Steven Michael Claypool

The paper identifies a bimodal m6A code in cardiac development, showing how m6A stoichiometry and context regulate metabolic switches and gene expression.

This study identifies Djp1 as a multifunctional Hsp40 cochaperone that regulates mitochondrial phospholipid metabolism through its interaction with Psd1 and independent roles.

This study identifies new lipid trafficking steps in mitochondria by rewiring phosphatidylethanolamine metabolism in yeast.

This study shows that TAFAZZIN deficiency disrupts mitochondrial maturation in cardiomyocytes, leading to structural and functional impairments associated with Barth syndrome cardiomyopathy.

This study shows that Tafazzin-mediated cardiolipin remodeling is essential for metabolic stress response and effector function of inflammatory T cells, with implications for Barth syndrome.

This study identifies Mdm38/LETM1 as a protein that links ion homeostasis and proteostasis in the mitochondrial inner membrane in yeast.

This study uses stem cell models to investigate tissue-specific pathologies in Barth Syndrome caused by TAFAZZIN deficiency, identifying mitophagy defects and potential therapeutic targets.

This study uses in-organello NMR to analyze how cardiolipin defects affect mitochondrial metabolism in yeast, showing altered metabolite kinetics and TCA cycle flux.

The paper identifies mitochondrial respiratory chain complex I and quality control dysregulation in TAZ-deficient cells and shows partial remediation via cardiolipin modulation.

The paper identifies PISD as a mitochondrial disease gene causing skeletal dysplasia, cataracts, and white matter changes through exome sequencing and functional analysis of patient fibroblasts.

The paper identifies a mitochondrial transporter interactome disruption in 22q11.2 syndrome, linking it to synapse function and behavior.