Modeling the mitochondrial cardiomyopathy of Barth syndrome with ...

Study of monogenic mitochondrial cardiomyopathies may yield insights into mitochondrial roles in cardiac development and disease.

Studying monogenic mitochondrial cardiomyopathies may yield insights into mitochondrial roles in cardiac development and disease.

Modeling the mitochondrial cardiomyopathy of Barth syndrome with ...

Study of monogenic mitochondrial cardiomyopathies may yield insights into mitochondrial roles in cardiac development and disease. Here, we ...

Da-Zhi Wang Laboratory - Boston Children's Hospital

Modeling the mitochondrial cardiomyopathy of Barth syndrome with induced pluripotent stem cell and heart-on-chip technologies ... G, Wang, McCain ...

Modeling the mitochondrial cardiomyopathy of Barth syndrome with ...

Modeling the mitochondrial cardiomyopathy of Barth syndrome with induced ... cardiomyopathies may yield insights into mitochondrial roles in cardiac development ...

iPSC-Based Modeling of Barth-Syndrome-Associated Cardiomyopathy

BTHS is characterized by cardiomyopathy, neutropenia, skeletal myopathy, and growth delay. Symptoms are usually observed early in infancy, but age at ...

Modeling the mitochondrial cardiomyopathy of Barth syndrome with ...

Abstract: Studying monogenic mitochondrial cardiomyopathies may yield insights into mitochondrial roles in cardiac development and disease.

Modeling the mitochondrial cardiomyopathy of Barth syndrome with ...

Study of monogenic mitochondrial cardiomyopathies may yield insights into mitochondrial roles in cardiac development and disease. Here, we combined patient- ...

Animal and Cellular Models of Barth Syndrome : Research

Cardiomyocyte-specific TAFAZZIN KO mice display dilated cardiomyopathy with impaired but stable fractional shortening. Mitochondrial contact sites, the cristae ...

Barth syndrome cardiomyopathy: targeting the mitochondria with ...

Mitochondrial dysfunction therefore plays a central role in a wide variety of metabolic and cardiac disorders, including heart failure (HF) and ...

Barth Syndrome: From Mitochondrial Dysfunctions Associated with ...

Mutations in the gene encoding the enzyme tafazzin, TAZ, cause Barth syndrome (BTHS). Individuals with this X-linked multisystem disorder present ...

Cardiolipin Remodeling Defects Impair Mitochondrial Architecture ...

Cardiomyopathy is a major clinical feature in Barth syndrome (BTHS), an X-linked mitochondrial lipid disorder caused by mutations in Tafazzin (TAZ).

Barth syndrome cardiomyopathy - Oxford Academic

WT. Modeling the mitochondrial cardiomyopathy ... RW. Dysfunctional cardiac mitochondrial bioenergetic, lipidomic, and signaling in a murine model of Barth ...

Modeling the mitochondrial cardiomyopathy of Barth syndrome with ...

News. Modeling the mitochondrial cardiomyopathy of Barth syndrome with induced pluripotent stem cell and heart-on-chip technologies. Institute for Stem Cell ...

Barth Syndrome Cardiomyopathy: An Update - MDPI

Cardiolipin Remodeling Defects Impair Mitochondrial Architecture and Function in a Murine Model of Barth Syndrome Cardiomyopathy. Circ. Heart Fail. 2021, 14 ...

Mitochondrial Cardiomyopathy: Molecular Epidemiology, Diagnosis ...

Recently, a myocardium-specific Isca1 knockout rat model of multiple mitochondrial dysfunction syndromes (MMDS) complicated with cardiomyopathy was applied to ...

Your heart on a chip: iPSC-based modeling of Barth-syndrome ...

Modeling the mitochondrial cardiomyopathy of Barth syndrome with induced pluripotent stem cell and heart-on-chip technologies. ... Articles in the Open Access ...

Wang, G., Mccain, M.L., Yang, L., He, A. and Pu, W.T. (2014 ...

Wang, G., Mccain, M.L., Yang, L., He, A. and Pu, W.T. (2014) Modeling the Mitochondrial Cardiomyopathy of Barth Syndrome with iPSC and Heart-on-Chip ...

Modeling the mitochondrial cardiomyopathy of Barth syndrome with ...

Modeling the mitochondrial cardiomyopathy of Barth syndrome with induced pluripotent stem cell and heart-on-chip technologies. ... cardiac development and disease ...

How can we use stem cell-derived cardiomyocytes to understand ...

Cardiac dysfunctions studied via iPSC-CM include: I) Syndromes with cardiomyopathies caused by mutations in nuclear-encoded mitochondria- ...