Fibroblast-Cardiomyocyte Interaction in Pediatric Restrictive Cardiomyopathy

Sato, Tatsuyuki; Ito, Masamichi

doi:10.1253/circj.cj-21-0100

Cited by 2 publications

(1 citation statement)

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“…Myofibroblasts participate in scar formation and play a role in ischemic damage, expressing α-smooth muscle actin (α-SMA) and increasing contractility. In contrast, fibroblasts seen in RCM have normal α-SMA expression and low contractility, while maintaining secretion of ECM proteins and cytokines [167]. Cell lines were cultured from human cardiac fibroblasts, taken from four pediatric RCM patients -three with TNNI3 mutations (K178E, R170W, R192H) and one with no detectable causative mutation.…”

Section: Paracrine Signaling and Extracellular Influence On Pathogenesismentioning

confidence: 99%

Restrictive cardiomyopathy: from genetics and clinical overview to animal modeling

Chintanaphol¹,

Orgil²,

Alberson³

et al. 2022

Rev. Cardiovasc. Med.

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Restrictive cardiomyopathy (RCM), a potentially devastating heart muscle disorder, is characterized by diastolic dysfunction due to abnormal muscle relaxation and myocardial stiffness resulting in restrictive filling of the ventricles. Diastolic dysfunction is often accompanied by left atrial or bi-atrial enlargement and normal ventricular size and systolic function. RCM is the rarest form of cardiomyopathy, accounting for 2-5% of pediatric cardiomyopathy cases, however, survival rates have been reported to be 82%, 80%, and 68% at 1-, 2-, and 5-years after diagnosis, respectively. RCM can be idiopathic, familial, or secondary to a systemic disorder, such as amyloidosis, sarcoidosis, and hereditary hemochromatosis. Approximately 30% of cases are familial RCM, and the genes that have been linked to RCM are cTnT, cTnI, MyBP-C, MYH7, MYL2, MYL3, DES, MYPN, TTN, BAG3, DCBLD2, LNMA, and FLNC. Increased Ca 2+ sensitivity, sarcomere disruption, and protein aggregates are some of the few mechanisms of pathogenesis that have been revealed by studies utilizing cell lines and animal models. Additional exploration into the pathogenesis of RCM is necessary to create novel therapeutic strategies to reverse restrictive cardiomyopathic phenotypes.

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Section: Paracrine Signaling and Extracellular Influence On Pathogenesismentioning

confidence: 99%