2024
VavCre mediated conditional deletion of Gba in mice recapitulates human Gaucher disease type 1, a platform to investigate the role of myeloid cells and altered hematopoiesis
Nair S, Belinsky G, Mistry P. VavCre mediated conditional deletion of Gba in mice recapitulates human Gaucher disease type 1, a platform to investigate the role of myeloid cells and altered hematopoiesis. Molecular Genetics And Metabolism 2024, 141: 107978. DOI: 10.1016/j.ymgme.2023.107978.Peer-Reviewed Original Research
2023
Severe pulmonary arterial hypertension in Gaucher disease type 1
Basiri M, Ruan J, Nair S, Guo L, Mistry P. Severe pulmonary arterial hypertension in Gaucher disease type 1. Molecular Genetics And Metabolism 2023, 138: 107021. DOI: 10.1016/j.ymgme.2022.107021.Peer-Reviewed Original ResearchThe risk of hepatocellular carcinoma is markedly increased in Gaucher disease
Basiri M, Ruan J, Nair S, Lau H, Mistry P, Taddei T. The risk of hepatocellular carcinoma is markedly increased in Gaucher disease. Molecular Genetics And Metabolism 2023, 138: 107022. DOI: 10.1016/j.ymgme.2022.107022.Peer-Reviewed Original ResearchSingle cell resolution of neurodegeneration in Gaucher disease
Nair S, Belinsky G, Ruan J, Basiri M, Klinger K, Mistry P. Single cell resolution of neurodegeneration in Gaucher disease. Molecular Genetics And Metabolism 2023, 138: 107233. DOI: 10.1016/j.ymgme.2022.107233.Peer-Reviewed Original Research
2022
Neuroinflammation in neuronopathic Gaucher disease: Role of microglia and NK cells, biomarkers, and response to substrate reduction therapy
Boddupalli CS, Nair S, Belinsky G, Gans J, Teeple E, Nguyen TH, Mehta S, Guo L, Kramer ML, Ruan J, Wang H, Davison M, Kumar D, Vidyadhara D, Zhang B, Klinger K, Mistry PK. Neuroinflammation in neuronopathic Gaucher disease: Role of microglia and NK cells, biomarkers, and response to substrate reduction therapy. ELife 2022, 11: e79830. PMID: 35972072, PMCID: PMC9381039, DOI: 10.7554/elife.79830.Peer-Reviewed Original ResearchConceptsNeuronopathic Gaucher diseaseAmelioration of neuroinflammationNK cellsGaucher diseaseSerum neurofilament light chainInvolvement of microgliaActivation of microgliaRole of microgliaProminent pathological featureNeurofilament light chainBlood-derived macrophagesRare neurodegenerative disorderGlucosylceramide synthaseNeuroinflammation pathwaysSerum NFMicroglia activationNeuronal injuryImmune infiltratesImproved survivalBrain macrophagesPathological featuresGD patientsClinical trialsMacrophage compartmentPatient management
2021
The clinical spectrum of SARS-CoV-2 infection in Gaucher disease: Effect of both a pandemic and a rare disease that disrupts the immune system
Narayanan P, Nair S, Balwani M, Malinis M, Mistry P. The clinical spectrum of SARS-CoV-2 infection in Gaucher disease: Effect of both a pandemic and a rare disease that disrupts the immune system. Molecular Genetics And Metabolism 2021, 135: 115-121. PMID: 34412940, PMCID: PMC8361210, DOI: 10.1016/j.ymgme.2021.08.004.Peer-Reviewed Case Reports and Technical NotesConceptsSARS-CoV-2 infectionType 1 Gaucher diseaseSARS-CoV-2Gaucher diseaseRare diseaseCOVID-19Immune system dysfunctionRare disease populationMedian agePediatric patientsCase seriesFemale patientsAdverse outcomesClinical spectrumIntensive careGD patientsSystem dysfunctionRetrospective analysisDisease populationHigh riskGeneral populationPatientsImmune systemDiseaseSimilar frequency
2020
Glucosylsphingosine but not Saposin C, is the target antigen in Gaucher disease-associated gammopathy
