Figure - PMC

Skip to main content

An official website of the United States government

Here's how you know

Here's how you know

Official websites use .gov
A .gov website belongs to an official government organization in the United States.

Secure .gov websites use HTTPS
A lock ( ) or https:// means you've safely connected to the .gov website. Share sensitive information only on official, secure websites.

View full-text article in PMC

. 2021 Aug 4;9:634190. doi: 10.3389/fcell.2021.634190

Search in PMC
Search in PubMed
View in NLM Catalog
Add to search

Copyright © 2021 Chien, Chien, Lin, Tsai, Chou, Yarmishyn, Rastegari, Wang, Leu, Yang, Wang, Jheng, Lai, Ching, Huo, Cherng and Wang.

This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

PMC Copyright notice

Generation and characterization of Fabry disease patient-specific iPSC-derived cardiomyocytes. (A) Transthoracic echocardiogram demonstrating marked ventricular septum (VS) and posterior wall (PW) hypertrophy of the left ventricle. (B) H&E staining of a myocardial biopsy sample showing markedly hypertrophic and disorganized myocytes with large perinuclear and sarcoplasmic vacuoles. (C) Toluidine blue staining of a myocardial biopsy sample showing accumulation of glycosphingolipids. (D) Transmission electron microscopy (TEM) image of a myocardial biopsy sample showing lamellar bodies (zebra bodies) representing lysosomes containing glycolipids. Magnification 60,000×. (E) Schematic outline of experimental approach to generate Fabry disease patient-specific iPSC-derived cardiomyocytes. (F) Sanger sequencing confirming the presence of IVS4 + 919 G > A mutation in FC-iPSC cell line. (G) Alkaline phosphatase staining of Ctrl-iPSC and FC-iPSC clones. (H) Immunofluorescent staining of cardiac troponin T (cTnT) in Ctrl-iPSC-CM and FC-iPSC-CM clones at post-induction day 60. (I) Quantification of area size of Ctrl-iPSC-CM and FC-iPSC-CM clones. (J) Quantification of GLA enzymatic activity in Ctrl-iPSC-CM and FC-iPSC-CM clones. In (I,J) mean values from three independent experiments are show with standard deviation error bars, ^∗p < 0.01 (Student’s t-test).