R. J. DeVita

Acyl-CoA dehydrogenase substrate promiscuity limits the potential for development of substrate reduction therapy in disorders of valine and isoleucine metabolism

This study investigates whether inhibiting ACAD8 and SBCAD can reduce toxic metabolite accumulation in valine and isoleucine metabolism disorders, finding that MCPA inhibits multiple ACADs and partially rescues lipoylation defects in ECHS1-deficient cells.

Human Beta Cell Mass Expansion In Vivo With A Harmine and Exendin-4 Combination: Quantification and Visualization By iDISCO+ 3D Imaging

This study shows a combination of DYRK1A inhibitor and GLP1R agonist increases human beta cell mass in vivo by 400-700% in mice, using iDISCO+ 3D imaging for quantification.

Inhibition and Crystal Structure of the Human DHTKD1-Thiamin Diphosphate Complex

This study identifies DHTKD1 inhibitors and reports its crystal structure with thiamin diphosphate, revealing how mutations affect its function and stability.

DHTKD1 and OGDH display in vivo substrate overlap and form a hybrid ketoacid dehydrogenase complex

The paper shows DHTKD1 and OGDH share in vivo substrate overlap and form a hybrid ketoacid dehydrogenase complex, with implications for GA1 therapy.