Recessive pathogenic variants in MCAT cause combined oxidative phosphorylation deficiency

Webb BD, Nowinski SM, Solmonson A, Ganesh J, Rodenburg RJ, Leandro J, Evans A, Vu HS, Naidich TP, Gelb BD, DeBerardinis RJ, Rutter J, Houten SM

Elife. doi:10.7554/eLife.68047

The secret life of lactate: A novel cell-cycle regulatory mechanism

Cluntun AA, Rutter J

Cell Metab. doi: 10.1016/j.cmet.2023.04.006

Cardiac gene therapy treats diabetic cardiomyopathy and lowers blood glucose

Li J, Richmond B, Cluntun AA, Bia R, Walsh MA, Shaw K, Symons JD, Franklin S, Rutter J, Funai K, Shaw RM, Hong T

JCI Insight. doi: 10.1172/jci.insight.166713

Ceramides Increase Fatty Acid Utilization in Intestinal Progenitors to Enhance Stemness and Increase Tumor Risk

Li Y, Chaurasia B, Rahman MM, Kaddai V, Maschek JA, Berg JA, Wilkerson JL, Mahmassani ZS, Cox J, Wei P, Meikle PJ, Atkinson D, Wang L, Poss AM, Playdon MC, Tippetts TS, Mousa EM, Nittayaboon K, Anandh Babu PV, Drummond MJ, Clevers H, Shayman JA, Hirabayashi Y, Holland WL, Rutter J, Edgar BA, Summers SA

Gastroenterology. doi: 10.1053/j.gastro.2023.07.017

Metaboverse enables automated discovery and visualization of diverse metabolic regulatory patterns

Berg JA, Zhou Y, Ouyang Y, Cluntun AA, Waller TC, Conway ME, Nowinski SM, Van Ry T, George I, Cox JE, Wang B and Rutter J.

Nat. Cell Biol. doi: 10.1038/s41556-023-01117-9

Protein-metabolite interactomics of carbohydrate metabolism reveal regulation of lactate dehydrogenase

Hicks KG et al

Science. doi: 10.1126/science.abm3452

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Metabolites as Signalling Molecules

Baker SA and Rutter J

Nat. Rev. Mol. Cell Biol. doi: 10.1038/s41580-022-00572-w

Glyoxylate protects against cyanide toxicity through metabolic modulation

Nielson JR, Nath AK, Doane KP, Shi X, Lee J, Tippetts EG, Saha K, Morningstar J, Hicks KG, Chan A, Zhao Y, Kelly A, Hendry-Hofer TB, Witeof A, Sips PY, Mahon S, Bebarta VS, Davisson VJ, Boss GR, Rutter J, MacRae CA, Brenner M, Gerszten RE, Peterson RT

Sci Rep. 2022 Mar 23; doi: 10.1038/s41598-022-08803-y

Paradoxical neuronal hyperexcitability in a mouse model of mitochondrial pyruvate import deficiency

De La Rossa A, Laporte MH, Astori S, Marissal T, Montessuit S, Sheshadri P, Ramos-Fernández E, Mendez P, Khani A, Quairiaux C, Taylor EB, Rutter J, Nunes JM, Carleton A, Duchen MR, Sandi C, Martinou JC

Elife. 2022 Feb 21;11:e72595. doi: 10.7554/eLife.72595

Collateral deletion of the mitochondrial AAA+ ATPase ATAD1 sensitizes cancer cells to proteasome dysfunction

Winter JM, Fresenius HL, Cunningham CN, Wei P, Keys HR, Berg JA, Bott AJ, Yadav T, Ryan JA, Sirohi D, Tripp SR, Barta P, Agarwal N, Letai A, Sabatini DM, Wohelver M, and Rutter J

Elife 2022 Nov 21;11:e82860. doi: 10.7554/eLife.82860

Mitochondrial pyruvate supports lymphoma proliferation by fueling a glutamate pyruvate transaminase 2-dependent glutaminolysis pathway

Wei P, Bott AJ, Cluntun AA, Morgan JT, Cunningham CN, Schell JC, Ouyang Y, Ficarro SB, Marto JA, Danial NN, deBerardinis RJ, and Rutter J.

