PXD040488

PXD040488 is an original dataset announced via ProteomeXchange.

Title	The atrial and ventricular myocardial proteome of end-stage lamin heart disease
Description	Background: Lamins A/C (encoded by the LMNA gene) can lead to dilated cardiomyopathy (DCM). Objectives: This study sought to undertake proteomic analysis of myocardial tissue to explore the postgenomic phenotype of end-stage lamin heart disease. Methods: Consecutive patients with end-stage lamin heart disease (LMNA-group, n=7) and ischaemic DCM (ICM-group, n=7) undergoing heart transplantation were enrolled. Samples were obtained from left atrium(LA), left ventricle(LV), right atrium(RA), right ventricle(RV) and interventricular septum(IVS). Liquid chromatography combined with mass-spectrometry was used for protein quantification. We compared protein concentrations in cardiac samples between LMNA and ICM groups. Proteins were considered differentially abundant if the quantitative difference was 1.5-fold and corrected p-value <0.05 at a false discovery rate of 0.01. Gene ontology(GO) enrichment analysis explored the related biological processes. Results: 4,247 proteins were identified in LMNA and ICM samples, of which 633 were differentially abundant in LA, 39 in LV, 181 in RA, 52 in RV, and 85 in IVS. Abundance of lamin A/C was reduced but lamin B (LMNB) increased in LMNA LA/RA tissue compared to ICM, but not in LV/RV. Transthyretin was more abundant in the LV/RV of LMNA compared to ICM while sarcomeric proteins such as titin and cardiac myosin heavy chain were generally reduced in RA/LA of LMNA. Protein expression profiling and GO enrichment analysis revealed sarcopenia, extracellular matrix(ECM) remodeling, deficient myocardial energetics, redox imbalances, and abnormal calcium handling in LMNA samples. Conclusion: Lamin heart disease is a biventricular and biatrial disease, characterized by sarcopenia, aberrant metabolism, and ECM remodeling. LMNB and transthyretin were unexpectedly abundant in the atria and ventricles respectively of patients with end-stage lamin heart disease potentially hinting to the possibility of compensatory responses.
HostingRepository	PRIDE
AnnounceDate	2024-10-22
AnnouncementXML	Submission_2024-10-22_06:27:08.139.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Gabriella Captur
SpeciesList	scientific name: Homo sapiens (Human); NCBI TaxID: 9606;
ModificationList	acetylated residue; monohydroxylated residue
Instrument	Q Exactive HF

Revision	Datetime	Status	ChangeLog Entry
0	2023-02-28 01:14:36	ID requested
1	2024-01-26 09:53:24	announced
⏵ 2	2024-10-22 06:27:10	announced	2024-10-22: Updated project metadata.

Topriceanu CC, Alfarih M, Hughes AD, Shiwani H, Chan F, Mohiddin SA, Moody W, Steeds RP, O'Brien B, Vowinckel J, Syrris P, Coats C, Pettit S, Arbustini E, Moon JC, Captur G, The atrial and ventricular myocardial proteome of end-stage lamin heart disease. Acta Myol, 42(2-3):43-52(2023) [pubmed]

10.36185/2532-1900-339;

submitter keyword: Lamin, Human, Heart

Gabriella Captur
contact affiliation	Consultant Cardiologist in Inherited Cardiac Conditions – Royal Free London Senior Clinical Lecturer – UCL MRC LHA @UCL 1-19 Torrington Place London UK WC1E 7HB
contact email	gabriella.captur@ucl.ac.uk
lab head
Gabriella Captur
contact affiliation	UCL
contact email	gabriella.captur@ucl.ac.uk
dataset submitter

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PRIDE project URI

• PRIDE
  1. PXD040488
     1. Label: PRIDE project
     2. Name: The atrial and ventricular myocardial proteome of end-stage lamin heart disease