UCL Discovery
UCL home » Library Services » Electronic resources » UCL Discovery

The atrial and ventricular myocardial proteome of endstage lamin heart disease

Topriceanu, CC; Alfarih, M; Hughes, AD; Shiwani, H; Chan, F; Mohiddin, SA; Moody, W; ... Captur, G; + view all (2023) The atrial and ventricular myocardial proteome of endstage lamin heart disease. Acta Myologica , 42 (2-3) pp. 43-52. 10.36185/2532-1900-339. Green open access

[thumbnail of document.pdf]
Preview
Text
document.pdf - Published Version

Download (439kB) | Preview

Abstract

Lamins A/C (encoded by LMNA gene) can lead to dilated cardiomyopathy (DCM). This pilot study sought to explore the postgenomic phenotype of end-stage lamin heart disease. Consecutive patients with end-stage lamin heart disease (LMNA-group, n = 7) and ischaemic DCM (ICM-group, n = 7) undergoing heart transplantation were prospectively enrolled. Samples were obtained from left atrium (LA), left ventricle (LV), right atrium (RA), right ventricle (RV) and interventricular septum (IVS), avoiding the infarcted myocardial segments in the ICM-group. Samples were analysed using a discovery 'shotgun' proteomics approach. We found that 990 proteins were differentially abundant between LMNA and ICM samples with the LA being most perturbed (16-fold more than the LV). Abundance of lamin A/C protein was reduced, but lamin B increased in LMNA LA/RA tissue compared to ICM, but not in LV/RV. Carbonic anhydrase 3 (CA3) was over-abundant across all LMNA tissue samples (LA, LV, RA, RV, and IVS) when compared to ICM. Transthyretin was more abundant in the LV/RV of LMNA compared to ICM, while sarcomeric proteins such as titin and cardiac alpha-cardiac myosin heavy chain were generally less abundant in RA/LA of LMNA. Protein expression profiling and enrichment analysis pointed towards sarcopenia, extracellular matrix remodeling, deficient myocardial energetics, redox imbalances, and abnormal calcium handling in LMNA samples. Compared to ICM, end-stage lamin heart disease is a biventricular but especially a biatrial disease appearing to have an abundance of lamin B, CA3 and transthyretin, potentially hinting to compensatory responses.

Type: Article
Title: The atrial and ventricular myocardial proteome of endstage lamin heart disease
Open access status: An open access version is available from UCL Discovery
DOI: 10.36185/2532-1900-339
Publisher version: https://doi.org/10.36185/2532-1900-339
Language: English
Additional information: © Gaetano Conte Academy - Mediterranean Society of Myology OPEN ACCESS This is an open access article distributed in accordance with the CC-BY-NC-ND (Creative Commons Attribution-NonCommercialNoDerivatives 4.0 International) license. The article can be used by giving appropriate credit and mentioning the license, but only for non-commercial purposes and only in the original version. For further information: https://creativecommons.org/licenses/by-ncnd/4.0/deed.en
Keywords: lamin heart disease, proteomics, remodelling, compensatory expression
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > Institute of Cardiovascular Science
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > Institute of Cardiovascular Science > Clinical Science
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > Institute of Cardiovascular Science > Population Science and Experimental Medicine
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > Institute of Cardiovascular Science > Population Science and Experimental Medicine > MRC Unit for Lifelong Hlth and Ageing
URI: https://discovery.ucl.ac.uk/id/eprint/10180878
Downloads since deposit
7Downloads
Download activity - last month
Download activity - last 12 months
Downloads by country - last 12 months

Archive Staff Only

View Item View Item