Edmund R.S. Kunji

CLN6 is a glycerophosphoglycerol epimerase in bis(monoacylglycero)phosphate biosynthesis

This study identifies CLN6 as a glycerophosphoglycerol epimerase essential for BMP biosynthesis, showing its deficiency disrupts lysosomal hydrolase homeostasis and can be reversed by BMP precursor supplementation.

Tissue-wide metabolic buffering confers resilience to mitochondrial dysfunction

The paper shows that tissue-wide metabolic buffering allows neural stem cells to maintain proliferation despite mitochondrial dysfunction, highlighting the role of tissue heterogeneity in resilience.

Stereospecific GPG acylation by CLN8 drives BMP biosynthesis and its loss leads to Batten disease

This study identifies CLN8 as a stereospecific GPG acyltransferase critical for BMP biosynthesis, showing its loss causes Batten disease and that S,S-LPG supplementation can restore BMP and improve neurological symptoms.

Molecular basis of mitochondrial leucine transport by human Sideroflexin 1

The paper identifies leucine as the substrate for SFXN1 and reveals its alternating-access transport mechanism using structural and biochemical analyses.

Mitochondrial carrier SLC25A34 links clock, diet, and temperature control of interorganellar lipid cycling

This paper identifies SLC25A34 as a key regulator of interorganellar lipid cycling, integrating circadian rhythms, diet, and temperature to control thermogenic metabolism.

Unique mitochondrial carrier has a role in mitochondrial dynamics during Plasmodium falciparum host switching

The paper investigates the role of PfAMC1 in mitochondrial dynamics during Plasmodium falciparum host switching, showing its localization and impact on mitochondrial morphology.

Key features of inhibitor binding to the human mitochondrial pyruvate carrier hetero-dimer

The paper identifies key chemical features of inhibitors binding to the human mitochondrial pyruvate carrier hetero-dimer and discovers new inhibitors with higher potency than existing ones.