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A Micro-Engineered Heart Tissue Model of Desmin-related Cardiomyopathy Caused by Mutant αB Crystallin

2025-10-03

Abstract excerpt

Protein quality control (PQC) is essential for maintaining sarcomere integrity in cardiomyocytes. α Crystallin B chain (CRYAB) R120G mutation disrupts CRYAB’s chaperone activity, leading to aggregation of CRYAB and its client proteins (including Desmin), leading to Desmin-related cardiomyopathy (DRM). Prior experimental systems for modeling DRM linked to CRYAB require massive overexpression of CRYAB mutant isoform...

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Literature Corpus work
0a40aa3e-954b-5544-b205-b044deaeaeae
DOI
10.1101/2025.10.02.680018
Open publication

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