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Maturation and Function of the Intercalated Disc: Report of Two Pediatric Cases Focusing on Cardiac Development and Myocardial Hyperplasia

The development of the normal human heart, ranging from gestational age to the mature adult heart, relies on a very delicate and timely orchestrated order of processes. One of the most striking alterations in time is the gradual extinction of the ability for cardiomyocytes to proliferate. Once passi...

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Autores principales: van Ham, Willem B., Meijboom, Esmeralda E. M., Ligtermoet, Merel L., Nikkels, Peter G. J., van Veen, Toon A. B.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10455643/
https://www.ncbi.nlm.nih.gov/pubmed/37623366
http://dx.doi.org/10.3390/jcdd10080354
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author van Ham, Willem B.
Meijboom, Esmeralda E. M.
Ligtermoet, Merel L.
Nikkels, Peter G. J.
van Veen, Toon A. B.
author_facet van Ham, Willem B.
Meijboom, Esmeralda E. M.
Ligtermoet, Merel L.
Nikkels, Peter G. J.
van Veen, Toon A. B.
author_sort van Ham, Willem B.
collection PubMed
description The development of the normal human heart, ranging from gestational age to the mature adult heart, relies on a very delicate and timely orchestrated order of processes. One of the most striking alterations in time is the gradual extinction of the ability for cardiomyocytes to proliferate. Once passing this event, cardiomyocytes grow and increase in contractile strength by means of physiological hypertrophy. This process, importantly, seems to depend on an adequate development of electromechanical coupling that is achieved by the appropriate formation of the intercellular junction named the intercalated disc (ICD). In this report, we describe two sudden death cases of young and apparently healthy-born individuals without external abnormalities compared to an age-matched control. Histological examination, including the comparison with the age-matched and histology-matched controls, showed a disturbed formation of the protein machinery composing the electromechanical junctions at the ICD and an increased nuclei count for both patients. As a cause or consequence, cardiomyocytes in both sudden death cases showed signs of a delayed developmental stage, presumably resulting in an exaggerated degree of hyperplasia.
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spelling pubmed-104556432023-08-26 Maturation and Function of the Intercalated Disc: Report of Two Pediatric Cases Focusing on Cardiac Development and Myocardial Hyperplasia van Ham, Willem B. Meijboom, Esmeralda E. M. Ligtermoet, Merel L. Nikkels, Peter G. J. van Veen, Toon A. B. J Cardiovasc Dev Dis Brief Report The development of the normal human heart, ranging from gestational age to the mature adult heart, relies on a very delicate and timely orchestrated order of processes. One of the most striking alterations in time is the gradual extinction of the ability for cardiomyocytes to proliferate. Once passing this event, cardiomyocytes grow and increase in contractile strength by means of physiological hypertrophy. This process, importantly, seems to depend on an adequate development of electromechanical coupling that is achieved by the appropriate formation of the intercellular junction named the intercalated disc (ICD). In this report, we describe two sudden death cases of young and apparently healthy-born individuals without external abnormalities compared to an age-matched control. Histological examination, including the comparison with the age-matched and histology-matched controls, showed a disturbed formation of the protein machinery composing the electromechanical junctions at the ICD and an increased nuclei count for both patients. As a cause or consequence, cardiomyocytes in both sudden death cases showed signs of a delayed developmental stage, presumably resulting in an exaggerated degree of hyperplasia. MDPI 2023-08-19 /pmc/articles/PMC10455643/ /pubmed/37623366 http://dx.doi.org/10.3390/jcdd10080354 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Brief Report
van Ham, Willem B.
Meijboom, Esmeralda E. M.
Ligtermoet, Merel L.
Nikkels, Peter G. J.
van Veen, Toon A. B.
Maturation and Function of the Intercalated Disc: Report of Two Pediatric Cases Focusing on Cardiac Development and Myocardial Hyperplasia
title Maturation and Function of the Intercalated Disc: Report of Two Pediatric Cases Focusing on Cardiac Development and Myocardial Hyperplasia
title_full Maturation and Function of the Intercalated Disc: Report of Two Pediatric Cases Focusing on Cardiac Development and Myocardial Hyperplasia
title_fullStr Maturation and Function of the Intercalated Disc: Report of Two Pediatric Cases Focusing on Cardiac Development and Myocardial Hyperplasia
title_full_unstemmed Maturation and Function of the Intercalated Disc: Report of Two Pediatric Cases Focusing on Cardiac Development and Myocardial Hyperplasia
title_short Maturation and Function of the Intercalated Disc: Report of Two Pediatric Cases Focusing on Cardiac Development and Myocardial Hyperplasia
title_sort maturation and function of the intercalated disc: report of two pediatric cases focusing on cardiac development and myocardial hyperplasia
topic Brief Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10455643/
https://www.ncbi.nlm.nih.gov/pubmed/37623366
http://dx.doi.org/10.3390/jcdd10080354
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