Janghoo Lim, PhD
Professor of Genetics and NeuroscienceDownloadHi-Res Photo
Cards
Appointments
Genetics
Fully Joint
Neuroscience
Fully Joint
Additional Titles
co-Director of Graduate Studies, Genetics
Contact Info
Appointments
Genetics
Fully Joint
Neuroscience
Fully Joint
Additional Titles
co-Director of Graduate Studies, Genetics
Contact Info
Appointments
Genetics
Fully Joint
Neuroscience
Fully Joint
Additional Titles
co-Director of Graduate Studies, Genetics
Contact Info
About
Titles
Professor of Genetics and Neuroscience
co-Director of Graduate Studies, Genetics
Biography
Janghoo Lim received his undergraduate and master’s degrees in South Korea. He completed his Ph.D. and postdoctoral training at Baylor College of Medicine in Houston, Texas. He joined Yale in 2010 and is currently a Professor in the Departments of Genetics and of Neuroscience.
Last Updated on July 02, 2025.
Appointments
Genetics
ProfessorFully JointNeuroscience
ProfessorFully Joint
Other Departments & Organizations
- Genetics
- Interdepartmental Neuroscience Program
- Molecular Cell Biology, Genetics and Development
- Neural Disorders
- Neuroscience
- Neuroscience Track
- Program in Cellular Neuroscience, Neurodegeneration and Repair
- Wu Tsai Institute
- Yale Combined Program in the Biological and Biomedical Sciences (BBS)
- Yale Stem Cell Center
- Yale Ventures
Education & Training
- Postdoctoral Fellow
- Baylor College of Medicine (2009)
- PhD
- Baylor College of Medicine (2004)
- MS
- Korea Advanced Institute of Science and Technology, Daejeon, Korea (1999)
- BS
- Chonnam National University, Gwangju, Korea (1997)
Research
Overview
Medical Research Interests
Developmental Disabilities; Mental Disorders; Motor Neuron Disease; Neurodegenerative Diseases; Neuromuscular Diseases; Spinocerebellar Ataxias
ORCID
0000-0002-5331-210X- View Lab Website
The Lim Lab at Yale
Research at a Glance
Yale Co-Authors
Frequent collaborators of Janghoo Lim's published research.
Publications Timeline
A big-picture view of Janghoo Lim's research output by year.
Research Interests
Research topics Janghoo Lim is interested in exploring.
Leon Tejwani, PhD
Benjamin Sanders
David van Dijk, PhD, MSc, BSc
Siyuan (Steven) Wang, PhD
Youngseob Jung
19Publications
278Citations
Spinocerebellar Ataxias
Neurodegenerative Diseases
Publications
2023
Longitudinal single-cell transcriptional dynamics throughout neurodegeneration in SCA1
Tejwani L, Ravindra N, Lee C, Cheng Y, Nguyen B, Luttik K, Ni L, Zhang S, Morrison L, Gionco J, Xiang Y, Yoon J, Ro H, Haidery F, Grijalva R, Bae E, Kim K, Martuscello R, Orr H, Zoghbi H, McLoughlin H, Ranum L, Shakkottai V, Faust P, Wang S, van Dijk D, Lim J. Longitudinal single-cell transcriptional dynamics throughout neurodegeneration in SCA1. Neuron 2023, 112: 362-383.e15. PMID: 38016472, PMCID: PMC10922326, DOI: 10.1016/j.neuron.2023.10.039.Peer-Reviewed Original ResearchCitationsAltmetricDysregulation of alternative splicing in spinocerebellar ataxia type 1
Olmos V, Thompson E, Gogia N, Luttik K, Veeranki V, Ni L, Sim S, Chen K, Krause D, Lim J. Dysregulation of alternative splicing in spinocerebellar ataxia type 1. Human Molecular Genetics 2023, 33: 138-149. PMID: 37802886, PMCID: PMC10979408, DOI: 10.1093/hmg/ddad170.Peer-Reviewed Original ResearchCitationsAltmetricMeSH Keywords and ConceptsConceptsAlternative splicing eventsSpinocerebellar ataxia type 1Splicing eventsAtaxin-1Ataxia type 1Mutant ataxin-1Alternative splicingGene expressionMisregulated alternative splicingCell-autonomous mannerDifferential gene expressionNew biological pathwaysMolecular mechanistic insightsDrosophila modelGenetic manipulationBulk RNABiological pathwaysPolyglutamine tractNeurodegenerative phenotypeAutonomous mannerMechanistic insightsSplicingPotential therapeutic strategyMouse cerebellumExpressionReduction of Nemo-like kinase increases lysosome biogenesis and ameliorates TDP-43-related neurodegeneration
