Jong M. Rho, MD
Professor of Pediatrics (Neurology)Cards
About
Research
Publications
2025
The Interplay between the Gut and Ketogenic Diets in Health and Disease
Mu C, Rho J, Shearer J. The Interplay between the Gut and Ketogenic Diets in Health and Disease. Advanced Science 2025, 12: e04249. PMID: 40847749, PMCID: PMC12463008, DOI: 10.1002/advs.202504249.Peer-Reviewed Original ResearchConceptsFungal communitiesGut microbiomeGut physiologyIntracellular processesGutDietary signalsKetogenic dietIntestinal immunityOverall health benefitsMetabolism-based treatmentsHealth and DiseaseSystemic health outcomesMicrobiomePotential side effectsPrebioticsProbioticsSystemic health effectsDietary interventionSide effectsKetone esterMultiple sclerosisPhysiologyDietDiet benefitsInteractionAn international survey on withdrawing the ketogenic diet therapy for epilepsy
Donnard N, Höhn S, Devlin A, Kossoff E, Schoeler N, van der Louw E, Rho J, Dozières-Puyravel B, Auvin S. An international survey on withdrawing the ketogenic diet therapy for epilepsy. Epilepsy & Behavior 2025, 171: 110481. PMID: 40440787, DOI: 10.1016/j.yebeh.2025.110481.Peer-Reviewed Original ResearchConceptsKetogenic diet therapyDiet therapyEvidence-based dataDiet toleranceSeizure recurrenceProspective studyWeaning durationEEG monitoringSeizure controlAspects of treatmentEpilepsy syndromesHealthcare professionalsWeaning protocolAdherence issuesClinical decisionsTherapySeizuresEpilepsyWeaningDietary ratioDiverse global regionsMutant prion protein enhances NMDA receptor activity, activates PKC, and triggers rapid excitotoxicity in mice
Lin J, Callender J, Mayfield J, McClatchy D, Ojeda-Juárez D, Pourhamzeh M, Soldau K, Kurt T, Danque G, Khuu H, Ronson J, Pizzo D, Du Y, Gruber M, Sevillano A, Wang J, Orrú C, Chen J, Funk G, Aguilar-Calvo P, Aulston B, Roy S, Rho J, Bui J, Newton A, Lipton S, Caughey B, Patrick G, Doré K, Yates J, Sigurdson C. Mutant prion protein enhances NMDA receptor activity, activates PKC, and triggers rapid excitotoxicity in mice. Journal Of Clinical Investigation 2025, 135: e186432. PMID: 40185484, PMCID: PMC12077891, DOI: 10.1172/jci186432.Peer-Reviewed Original ResearchConceptsN-methyl-D-aspartate receptorsProtein kinase CAmino terminusPrion proteinN-methyl-D-aspartateMutant prion proteinNMDA receptor activationN-linked glycosylation sitesExcitatory-inhibitory imbalanceHippocampal pyramidal neuronsDownstream signaling eventsActivate protein kinase CNMDAR channelsNeuronal hyperexcitabilityFunctional motifsGlutamate receptorsCalcium influxPhosphoproteomic analysisPyramidal neuronsGlycosylation sitesSignaling eventsReceptor activationPrion-infected miceDendritic beadingSynapse loss
2024
Role of cholesterol in modulating brain hyperexcitability
Wheless J, Rho J. Role of cholesterol in modulating brain hyperexcitability. Epilepsia 2024, 66: 33-46. PMID: 39487852, PMCID: PMC11742637, DOI: 10.1111/epi.18174.Peer-Reviewed Original ResearchConceptsN-methyl-D-aspartateMultiple animal modelsPositive allosteric modulatorsEpileptic encephalopathyModulation of N-methyl-D-aspartateOverstimulation of NMDA receptorsAnimal models of epilepsyExcitatory amino acid transporter 2Amino acid transporter 2Models of epilepsyMetabolism of brain cholesterolBlood-brain barrierCentral nervous systemActive metabolismBrain cholesterolNMDA receptorsNeuronal hyperexcitabilityPrimary metabolic enzymesGlutamate releaseNeuronal excitabilityMechanism of actionExtracellular glutamateGlutamate reuptakeBrain hyperexcitabilityCholesterol 24-hydroxylaseExogenous ketones exert antiseizure effects and modulate the gut microbiome and mycobiome in a clinically relevant murine model of epilepsy
Mu C, Kesler M, Chen X, Shearer J, Teskey G, Rho J. Exogenous ketones exert antiseizure effects and modulate the gut microbiome and mycobiome in a clinically relevant murine model of epilepsy. Epilepsia 2024, 65: 3676-3688. PMID: 39412260, DOI: 10.1111/epi.18150.Peer-Reviewed Original ResearchConceptsGut microbiomeAntiseizure effectsOral gavageBacterial microbiomeSC injectionAntiseizure activitySeizure countsAmplicon sequence variantsClinically relevant murine modelHuman temporal lobe epilepsyExogenous ketonesFecal bacterial microbiomeRoute of administrationFungal communitiesFecal mycobiomeTemporal lobe epilepsySequence variantsTreatment of epilepsyKetone esterMycobiomeKcna1-nullKO miceMicrobiomeFemale miceMurine modelVocal communication in asocial BTBR mice is more malleable by a ketogenic diet in juveniles than adults