Nair S, Bar N, Xu ML, Dhodapkar M, Mistry PK. Glucosylsphingosine but not Saposin C, is the target antigen in Gaucher disease-associated gammopathy. Molecular Genetics And Metabolism 2020, 129: 286-291. PMID: 32044242, PMCID: PMC8223251, DOI: 10.1016/j.ymgme.2020.01.009.Peer-Reviewed Original ResearchConceptsGaucher disease type 1Monoclonal gammopathyAntigenic targetsClonal immunoglobulinDisease type 1B cell activationAccumulation of glucosylceramideGD1 patientsImmunogenic lipidsMetabolic inflammationMultiple myelomaGD patientsHigh riskTarget antigenCell activationImmunoglobulin typeGammopathyType 1PatientsGenetic deficiencyAge-related phenotypesSaposin CClonal IgLysosomal glucocerebrosidaseGlcSphDelineating the role of myeloid cells and brain microglia in Gaucher disease
Boddupalli V, Nair S, Belinsky G, Mehta S, Mistry P. Delineating the role of myeloid cells and brain microglia in Gaucher disease. Molecular Genetics And Metabolism 2020, 129: s31. DOI: 10.1016/j.ymgme.2019.11.054.Peer-Reviewed Original Research
2018
Antigen-mediated regulation in monoclonal gammopathies and myeloma
Nair S, Sng J, Boddupalli CS, Seckinger A, Chesi M, Fulciniti M, Zhang L, Rauniyar N, Lopez M, Neparidze N, Parker T, Munshi NC, Sexton R, Barlogie B, Orlowski R, Bergsagel L, Hose D, Flavell RA, Mistry PK, Meffre E, Dhodapkar MV. Antigen-mediated regulation in monoclonal gammopathies and myeloma. JCI Insight 2018, 3: e98259. PMID: 29669929, PMCID: PMC5931125, DOI: 10.1172/jci.insight.98259.Peer-Reviewed Original ResearchConceptsMultiple myelomaPlasma cellsGaucher diseaseAntigenic stimulationMonoclonal gammopathyAntigen-driven stimulationMonoclonal IgClonal IgB cell receptorSingle tumor cellsPatient cohortUndetermined significanceVivo responsivenessMalignant cloneGammopathyTumor growthMonoclonal tumorsCell receptorTumor cellsAntigenGene expression profilingStimulationClonal natureMyelomaTumors
2017
Natural Killer T Cells in Cancer Immunotherapy
Nair S, Dhodapkar MV. Natural Killer T Cells in Cancer Immunotherapy. Frontiers In Immunology 2017, 8: 1178. PMID: 29018445, PMCID: PMC5614937, DOI: 10.3389/fimmu.2017.01178.Peer-Reviewed Original ResearchAssociation of lysolipids with Gaucher disease - associated and sporadic gammopathies
Nair S, Sng J, Zhang L, Branagan A, Liu J, Boddupalli C, Meffre E, Mistry P, Dhodapkar M. Association of lysolipids with Gaucher disease - associated and sporadic gammopathies. Molecular Genetics And Metabolism 2017, 120: s100. DOI: 10.1016/j.ymgme.2016.11.251.Peer-Reviewed Original Research
2016
ABC transporters and NR4A1 identify a quiescent subset of tissue-resident memory T cells
Boddupalli CS, Nair S, Gray SM, Nowyhed HN, Verma R, Gibson JA, Abraham C, Narayan D, Vasquez J, Hedrick CC, Flavell RA, Dhodapkar KM, Kaech SM, Dhodapkar MV. ABC transporters and NR4A1 identify a quiescent subset of tissue-resident memory T cells. Journal Of Clinical Investigation 2016, 126: 3905-3916. PMID: 27617863, PMCID: PMC5096804, DOI: 10.1172/jci85329.Peer-Reviewed Original ResearchConceptsTissue-resident memory T cellsMemory T cellsT cellsTRM cellsCellular therapyAdoptive cellular therapyImmune-deficient micePotential cellular therapySP T cellsSide population cellsHuman T cellsPutative subsetsAdoptive transferDistinct gene expression profilesCell mobilizationImmune surveillanceQuiescent subsetPopulation cellsMiceTherapyQuiescent phenotypeDistinct subsetsMember 1Nuclear receptorsSignature genesClonal Immunoglobulin against Lysolipids in the Origin of Myeloma