Tejwani L, Jung Y, Kokubu H, Sowmithra S, Ni L, Lee C, Sanders B, Lee P, Xiang Y, Luttik K, Soriano A, Yoon J, Park J, Ro H, Ju H, Liao C, Tieze S, Rigo F, Jafar-Nejad P, Lim J. Reduction of Nemo-like kinase increases lysosome biogenesis and ameliorates TDP-43-related neurodegeneration. Journal Of Clinical Investigation 2023, 133: e138207. PMID: 37384409, PMCID: PMC10425213, DOI: 10.1172/jci138207.Peer-Reviewed Original ResearchCitationsAltmetricMeSH Keywords and ConceptsConceptsAmyotrophic lateral sclerosisTDP-43-related neurodegenerationNeurodegenerative disordersTransactive response DNA-binding protein 43Sporadic amyotrophic lateral sclerosisDNA-binding protein 43Subset of patientsTDP-43 speciesTDP-43 inclusionsDistinct mouse modelsTDP-43 proteinopathyFamilial amyotrophic lateral sclerosisNemo-like kinaseMultiple neurodegenerative disordersAutophagy/lysosome pathwayTDP-43-positive aggregatesALS patientsALS casesSporadic ALSPharmacological reductionProtein 43Lateral sclerosisMouse modelParkinson's diseaseTDP-43
2022
A Novel Missense Mutation in ERCC8 Co-Segregates with Cerebellar Ataxia in a Consanguineous Pakistani Family
Gauhar Z, Tejwani L, Abdullah U, Saeed S, Shafique S, Badshah M, Choi J, Dong W, Nelson-Williams C, Lifton RP, Lim J, Raja GK. A Novel Missense Mutation in ERCC8 Co-Segregates with Cerebellar Ataxia in a Consanguineous Pakistani Family. Cells 2022, 11: 3090. PMID: 36231052, PMCID: PMC9564319, DOI: 10.3390/cells11193090.Peer-Reviewed Original ResearchCitationsAltmetricMeSH Keywords and ConceptsConceptsAutosomal recessive cerebellar ataxiaCerebellar ataxiaProgressive gait ataxiaMagnetic resonance imagingT mutationHeterogeneous rare disordersNovel homozygous missense mutationWhole-exome sequencingMissense mutationsGait ataxiaMovement disordersDifferential diagnosisRare disorderCerebellar atrophyHomozygous missense mutationConsanguineous Pakistani familyNovel missense mutationResonance imagingBody imbalanceExome sequencingYoung adultsHomozygous mutationPakistani familyAtaxiaType AIdentifying Disease Signatures in the Spinocerebellar Ataxia Type 1 Mouse Cortex
Luttik K, Olmos V, Owens A, Khan A, Yun J, Driessen T, Lim J. Identifying Disease Signatures in the Spinocerebellar Ataxia Type 1 Mouse Cortex. Cells 2022, 11: 2632. PMID: 36078042, PMCID: PMC9454518, DOI: 10.3390/cells11172632.Peer-Reviewed Original ResearchCitationsAltmetricMeSH Keywords and ConceptsConceptsSCA1 mouse modelSpinocerebellar ataxia type 1Brain regionsMotor cortexMouse modelPurkinje cellsUnique gene expression changesCranial nerve nucleiBroad brain regionsSpecific neuronal populationsCerebellar Purkinje cellsInferior olive nucleusRegion-specific mechanismsCortical pathologyAtaxin-1Synaptic dysfunctionNerve nucleiSpinocerebellar tractSpinal cordProgressive degenerationTranscriptomic changesNeuronal populationsMouse cortexMutant ataxin-1Type 1Differential effects of Wnt-β-catenin signaling in Purkinje cells and Bergmann glia in spinocerebellar ataxia type 1
Luttik K, Tejwani L, Ju H, Driessen T, Smeets CJLM, Edamakanti CR, Khan A, Yun J, Opal P, Lim J. Differential effects of Wnt-β-catenin signaling in Purkinje cells and Bergmann glia in spinocerebellar ataxia type 1. Proceedings Of The National Academy Of Sciences Of The United States Of America 2022, 119: e2208513119. PMID: 35969780, PMCID: PMC9407543, DOI: 10.1073/pnas.2208513119.Peer-Reviewed Original ResearchCitationsAltmetricMeSH Keywords and ConceptsConceptsWnt-β-cateninSpinocerebellar ataxia type 1Ataxia type 1Cell typesWnt-β-catenin signalingWnt-β-catenin pathwayDifferent cell typesMultiple cell typesSCA1 mouse modelCerebellar cell populationsAtaxin-1Genetic manipulationCerebellar patterningBergmann gliaSCA1 pathogenesisSpecific neuronal populationsPurkinje cellsCerebellar neurodegenerationDistinct responsesCell populationsPathwayNeurodegenerative diseasesMouse cerebellumCritical roleActivationThe extra-cerebellar effects of spinocerebellar ataxia type 1 (SCA1): looking beyond the cerebellum
