Featured Publications
The Human Knockout Gene CLYBL Connects Itaconate to Vitamin B12
Shen H, Campanello GC, Flicker D, Grabarek Z, Hu J, Luo C, Banerjee R, Mootha VK. The Human Knockout Gene CLYBL Connects Itaconate to Vitamin B12. Cell 2017, 171: 771-782.e11. PMID: 29056341, PMCID: PMC5827971, DOI: 10.1016/j.cell.2017.09.051.Peer-Reviewed Original ResearchConceptsMethylmalonyl-CoA mutaseCitramalyl-CoA lyaseAnti-microbial metabolitesCell-autonomous defectSubstrate analogue inhibitorsMammalian cellsStructural biologyPathway assignmentItaconyl-CoAMitochondrial enzymesCellular activitiesMetabolic pathwaysDependent methylmalonyl-CoA mutaseHomozygous lossConsistent phenotypeConsequence of exposureVertebratesCellsEnzymologyBiologyMutaseLyasePhenotypeEnzymePathway
2023
Dual regulation of SLC25A39 by AFG3L2 and iron controls mitochondrial glutathione homeostasis
Shi X, DeCiucis M, Grabinska K, Kanyo J, Liu A, Lam T, Shen H. Dual regulation of SLC25A39 by AFG3L2 and iron controls mitochondrial glutathione homeostasis. Molecular Cell 2023, 84: 802-810.e6. PMID: 38157846, PMCID: PMC10922821, DOI: 10.1016/j.molcel.2023.12.008.Peer-Reviewed Original ResearchProtein quality controlMitochondrial protein quality controlDual regulationMetabolic compartmentalizationIron homeostasisMitochondrial iron-sulfur clustersIron-sulfur clustersMitochondrial transportersProtein regulationMammalian cellsCRISPR knockoutCysteine residuesTransporter regulationLoop 1SLC25A39Glutathione homeostasisMetabolic sensingGlutathione uptakeMature neuronsProtein levelsHomeostasisRegulationAFG3L2Biochemical featuresMitochondrial glutathione levels