Sci. Adv 2022 Sep 30; doi: 10.1126/sciadv.abq0117. Epub 2022 Sep 30

Stressed to death: Mitochondrial stress responses connect respiration and apoptosis in cancer

Winter JM, Yadav T, and Rutter J.

Mol. Cell 2022 Aug 9;S1097-2765(22)00708-0. doi: 10.1016/j.molcel.2022.07.012

Protective mitochondrial fission induced by stress-responsive protein GJA1-20k

Shimura D, Nuebel E, Baum R, Valdez SE, Xiao S, Warren JS, Palatinus JA, Hong T, Rutter J, Shaw RM

Elife. 2021 Oct 5; doi: 10.7554/eLife.69207

Sugar phosphate activation of the stress sensor eIF2B

Hao Q, Heo JM, Nocek BP, Hicks KG, Stoll VS, Remarcik C, Hackett S, LeBon L, Jain R, Eaton D, Rutter J, Wong YL, Sidrauski C

Nat Commun. 2021 Jun 8; doi: 10.1038/s41467-021-23836-z

Maestro of the SereNADe: SLC25A51 Orchestrates Mitochondrial NAD

Ouyang Y, Bott AJ, Rutter J

Trends Biochem Sci. 2021May; doi: 10.1016/j.tibs.2021.02.001

The biochemical basis of mitochondrial dysfunction in Zellweger Spectrum Disorder

Nuebel E , Morgan JT , Fogarty S, Winter JM , Lettlova S , Berg JA , Chen YC , Kidwell CU , Maschek JA , Clowers KJ, Argyriou C, Chen L, Witting I, Cox JE, Roh-Johnson M, Braverman N, Bonkowsky J, Gygi SP, and Rutter J.

EMBO Rep. 2021 Aug 5;e51991. doi: 10.15252/embr.202051991.

The pyruvate-lactate axis modulates cardiac hypertrophy and heart failure

Cluntun AA, Badolia R, Lettlova S, Parnell KM, Shankar TS, Diakos NA, Olson KA, Taleb I, Tatum SM, Berg JA, Cunningham CN, Van Ry T, Bott AJ, Krokidi AT, Fogarty S, Skedros S, Swiatek WI, Yu X, Luo B, Merx S, Navankasattusas S, Cox JE, Ducker GS, Holland WL, McKellar SH, Rutter J, and Drakos SG.

Cell Metab. 2021 Mar 2; doi: 10.1016/j.cmet.2020.12.003. Epub 2020 Dec 16.

Callyspongiolide Is a Potent Inhibitor of the Vacuolar ATPase

Fogarty S, Ouyang Y, Liubo L, Chen YC, Rane H, Manoni F, Parra KJ, Rutter J and Harran PG.

J Nat Prod 2020 Nov5 doi: 10.1021

Mitochondrial fatty acid synthesis coordinates oxidative metabolism in mammalian mitochondria

Nowinski SM, Solmonson A, Rusin SF, Maschek JA, Bensard CL, Fogarty S, Jeong MY, Lettlova S, Berg JA, Morgan JT, Ouyang Y, Naylor BC, Paulo JA, Funai K, Cox JE, Gygi SP, Winge DR, DeBerardinis RJ and Rutter J.

Elife. 2020 Aug 17;9:e58041. doi: 10.7554/eLife.58041.

20,000 Picometers Under the OMM: Diving into the Vastness of Mitochondrial Metabolite Transport.

Cunningham CN and Rutter J.

EMBO Rep. 2020 Apr 23:e50071. doi: 10.15252/embr.202050071.

Reign in the membrane: How common lipids govern mitochondrial function.

Funai K, Summers SA, and Rutter J.

