CARDIAC Publication-derived

TAZ Mutation

Source Wang et al. · Department of Biomedical Engineering, Wyss Institute, Harvard University, Boston, USA · 10.1038/nm.3545

👤 Wang G, McCain ML, Yang L, He A, Bhatt DL, Pasqualini FS, Agarwal A, Yuan H, Jiang D, Zhang D, Zangi L, Geva J, Roberts AE, Ma Q, Ding J, Chen J, Wang DZ, Li K, Wang J, Wanders RJ, Kuber W, Church GM, Bhatt S, Parker KK, Bhatt AB, Pu WT ⏱ 16800 min 🧫 iPSC-Derived (Patient-Specific iPSCs — Barth Syndrome)

Abstract

Wang et al. combined patient iPSC-CMs with muscular thin film technology to model Barth syndrome, revealing that TAZ mutations cause cardiolipin remodeling defects, mitochondrial fragmentation, and impaired contractility. TAZ mRNA delivery rescued the phenotype, providing proof-of-concept for gene therapy approaches to this mitochondrial cardiomyopathy.

Cell source

iPSC-Derived (Patient-Specific iPSCs — Barth Syndrome)

Application

Mitochondrial Cardiomyopathy

Full SOP

🔬

Create a free account to access this protocol

Join OrganMatch to unlock step-by-step procedures, reagent concentrations, QC checklists, and downloadable batch record templates.

Create free account

Already registered? Log in

Attribution

This SOP was authored by Organthis based on the published method in Wang et al.. The originating laboratory holds no rights in this SOP and has not endorsed it unless marked Verified.

This wording is awaiting legal review.

Something wrong with this entry? Report an issue with this protocol

Need a commercial licence?

Use this protocol in your therapeutic or diagnostic pipeline.

Request Commercial Licence