Olmos V, Gogia N, Luttik K, Haidery F, Lim J. The extra-cerebellar effects of spinocerebellar ataxia type 1 (SCA1): looking beyond the cerebellum. Cellular And Molecular Life Sciences 2022, 79: 404. PMID: 35802260, PMCID: PMC9993484, DOI: 10.1007/s00018-022-04419-7.Peer-Reviewed Reviews, Practice Guidelines, Standards, and Consensus StatementsCitationsAltmetricMeSH Keywords and ConceptsConceptsSpinocerebellar ataxia type 1Type 1Ataxia type 1Cerebellar Purkinje cell lossProgressive motor deficitsSCA1 patientsPurkinje cell lossMouse model studiesMotor deficitsLimb incoordinationNumber of CAGMouse modelRespiratory problemsMemory impairmentCell lossCerebellar regionsCognitive defectsNeurodegenerative diseasesPatientsAtaxin-1 proteinDiverse pathologiesATXN1 expressionCerebellumDiseaseFurther investigationExploring the Role of Posttranslational Modifications in Spinal and Bulbar Muscular Atrophy
Gogia N, Ni L, Olmos V, Haidery F, Luttik K, Lim J. Exploring the Role of Posttranslational Modifications in Spinal and Bulbar Muscular Atrophy. Frontiers In Molecular Neuroscience 2022, 15: 931301. PMID: 35726299, PMCID: PMC9206542, DOI: 10.3389/fnmol.2022.931301.Peer-Reviewed Reviews, Practice Guidelines, Standards, and Consensus StatementsCitationsAltmetricConceptsBulbar muscular atrophyAR proteinMuscular atrophyBulbar motor neuronsPolymorphic CAG trinucleotide repeatProgressive neuromuscular diseaseMain genetic causeMutant ARMotor neuronsTherapeutic approachesAR functionNeuromuscular diseaseProtective roleDisease pathophysiologyGenetic causeSkeletal muscleDiseaseCAG trinucleotide repeatAtrophySBMACell deathPolyQ tract expansionTractPolyQ disordersPolyglutamine expansionChapter 8 Exploring the role of protein quality control in aging and age-associated neurodegenerative diseases
Gogia N, Olmos V, Haidery F, Luttik K, Tejwani L, Lim J. Chapter 8 Exploring the role of protein quality control in aging and age-associated neurodegenerative diseases. 2022, 139-171. DOI: 10.1016/b978-0-323-90235-9.00012-4.ChaptersConceptsPQC systemAge-associated neurodegenerative diseasesAge-related neurodegenerative diseasesProtein quality control systemCell deathNeurodegenerative diseasesProtein quality controlHallmark of agingAlters cellular functionCellular proteomeCellular functionsProtein misfoldingQuality control systemCellular agingBiological processesPostmitotic natureNeuronal cell deathProteinDisease symptomsEfficient clearanceTherapeutic targetCellsDisease conditionsNervous system tissueEffective therapeutics
2021
Microglia regulate brain Progranulin levels through the endocytosis-lysosomal pathway
Dong T, Tejwani L, Jung Y, Kokubu H, Luttik K, Driessen TM, Lim J. Microglia regulate brain Progranulin levels through the endocytosis-lysosomal pathway. JCI Insight 2021, 6: e136147. PMID: 34618685, PMCID: PMC8663778, DOI: 10.1172/jci.insight.136147.Peer-Reviewed Original ResearchCitationsAltmetricMeSH Keywords and ConceptsConceptsPGRN levelsNovel potential therapeutic targetFrontotemporal lobar degenerationPotential therapeutic targetNeuronal ceroid lipofuscinosisPGRN deficiencyPGRN expressionLysosomal pathwayProgranulin levelsPathological changesHaploinsufficient miceTherapeutic targetMicrogliaNeuropathological phenotypeAlzheimer's diseaseProgranulinCeroid lipofuscinosisGlycoprotein progranulinNeurodegenerative diseasesDiseaseMiceGenetic alterationsNemo-like kinaseGenetic interaction studiesGenetic variants
Academic Achievements & Community Involvement
Honors
honor Blavatnik Accelerator Award
07/02/2025Yale University AwardBlavatnik Family FoundationDetailsUnited Stateshonor Young Investigator Award for SCA Research
01/01/2014International AwardNational Ataxia FoundationDetailsUnited Stateshonor Child Health Research Award
06/05/2012Regional AwardCharles H. Hood FoundationDetailsUnited Stateshonor Sloan Research Fellowship
02/15/2012International AwardAlfred P. Sloan FoundationDetailsUnited Stateshonor Young Investigator Award for SCA Research
01/01/2012International AwardNational Ataxia FoundationDetailsUnited States
News
News
- July 08, 2025Source: Yale Ventures
Eight Translational Biotech Projects Selected for 2025 Blavatnik Accelerator Awards
- March 20, 2024
From the journals
- January 09, 2024
The Most Popular Neuroscience Stories of 2023
- November 27, 2023
Yale Researchers Shed Light on the Progression of Neurodegeneration
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