Curr Opin Cell Biol. 2020 Feb 24;63:162-173. doi: 10.1016/j.ceb.2020.01.006.

XPRESSyourself: Enhancing, standardizing, and automating ribosome profiling computational analyses yields improved insight into data.

Berg JA, Belyeu JR, Morgan JT, Ouyang Y, Bott AJ, Quinlan AR, Gertz J, Rutter J.

PLoS Comput Biol. 2020 Jan 31;16(1):e1007625. doi: 10.1371/journal.pcbi.1007625

Compartment and hub definitions tune metabolic networks for metabolomic interpretations.

Waller TC, Berg JA, Lex A, Chapman BE, Rutter J.

GigaScience, Volume 9, Issue 1, January 2020, giz137, https://doi.org/10.1093/gigascience/giz137

Targeting a ceramide double bond improves insulin resistance and hepatic steatosis

Chaurasia B, Tippetts TS, Mayoral Monibas R, Liu J, Li Y, Wang L, Wilkerson JL, Sweeney CR, Pereira RF, Sumida DH, Maschek JA, Cox JE, Kaddai V, Lancaster GI, Siddique MM, Poss A, Pearson M, Satapati S, Zhou H, McLaren DG, Previs SF, Chen Y, Qian Y, Petrov A, Wu M, Shen X, Yao J, Nunes CN, Howard AD, Wang L, Erion MD, Rutter J, Holland WL, Kelley DE, Summers SA

Science. 2019 Jul 26;365(6451):386-392.

doi: 10.1126/science.aav3722.

Regulation of Tumor Initiation by the Mitochondrial Pyruvate Carrier.

Bensard CL, Wisidigama DR, Olson KA, Berg JA, Krah NM, Schell JC, Nowinski SM, Fogarty S, Bott AJ, Wei P, Dove KK, Tanner JM, Panic V, Cluntun A, Lettlova S, Earl CS, Namnath DF, Vázquez-Arreguín K, Villanueva CJ, Tantin D, Murtaugh LC, Evason KJ, Ducker GS, Thummel CS, Rutter J.

Cell Metab. 2019 Dec 2. pii: S1550-4131(19)30609-6. doi: 10.1016/j.cmet.2019.11.002.

Vms1p is a release factor for the ribosome-associated quality control complex.

Zurita Rendón O, Fredrickson EK, Howard CJ, Van Vranken J, Fogarty S, Tolley ND, Kalia R, Osuna BA, Shen PS, Hill CP, Frost A, Rutter J. (2018)

Nat Commun. 2018 Jun 6;9(1):2197. doi: 10.1038/s41467-018-04564-3.

The Force Is Strong with This One: Metabolism (Over)powers Stem Cell Fate.

Wei P, Dove KK, Bensard C, Schell JC, Rutter J. (2018)

Trends Cell Biol. 2018 Jul;28(7):551-559. doi: 10.1016/j.tcb.2018.02.007. Epub 2018 Mar 16.

Lipid-mediated signals that regulate mitochondrial biology.

Nielson JR, Rutter JP. (2018)

J Biol Chem. 2018 May 18;293(20):7517-7521. doi: 10.1074/jbc.R117.001655. Epub 2018 Jan 18.

Global Analysis of Plasma Lipids Identifies Liver-Derived Acylcarnitines as a Fuel Source for Brown Fat Thermogenesis

Simcox J, Geoghegan G, Maschek JA, Bensard CL, Pasquali M, Miao R, Lee S, Jiang L, Huck I, Kershaw EE, Donato AJ, Apte U, Longo N, Rutter J, Schreiber R, Zechner R, Cox J, Villanueva CJ

Cell Metab. 2017 Sep 5;26(3):509-522.e6. doi: 10.1016/j.cmet.2017.08.006.

Structure of human Fe-S assembly subcomplex reveals unexpected cysteine desulfurase architecture and acyl-ACP-ISD11 interactions

Cory SA, Van Vranken JG, Brignole EJ, Patra S, Winge DR, Drennan CL, Rutter J, Barondeau DP

Proc Natl Acad Sci U S A. 2017 Jul 3;114(27):E5325-E5334. doi: 10.1073/pnas.1702849114.

