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Page 1
Triacylglycerol mobilization underpins mitochondrial stress recovery.
Baker ZN, Zhu Y, Guerra RM, Smith AJ, Arra A, Serrano LR, Overmyer KA, Mukherji S, Craig EA, Coon JJ, Pagliarini DJ. Baker ZN, et al. Among authors: pagliarini dj. Nat Cell Biol. 2025 Jan 8. doi: 10.1038/s41556-024-01586-6. Online ahead of print. Nat Cell Biol. 2025. PMID: 39779944
Identification of a new COQ4 spliceogenic variant causing severe primary coenzyme Q deficiency.
Alcázar-Fabra M, Østergaard E, Fernández-Ayala DJM, Desbats MA, Morbidoni V, Tomás-Gallado L, García-Corzo L, Blanquer-Roselló MDM, Bartlett AK, Sánchez-Cuesta A, Sena L, Cortés-Rodríguez A, Cascajo-Almenara MV, Pagliarini DJ, Trevisson E, Gronborg SW, Brea-Calvo G. Alcázar-Fabra M, et al. Among authors: pagliarini dj. Mol Genet Metab Rep. 2024 Dec 14;42:101176. doi: 10.1016/j.ymgmr.2024.101176. eCollection 2025 Mar. Mol Genet Metab Rep. 2024. PMID: 39759098 Free PMC article.
Fast and deep phosphoproteome analysis with the Orbitrap Astral mass spectrometer.
Lancaster NM, Sinitcyn P, Forny P, Peters-Clarke TM, Fecher C, Smith AJ, Shishkova E, Arrey TN, Pashkova A, Robinson ML, Arp N, Fan J, Hansen J, Galmozzi A, Serrano LR, Rojas J, Gasch AP, Westphall MS, Stewart H, Hock C, Damoc E, Pagliarini DJ, Zabrouskov V, Coon JJ. Lancaster NM, et al. Among authors: pagliarini dj. Nat Commun. 2024 Aug 15;15(1):7016. doi: 10.1038/s41467-024-51274-0. Nat Commun. 2024. PMID: 39147754 Free PMC article.
Missense variants in CMS22 patients reveal that PREPL has both enzymatic and nonenzymatic functions.
Monnens Y, Theodoropoulou A, Rosier K, Bhalla K, Mahy A, Vanhoutte R, Meulemans S, Cavani E, Antanasijevic A, Lemmens I, Lee JA, Spellicy CJ, Schroer RJ, Maselli RA, Laverty CG, Agostinis P, Pagliarini DJ, Verhelst S, Marcaida MJ, Rochtus A, Dal Peraro M, Creemers JW. Monnens Y, et al. Among authors: pagliarini dj. JCI Insight. 2024 Sep 10;9(17):e179276. doi: 10.1172/jci.insight.179276. JCI Insight. 2024. PMID: 39078710 Free PMC article.
Systematic analysis of NDUFAF6 in complex I assembly and mitochondrial disease.
Sung AY, Guerra RM, Steenberge LH, Alston CL, Murayama K, Okazaki Y, Shimura M, Prokisch H, Ghezzi D, Torraco A, Carrozzo R, Rötig A, Taylor RW, Keck JL, Pagliarini DJ. Sung AY, et al. Among authors: pagliarini dj. Nat Metab. 2024 Jun;6(6):1128-1142. doi: 10.1038/s42255-024-01039-2. Epub 2024 May 8. Nat Metab. 2024. PMID: 38720117 Free PMC article.
BRD4-mediated epigenetic regulation of endoplasmic reticulum-mitochondria contact sites is governed by the mitochondrial complex III.
Chen B, Stark DC, Jadhav PV, Lynn-Nguyen TM, Halligan BS, Rossiter NJ, Sindoni N, Shin M, Paulo JA, Chang M, Koo I, Koshkin S, Eyunni S, Ronchi P, Paulsen MT, Morlacchi P, Hanna DA, Lin J, Guerra RM, Pagliarini DJ, Banerjee R, Parolia A, Ljungman ME, Patterson AD, Mancias JD, Mosalaganti S, Sexton JZ, Calì T, Lyssiotis CA, Shah YM. Chen B, et al. Among authors: pagliarini dj. bioRxiv [Preprint]. 2024 Nov 5:2024.02.02.578646. doi: 10.1101/2024.02.02.578646. bioRxiv. 2024. PMID: 38352460 Free PMC article. Preprint.
COQ4 is required for the oxidative decarboxylation of the C1 carbon of coenzyme Q in eukaryotic cells.
Pelosi L, Morbiato L, Burgardt A, Tonello F, Bartlett AK, Guerra RM, Ferizhendi KK, Desbats MA, Rascalou B, Marchi M, Vázquez-Fonseca L, Agosto C, Zanotti G, Roger-Margueritat M, Alcázar-Fabra M, García-Corzo L, Sánchez-Cuesta A, Navas P, Brea-Calvo G, Trevisson E, Wendisch VF, Pagliarini DJ, Salviati L, Pierrel F. Pelosi L, et al. Among authors: pagliarini dj. Mol Cell. 2024 Mar 7;84(5):981-989.e7. doi: 10.1016/j.molcel.2024.01.003. Epub 2024 Jan 30. Mol Cell. 2024. PMID: 38295803 Free PMC article.
ACAD10 and ACAD11 enable mammalian 4-hydroxy acid lipid catabolism.
Rashan EH, Bartlett AK, Khana DB, Zhang J, Jain R, Smith AJ, Baker ZN, Cook T, Caldwell A, Chevalier AR, Pfleger BF, Yuan P, Amador-Noguez D, Simcox JA, Pagliarini DJ. Rashan EH, et al. Among authors: pagliarini dj. bioRxiv [Preprint]. 2024 Jan 9:2024.01.09.574893. doi: 10.1101/2024.01.09.574893. bioRxiv. 2024. PMID: 38260250 Free PMC article. Preprint.
94 results