D. J. Pagliarini

ACAD10 and ACAD11 enable mammalian 4-hydroxy acid lipid catabolism

This study identifies ACAD10 and ACAD11 as enzymes critical for metabolizing 4-hydroxy fatty acids in mammals, revealing their unique mechanisms and subcellular localization.

COQ4 is required for the oxidative decarboxylation of the C1 carbon of Coenzyme Q in eukaryotic cells

This study identifies COQ4 as catalyzing the oxidative decarboxylation of the C1 carbon in CoQ biosynthesis in eukaryotic cells.

Coenzyme Q4 is a functional substitute for coenzyme Q10 and can be targeted to the mitochondria

The paper shows CoQ4 can substitute for CoQ10 in cells and describes mitochondrial-targeting compounds that deliver CoQ4.

TOMM40 and TOMM22 of the Translocase Outer Mitochondrial Membrane Complex rescue statin-impaired mitochondrial dynamics, morphology, and mitophagy in skeletal myotubes

This study shows that statin-induced downregulation of TOMM40 and TOMM22 disrupts mitochondrial dynamics, morphology, and mitophagy in skeletal myotubes, and overexpression of these genes rescues these effects.

Hem25p is a mitochondrial IPP transporter

The paper identifies Hem25p as a mitochondrial IPP transporter in yeast, essential for CoQ biosynthesis.

Pptc7 maintains mitochondrial protein content by suppressing receptor-mediated mitophagy

This study shows that Pptc7 maintains mitochondrial protein content by suppressing receptor-mediated mitophagy, leading to metabolic defects and early lethality in mice.

Aim18p and Aim46p are CHI-domain-containing mitochondrial hemoproteins in Saccharomyces cerevisiae.

The paper identifies Aim18p and Aim46p as mitochondrial hemoproteins in yeast with structural similarities to CHI proteins but distinct functional roles.

Structure and functionality of a multimeric human COQ7:COQ9 complex

The paper presents the structure and functional analysis of a multimeric COQ7:COQ9 complex involved in CoQ biosynthesis, revealing its mechanism for accessing hydrophobic precursors and coordinating CoQ synthesis.

Small Molecule Modulation of the Archetypal UbiB protein COQ8

The paper develops small molecule inhibitors and activators for COQ8A, a UbiB family protein, using crystallography, NMR, and mass spectrometry to modulate its function in CoQ biosynthesis.

UbiB proteins regulate cellular CoQ distribution

This study identifies two mitochondrial proteins, Cqd1 and Cqd2, that regulate CoQ distribution and oxidative stress resistance in yeast.

Label-free Method for Classification of T cell Activation

This paper develops a label-free method using NAD(P)H and FAD autofluorescence imaging and machine learning to classify T cell activation states and subtypes with high accuracy.

Pptc7 is an essential phosphatase for promoting mammalian mitochondrial metabolism and biogenesis

This study shows that Pptc7 is essential for mitochondrial metabolism and biogenesis, as its deletion in mice causes metabolic defects and early death.

Post-Transcriptional Control of Coenzyme Q Biosynthesis Revealed by Transomic Analysis of the RNA-Binding Protein Puf3p

This study identifies Puf3p as a regulator of CoQ biosynthesis through transomic analysis, revealing its role in controlling Coq5p levels and mitochondrial biogenesis.