Sterol Oxidation Mediates Stress-Responsive Vms1 Translocation to Mitochondria.

Nielson JR, Fredrickson EK, Waller TC, Rendón OZ, Schubert HL, Lin Z, Hill CP, Rutter J. (2017)

Mol Cell. 2017 Nov 16;68(4):673-685.e6. doi: 10.1016/j.molcel.2017.10.022.

Control of intestinal stem cell function and proliferation by mitochondrial pyruvate metabolism.

Schell JC, Wisidagama DR, Bensard C, Zhao H, Wei P, Tanner J, Flores A, Mohlman J, Sorensen LK, Earl CS, Olson KA, Miao R, Waller TC, Delker D, Kanth P, Jiang L, DeBerardinis RJ, Bronner MP, Li DY, Cox JE, Christofk HR, Lowry WE, Thummel CS, Rutter J. (2017)

Nat Cell Biol. 2017 Sep;19(9):1027-1036. doi: 10.1038/ncb3593. Epub 2017 Aug 14.

EWS/FLI is a Master Regulator of Metabolic Reprogramming in Ewing Sarcoma.

Tanner JM, Bensard C, Wei P, Krah NM, Schell JC, Gardiner J, Schiffman J, Lessnick SL, Rutter J. (2017)

Mol Cancer Res. 2017 Nov;15(11):1517-1530. doi: 10.1158/1541-7786.MCR-17-0182. Epub 2017 Jul 18.

Mitochondria link metabolism and epigenetics in haematopoiesis.

Schell JC, Rutter J. (2017)

Nat Cell Biol. 2017 May 31;19(6):589-591. doi: 10.1038/ncb3540.

Pask integrates hormonal signaling with histone modification via Wdr5 phosphorylation to drive myogenesis.

Kikani CK, Wu X, Paul L, Sabic H, Shen Z, Shakya A, Keefe A, Villanueva C, Kardon G, Graves B, Tantin D, Rutter J. (2016)

Elife. 2016 Sep 23;5. pii: e17985. doi: 10.7554/eLife.17985.

The mitochondrial acyl carrier protein (ACP) coordinates mitochondrial fatty acid synthesis with iron sulfur cluster biogenesis.

Van Vranken JG, Jeong MY, Wei P, Chen YC, Gygi SP, Winge DR, Rutter J. (2016)

Elife. 2016 Aug 19;5. pii: e17828. doi: 10.7554/eLife.17828.

Pyruvate and Metabolic Flexibility: Illuminating a Path Toward Selective Cancer Therapies.

Olson KA, Schell JC, Rutter J. (2016)

Trends Biochem Sci. 2016 Mar;41(3):219-230. doi: 10.1016/j.tibs.2016.01.002. Epub 2016 Feb 10.

You Down With ETC? Yeah, You Know D!

Van Vranken JG, Rutter J

Cell. 2015 Jul 30;162(3):471-3.

doi: 10.1016/j.cell.2015.07.027. PMID: 26232217.

Power(2): the power of yeast genetics applied to the powerhouse of the cell.

Rutter J, Hughes AL. (2015)

Trends Endocrinol Metab. 2015 Feb;26(2):59-68. doi: 10.1016/j.tem.2014.12.002. Epub 2015 Jan 12.

Protein-mediated assembly of succinate dehydrogenase and its cofactors.

Van Vranken JG, Na U, Winge DR, Rutter J.

Crit Rev Biochem Mol Biol. 2015 Mar-Apr;50(2):168-80.

The LYR factors SDHAF1 and SDHAF3 mediate maturation of the iron-sulfur subunit of succinate dehydrogenase

Na U, Yu W, Cox J, Bricker DK, Brockmann K, Rutter J, Thummel CS, Winge DR

Cell Metab. 2014 Aug 5;20(2):253-66.

doi: 10.1016/j.cmet.2014.05.014.