Nair S, Branagan AR, Liu J, Boddupalli CS, Mistry PK, Dhodapkar MV. Clonal Immunoglobulin against Lysolipids in the Origin of Myeloma. New England Journal Of Medicine 2016, 374: 555-561. PMID: 26863356, PMCID: PMC4804194, DOI: 10.1056/nejmoa1508808.Peer-Reviewed Original Research
2014
Type II NKT-TFH cells against Gaucher lipids regulate B-cell immunity and inflammation
Nair S, Boddupalli CS, Verma R, Liu J, Yang R, Pastores GM, Mistry PK, Dhodapkar MV. Type II NKT-TFH cells against Gaucher lipids regulate B-cell immunity and inflammation. Blood 2014, 125: 1256-1271. PMID: 25499455, PMCID: PMC4335081, DOI: 10.1182/blood-2014-09-600270.Peer-Reviewed Original ResearchConceptsI NKT cellsNKT cellsB cell activationT cellsB cellsTetramer-positive T cellsType II natural killer T cellsT cell receptor usageType I NKT cellsType II NKT cellsNatural killer T cellsFollicular helper phenotypeGD mouse modelMetabolic lipid disordersKiller T cellsB cell immunityGerminal center B cellsB-cell malignanciesAntilipid antibodiesDisease activityCytokine profileChronic inflammationHelper phenotypeHumoral immunityCognate helpType II NKT-TFH Cells Against Gaucher Lipids Regulate B Cell Immunity and Inflammation
Nair S, Boddupalli C, Verma R, Liu J, Ruhua Y, Pastores G, Mistry P, Dhodapkar M. Type II NKT-TFH Cells Against Gaucher Lipids Regulate B Cell Immunity and Inflammation. Blood 2014, 124: 755. DOI: 10.1182/blood.v124.21.755.755.Peer-Reviewed Original ResearchType II NKT cellsType I NKT cellsLipid-specific T cellsI NKT cellsNKT cellsB cell immunityT cellsTFH markersAnti-lipid antibodiesΑ-GalCerGaucher diseaseB cellsCD1d tetramersTFH phenotypeCell immunityHuman PBMCsGD miceMHC-like molecule CD1dNatural killer T cellsWild-type control miceCD1d-dependent mannerDiverse NKT cellsExpansion of plasmablastsFollicular helper phenotypeAutologous B cells
2013
Endocytosis of Mycobacterium tuberculosis Heat Shock Protein 60 Is Required to Induce Interleukin-10 Production in Macrophages*
Parveen N, Varman R, Nair S, Das G, Ghosh S, Mukhopadhyay S. Endocytosis of Mycobacterium tuberculosis Heat Shock Protein 60 Is Required to Induce Interleukin-10 Production in Macrophages*. Journal Of Biological Chemistry 2013, 288: 24956-24971. PMID: 23846686, PMCID: PMC3750191, DOI: 10.1074/jbc.m113.461004.Peer-Reviewed Original ResearchConceptsToll-like receptorsIL-10 productionPro-inflammatory responseHeat shock protein 60Shock protein 60Immune responseInnate responseHost protective immune responsesProtein 60Interleukin-10 productionType immune responseProtective immune responseProduction of interleukinAnti-tuberculosis immunityTumor necrosis factorInnate immune responseMacrophage innate responsesMycobacterium tuberculosis bacteriaTLR4 receptorNecrosis factorP38 MAPK activationTLR2M. tuberculosisTuberculosis bacteriaP38 MAPK
2012
Clinical regressions and broad immune activation following combination therapy targeting human NKT cells in myeloma
Richter J, Neparidze N, Zhang L, Nair S, Monesmith T, Sundaram R, Miesowicz F, Dhodapkar KM, Dhodapkar MV. Clinical regressions and broad immune activation following combination therapy targeting human NKT cells in myeloma. Blood 2012, 121: 423-430. PMID: 23100308, PMCID: PMC3548165, DOI: 10.1182/blood-2012-06-435503.Peer-Reviewed Original ResearchConceptsHuman iNKT cellsINKT cellsCombination therapyAntitumor T-cell immunitySerum soluble IL2 receptorMonocyte-derived dendritic cellsNatural killer T cellsBroad immune activationLow-dose lenalidomideSoluble IL2 receptorCycles of therapyHuman NKT cellsT cell immunityKiller T cellsInnate immune cellsInduction of eosinophiliaActivation of monocytesPrevention of cancerCycles of combinationAsymptomatic myelomaClinical myelomaMeasurable diseaseClinical regressionClinical responseNKT cellsPathogenesis in tuberculosis: transcriptomic approaches to unraveling virulence mechanisms and finding new drug targets
