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患有转甲状腺素蛋白淀粉样变性病患者的心肌因纤维化增加而产生的力量较小。

Myocardium From Patients With ATTR Amyloidosis Produces Less Force Secondary to Increased Fibrosis.

作者信息

Milburn Gregory N, Bell Jania, Wellette-Hunsucker Austin G, Ruml Hollings, Yackzan Andrew T, Campbell Kenneth S

机构信息

Department of Physiology, University of Kentucky, Lexington, Kentucky, USA; Division of Cardiovascular Medicine, University of Kentucky, Lexington, Kentucky, USA.

Division of Cardiovascular Medicine, University of Kentucky, Lexington, Kentucky, USA.

出版信息

JACC Basic Transl Sci. 2025 Jul;10(7):101271. doi: 10.1016/j.jacbts.2025.03.003. Epub 2025 May 8.

DOI:10.1016/j.jacbts.2025.03.003
PMID:40338773
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12434279/
Abstract

Amyloid transthyretin cardiac amyloidosis is one of the most common infiltrative cardiomyopathies. Contractile, biochemical, and histological assays were performed on myocardium from patients with and without amyloid transthyretin amyloidosis. Force was reduced in amyloidosis, but calcium sensitivity was increased. The change in calcium sensitivity may reflect dephosphorylation of troponin I. The proportion of stiffness attributable to the extracellular matrix was larger in amyloidosis. Septal fibrosis and amyloid burden correlated with measurements from LV samples. Technetium pyrophosphate scans may detect increased microcalcifications in amyloidosis myocardium. Replacement of myocytes with extracellular matrix is the most important factor depressing contractile force in amyloidosis myocardium.

摘要

淀粉样变甲状腺素运载蛋白型心脏淀粉样变性是最常见的浸润性心肌病之一。对患有和未患有淀粉样变甲状腺素运载蛋白淀粉样变性的患者的心肌进行了收缩、生化和组织学检测。淀粉样变性患者的心肌收缩力降低，但钙敏感性增加。钙敏感性的变化可能反映了肌钙蛋白I的去磷酸化。淀粉样变性患者中由细胞外基质导致的僵硬度比例更大。间隔纤维化和淀粉样蛋白负荷与左心室样本的测量结果相关。焦磷酸锝扫描可能检测到淀粉样变性心肌中微钙化增加。心肌细胞被细胞外基质替代是导致淀粉样变性心肌收缩力降低的最重要因素。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d907/12434279/46ce6a223562/gr9.jpg
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https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d907/12434279/46ce6a223562/gr9.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d907/12434279/e8ad86fb67c9/ga1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d907/12434279/8038d0d0d296/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d907/12434279/77ff6702fca9/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d907/12434279/09148eb06bb8/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d907/12434279/a68c22c624b5/gr4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d907/12434279/a5d36dfdd7d4/gr5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d907/12434279/a49688f8b97a/gr6.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d907/12434279/4926411076cd/gr7.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d907/12434279/06c03f07f279/gr8.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d907/12434279/46ce6a223562/gr9.jpg

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本文引用的文献

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