A role for the mitochondrial pyruvate carrier as a repressor of the Warburg effect and colon cancer cell growth.

Schell JC, Olson KA, Jiang L, Hawkins AJ, Van Vranken JG, Xie J, Egnatchik RA, Earl EG, DeBerardinis RJ, Rutter J.

Mol Cell. 2014 Nov 6;56(3):400-13.

PAS kinase drives lipogenesis through SREBP-1 maturation.

Wu X, Romero D, Swiatek WI, Dorweiler I, Kikani CK, Sabic H, Zweifel BS, McKearn J, Blitzer JT, Nickols GA, Rutter J.

Cell Rep. 2014 Jul 10;8(1):242-55.

SDHAF4 promotes mitochondrial succinate dehydrogenase activity and prevents neurodegeneration.

Van Vranken JG, Bricker DK, Dephoure N, Gygi SP, Cox JE, Thummel CS, Rutter J.

Cell Metab. 2014 Aug 5;20(2):241-52.

Msp1/ATAD1 maintains mitochondrial function by facilitating the degradation of mislocalized tail-anchored proteins.

Chen YC, Umanah GK, Dephoure N, Andrabi SA, Gygi SP, Dawson TM, Dawson VL, Rutter J.

EMBO J. 2014 Jul 17;33(14):1548-64.

Hallmarks of a new era in mitochondrial biochemistry.

Pagliarini DJ, Rutter J.

Genes Dev. 2013 Dec 15;27(24):2615-27.

Per-Arnt-Sim kinase regulates pancreatic duodenal homeobox-1 protein stability via phosphorylation of glycogen synthase kinase 3β in pancreatic β-cells.

Semache M, Zarrouki B, Fontés G, Fogarty S, Kikani C, Chawki MB, Rutter J, Poitout V.

J Biol Chem. 2013 Aug 23;288(34):24825-33.

Intramolecular interactions control Vms1 translocation to damaged mitochondria.

Heo JM, Nielson JR, Dephoure N, Gygi SP, Rutter J.

Mol Biol Cell. 2013 May;24(9):1263-73.

A mitochondrial pyruvate carrier required for pyruvate uptake in yeast, Drosophila, and humans.

Bricker DK, Taylor EB, Schell JC, Orsak T, Boutron A, Chen YC, Cox JE, Cardon CM, Van Vranken JG, Dephoure N, Redin C, Boudina S, Gygi SP, Brivet M, Thummel CS, Rutter J.

Science. 2012 Jul 6;337(6090):96-100.

Identification of a protein mediating respiratory supercomplex stability.

Chen YC, Taylor EB, Dephoure N, Heo JM, Tonhato A, Papandreou I, Nath N, Denko NC, Gygi SP, Rutter J.

Cell Metab. 2012 Mar 7;15(3):348-60.

PAS kinase promotes cell survival and growth through activation of Rho1.

Cardon CM, Beck T, Hall MN, Rutter J.

Sci Signal. 2012 Jan 31;5(209):ra9.

PAS kinase: integrating nutrient sensing with nutrient partitioning.

Cardon CM, Rutter J.

Semin Cell Dev Biol. 2012 Aug;23(6):626-30.

Revealing the allosterome: systematic identification of metabolite-protein interactions.

Orsak T, Smith TL, Eckert D, Lindsley JE, Borges CR, Rutter J.

Biochemistry. 2012 Jan 10;51(1):225-32.

Human mutation within Per-Arnt-Sim (PAS) domain-containing protein kinase (PASK) causes basal insulin hypersecretion

Semplici F, Vaxillaire M, Fogarty S, Semache M, Bonnefond A, Fontés G, Philippe J, Meur G, Diraison F, Sessions RB, Rutter J, Poitout V, Froguel P, Rutter GA

J Biol Chem. 2011 Dec 23;286(51):44005-44014.

doi: 10.1074/jbc.M111.254995.