Mukhopadhyay S, Nair S, Ghosh S. Pathogenesis in tuberculosis: transcriptomic approaches to unraveling virulence mechanisms and finding new drug targets. FEMS Microbiology Reviews 2012, 36: 463-485. PMID: 22092372, DOI: 10.1111/j.1574-6976.2011.00302.x.Peer-Reviewed Original ResearchConceptsMajor health problemHost immune responseEmergence of strainsDrug targetsDendritic cellsEffective drug targetsInhibition of apoptosisImmune responseNew drug targetsHealth problemsLipid metabolismMultiple drugsTuberculosisComplex etiologyMycobacterium tuberculosisIntracellular life styleTranscriptome signaturesVirulence mechanismsGenome-wide expression profilingDrugsAntibiotic drugsExpression profilingProtein secretionLife stylePoor understanding
2011
The PPE18 Protein of Mycobacterium tuberculosis Inhibits NF-κB/rel–Mediated Proinflammatory Cytokine Production by Upregulating and Phosphorylating Suppressor of Cytokine Signaling 3 Protein
Nair S, Pandey AD, Mukhopadhyay S. The PPE18 Protein of Mycobacterium tuberculosis Inhibits NF-κB/rel–Mediated Proinflammatory Cytokine Production by Upregulating and Phosphorylating Suppressor of Cytokine Signaling 3 Protein. The Journal Of Immunology 2011, 186: 5413-5424. PMID: 21451109, DOI: 10.4049/jimmunol.1000773.Peer-Reviewed Original ResearchMeSH KeywordsAntigens, BacterialBacterial ProteinsBlotting, WesternCell LineCell SeparationCytokinesElectrophoretic Mobility Shift AssayFlow CytometryHumansImmunoprecipitationInflammationMacrophagesMycobacterium tuberculosisNF-kappa BPhosphorylationReverse Transcriptase Polymerase Chain ReactionRNA, Small InterferingSignal TransductionSuppressor of Cytokine Signaling 3 ProteinSuppressor of Cytokine Signaling ProteinsTranscription Factor RelATransfectionTuberculosisUp-RegulationConceptsTranscription factorsNF-κB/Rel transcription factorsNuclear translocationNF-κB/RelRel transcription factorsSubsequent intracellular survivalC-Rel transcription factorPhosphorylation sitesTyrosine phosphorylationSpecific knockdownSuccessful infectionIntracellular survivalRel subunitsCytokine signaling-3 (SOCS-3) proteinNovel mechanismIκB kinasePhosphorylationProteinNuclear levelsNF-κB activationSOCS3Phosphorylation of IκBαSuppressorTNF-α productionTranslocation
2010
Glutathione-Redox Balance Regulates c-rel–Driven IL-12 Production in Macrophages: Possible Implications in Antituberculosis Immunotherapy
Alam K, Ghousunnissa S, Nair S, Valluri VL, Mukhopadhyay S. Glutathione-Redox Balance Regulates c-rel–Driven IL-12 Production in Macrophages: Possible Implications in Antituberculosis Immunotherapy. The Journal Of Immunology 2010, 184: 2918-2929. PMID: 20164428, DOI: 10.4049/jimmunol.0900439.Peer-Reviewed Original ResearchConceptsIL-12 productionImmune responseGlutathione redox balanceEnhanced Th1 responseProtective Th1 typeTNF-alpha levelsCellular immune responsesIFN-gamma productionIL-12 inductionBacillus Calmette-GuérinIL-12 cytokinesActive tuberculosisTh1 responseTh1 typeGlutathione ethyl esterNAC treatmentP70 productionCalmette-GuérinIntracellular reduced glutathioneP65 NF-kappaB.Pathophysiological disordersNF-kappaB.IkappaBalpha phosphorylationGSH donorMacrophages