Jong M. Rho, MD
Elizabeth Mears and House Jameson Professorship Of Pediatrics (Neurology)Cards
About
Research
Publications
2026
Symptom impact and safety of ketogenic therapy in adults with anorexia nervosa: a feasibility trial
Frank G, Scolnick B, Beckwith C, Shott M, Kilty S, Swindle S, Lutter M, Rho J. Symptom impact and safety of ketogenic therapy in adults with anorexia nervosa: a feasibility trial. Communications Medicine 2026, 6: 315. PMID: 42236862, PMCID: PMC13234144, DOI: 10.1038/s43856-026-01644-0.Peer-Reviewed Original ResearchKetogenic dietary therapiesDietary therapyKetogenic therapyWeight restorationCore symptomsEDE-QClinically significant weight lossRisk of relapseAnorexia nervosaCore symptoms of ANDepression scoresSignificant weight lossWorsening of symptomsEating Disorder Examination QuestionnaireEDE-Q global scoreEating Disorder SymptomsSymptoms of ANSevere psychiatric disordersEDE-Q scoresThree-month follow-upFollow-upSymptom scoresNormal rangeTherapyFood restriction
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 Reviews, Practice Guidelines, Standards, and Consensus StatementsConceptsFungal 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 Reviews, Practice Guidelines, Standards, and Consensus StatementsConceptsN-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.BooksKetogenic 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 disordersEpilepsy83 Pathophysiology of Seizures and Epilepsy
Stafstrom C, Rho J, Brumback A, Glykys J. 83 Pathophysiology of Seizures and Epilepsy. 2024, 770-776.e1. DOI: 10.1016/b978-0-443-10944-7.00083-4.Peer-Reviewed Original ResearchPathophysiological featuresCellular basisDysfunction of ion channelsPathophysiology of seizuresFeatures of seizuresCellular hyperexcitabilityNeuronal firingAntiseizure medicationsSynaptic transmissionSeizure activityNeuronal functionSeizuresIon channelsExcessive excitationEpilepsyHyperexcitabilityPathophysiologyDysfunctionSynaptically
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 profilesNeuronsCells
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