Mitochondrial quality control by the ubiquitin-proteasome system.

Taylor EB, Rutter J.

Biochem Soc Trans. 2011 Oct;39(5):1509-13.

Ubiquitin-dependent mitochondrial protein degradation.

Heo JM, Rutter J.

Int J Biochem Cell Biol. 2011 Oct;43(10):1422-6.

Pancreatic and duodenal homeobox 1 (PDX1) phosphorylation at serine-269 is HIPK2-dependent and affects PDX1 subnuclear localization

An R, da Silva Xavier G, Semplici F, Vakhshouri S, Hao HX, Rutter J, Pagano MA, Meggio F, Pinna LA, Rutter GA

Biochem Biophys Res Commun. 2010 Aug 20

doi: 10.1016/j.bbrc.2010.07.035

A stress-responsive system for mitochondrial protein degradation.

Heo JM, Livnat-Levanon N, Taylor EB, Jones KT, Dephoure N, Ring J, Xie J, Brodsky JL, Madeo F, Gygi SP, Ashrafi K, Glickman MH, Rutter J.

Mol Cell. 2010 Nov 12;40(3):465-80.

Structural bases of PAS domain-regulated kinase (PASK) activation in the absence of activation loop phosphorylation.

Kikani CK, Antonysamy SA, Bonanno JB, Romero R, Zhang FF, Russell M, Gheyi T, Iizuka M, Emtage S, Sauder JM, Turk BE, Burley SK, Rutter J.

J Biol Chem. 2010 Dec 24;285(52):41034-43.

Succinate dehydrogenase - Assembly, regulation and role in human disease.

Rutter J, Winge DR, Schiffman JD.

Mitochondrion. 2010 Jun;10(4):393-401.

The role of PAS kinase in PASsing the glucose signal.

Grose JH, Rutter J.

Sensors (Basel). 2010;10(6):5668-82.

SDH5, a gene required for flavination of succinate dehydrogenase, is mutated in paraganglioma.

Hao HX, Khalimonchuk O, Schraders M, Dephoure N, Bayley JP, Kunst H, Devilee P, Cremers CW, Schiffman JD, Bentz BG, Gygi SP, Winge DR, Kremer H, Rutter J.

Science. 2009 Aug 28;325(5944):1139-42.

Regulation and function of yeast PAS kinase: a role in the maintenance of cellular integrity.

Grose JH, Sundwall E, Rutter J.

Cell Cycle. 2009 Jun 15;8(12):1824-32.

The role of PAS kinase in regulating energy metabolism.

Hao HX, Rutter J.

IUBMB Life. 2008 Apr;60(4):204-9.

Yeast PAS kinase coordinates glucose partitioning in response to metabolic and cell integrity signaling.

Grose JH, Smith TL, Sabic H, Rutter J.

EMBO J. 2007 Nov 28;26(23):4824-30.

PAS kinase is required for normal cellular energy balance.

Hao HX, Cardon CM, Swiatek W, Cooksey RC, Smith TL, Wilde J, Boudina S, Abel ED, McClain DA, Rutter J.

Proc Natl Acad Sci U S A. 2007 Sep 25;104(39):15466-71.

Regulation of glucose partitioning by PAS kinase and Ugp1 phosphorylation.

Smith TL, Rutter J.

Mol Cell. 2007 May 25;26(4):491-9.

Whence cometh the allosterome?

Lindsley JE, Rutter J.

Proc Natl Acad Sci U S A. 2006 Jul 11;103(28):10533-5.

Control of mammalian glycogen synthase by PAS kinase.

Wilson WA, Skurat AV, Probst B, de Paoli-Roach A, Roach PJ, Rutter J.

Proc Natl Acad Sci U S A. 2005 Nov 15;102(46):16596-601.

Nutrient sensing and metabolic decisions.

Lindsley JE, Rutter J.

Comp Biochem Physiol B Biochem Mol Biol. 2004 Dec;139(4):543-59.