Entry
0	2023-01-23 07:12:35	ID requested
⏵ 1	2023-05-10 15:05:40	announced
2	2023-11-14 08:48:00	announced	2023-11-14: Updated project metadata.

10.6019/PXD039613;

Moore J, Ewoldt J, Venturini G, Pereira AC, Padilha K, Lawton M, Lin W, Goel R, Luptak I, Perissi V, Seidman CE, Seidman J, Chin MT, Chen C, Emili A, Multi-Omics Profiling of Hypertrophic Cardiomyopathy Reveals Altered Mechanisms in Mitochondrial Dynamics and Excitation-Contraction Coupling. Int J Mol Sci, 24(5):(2023) [pubmed]

submitter keyword: myectomy,hiPSC-CM, HCM

AndrewEmili
contact affiliation	Center for Network Systems Biology, Department of Biochemistry, Boston University School of Medicine, Boston, MA, USA
contact email	aemili@bu.edu
lab head
JarrodMoore
contact affiliation	Boston University School of Medicine
contact email	jmoore5@bu.edu
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/2023/05/PXD039613

PRIDE project URI

• PRIDE
  1. PXD039613
     1. Label: PRIDE project
     2. Name: Multi-omics profiling of hypertrophic cardiomyopathy reveals altered mechanisms in mitochondrial dynamics and excitation-contraction coupling