Möhrle D, Murari K, Rho J, Cheng N. Vocal communication in asocial BTBR mice is more malleable by a ketogenic diet in juveniles than adults. Neuroscience 2024, 561: 43-64. PMID: 39413868, DOI: 10.1016/j.neuroscience.2024.10.001.Peer-Reviewed Original ResearchConceptsAdult BTBR miceBTBR miceUltrasonic vocalizationsVocal communicationAutism spectrum disorderBTBR mouse strainCore symptomsLanguage developmentSpectrum disorderSocial communicationAutismSocial encountersSocial interactionBrief treatmentStructure of ultrasonic vocalizationsVocalizationsDeficitsEffects of KDTemporal structureAnimal modelsNeurological conditionsIntervention studiesMouse strainsBTBRIntractable epilepsyMechanisms of Ketogenic Diet Action
Williams R, Boison D, Masino S, Rho J. Mechanisms of Ketogenic Diet Action. 2024, 1635-1666. DOI: 10.1093/med/9780197549469.003.0079.Peer-Reviewed Original ResearchKetogenic dietMedium-chain triglyceride dietMetabolism-based treatmentsModulation of ion channelsInhibitory-excitatory balanceMedically intractable epilepsyGut microbiomeFatty acid modulationTriglyceride dietBlood metabolomeGABAergic neurotransmissionKD actionAntiepileptogenic effectsMitochondrial bioenergeticsAntiseizure effectsClinical effectsIntractable epilepsyMetabolic changesHormonal effectsClinical therapeuticsMechanistic frameworkIon channelsMetabolic basisNeurological disordersEpilepsy
2023
Human retinal ganglion cell neurons generated by synchronous BMP inhibition and transcription factor mediated reprogramming
Agarwal D, Dash N, Mazo K, Chopra M, Avila M, Patel A, Wong R, Jia C, Do H, Cheng J, Chiang C, Jurlina S, Roshan M, Perry M, Rho J, Broyer R, Lee C, Weinreb R, Gavrilovici C, Oesch N, Welsbie D, Wahlin K. Human retinal ganglion cell neurons generated by synchronous BMP inhibition and transcription factor mediated reprogramming. Npj Regenerative Medicine 2023, 8: 55. PMID: 37773257, PMCID: PMC10541876, DOI: 10.1038/s41536-023-00327-x.Peer-Reviewed Original ResearchRetinal ganglion cellsPluripotent stem cellsSmall molecule inhibitorsRetinal ganglion cell neuronsGanglion cell neuronsHuman pluripotent stem cellsOptic neuropathyCell transplantationRetinal repairAxonal injury modelElectrophysiological propertiesSynaptic transmissionCell replacementBMP inhibitionCell neuronsInjury modelStem cellsGanglion cellsNeuronal deathGene cassettesCellular reprogrammingBlock neuronal deathTranscriptional profilesNeuronsCellsPostictal hypoxia involves reactive oxygen species and is ameliorated by chronic mitochondrial uncoupling
Villa B, George A, Shutt T, Sullivan P, Rho J, Teskey G. Postictal hypoxia involves reactive oxygen species and is ameliorated by chronic mitochondrial uncoupling. Neuropharmacology 2023, 238: 109653. PMID: 37422182, DOI: 10.1016/j.neuropharm.2023.109653.Peer-Reviewed Original ResearchConceptsPostictal hypoxiaNovel object recognition taskObject recognition taskHippocampus of ratsPostictal cognitive impairmentCognitive deficitsCognitive dysfunctionCognitive impairmentMetabolic componentsRecognition taskProlonged severe hypoxiaSeizure inductionMitochondrial uncouplingPotential therapeutic strategyArteriole vasoconstrictionHippocampusMild mitochondrial uncouplingEvoked seizuresSeizure controlTherapeutic strategiesPostictal stateTherapeutic benefitPathological sequelaeMitochondrial functionTissue oxygenationIntermittent vs continuous ketogenic diet: Impact on seizures, gut microbiota, and mitochondrial metabolism
Shearer J, Scantlebury M, Rho J, Tompkins T, Mu C. Intermittent vs continuous ketogenic diet: Impact on seizures, gut microbiota, and mitochondrial metabolism. Epilepsia 2023, 64: e177-e183. PMID: 37335622, DOI: 10.1111/epi.17688.Peer-Reviewed Original ResearchConceptsGut microbiotaInfantile spasms syndromeKetogenic dietGut microbial alterationsNormal dietMitochondrial bioenergetic functionGut microbesSpasm frequencyFecal microbiotaMitochondrial metabolismNeonatal rat modelStreptococcus thermophilusBioenergetic functionMitochondrial bioenergeticsMicrobial alterationsMicrobiotaAnti-epileptic effectsGutNeonatal ratsAnti-epilepticsEpilepsy inductionMetabolic benefitsSpasmRatsMicrobes
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