Featured Publications
Association Between Progranulin and Gaucher Disease
Jian J, Zhao S, Tian QY, Liu H, Zhao Y, Chen WC, Grunig G, Torres PA, Wang BC, Zeng B, Pastores G, Tang W, Sun Y, Grabowski GA, Kong MX, Wang G, Chen Y, Liang F, Overkleeft HS, Saunders-Pullman R, Chan GL, Liu CJ. Association Between Progranulin and Gaucher Disease. EBioMedicine 2016, 11: 127-137. PMID: 27515686, PMCID: PMC5049935, DOI: 10.1016/j.ebiom.2016.08.004.Peer-Reviewed Original ResearchMeSH KeywordsAdultAgedAllelesAnimalsCase-Control StudiesDisease Models, AnimalEnzyme ActivationFemaleGaucher DiseaseGene FrequencyGenetic Association StudiesGenotypeHumansIntercellular Signaling Peptides and ProteinsLysosomesMaleMiceMice, KnockoutMiddle AgedMutationPhenotypePolymorphism, Single NucleotideProgranulinsProtein TransportConceptsGD patientsHealthy controlsGaucher diseaseGRN genePGRN KO miceSerum PGRN levelsPGRN-deficient miceHealthy control samplesSerum levelsPGRN levelsGaucher-like cellsKO miceTherapeutic effectRecombinant PGRNGeneral populationPatientsAnimal modelsBone marrowGBA1 geneLysosomal localizationProgranulin geneNull micePGRNDiseaseMice
2018
Chitinase-3-like Protein 1: A Progranulin Downstream Molecule and Potential Biomarker for Gaucher Disease
Jian J, Chen Y, Liberti R, Fu W, Hu W, Saunders-Pullman R, Pastores G, Chen Y, Sun Y, Grabowski G, Liu C. Chitinase-3-like Protein 1: A Progranulin Downstream Molecule and Potential Biomarker for Gaucher Disease. EBioMedicine 2018, 28: 251-260. PMID: 29396296, PMCID: PMC5835567, DOI: 10.1016/j.ebiom.2018.01.022.Peer-Reviewed Original ResearchConceptsGD patientsHealthy controlsGaucher diseaseDownstream moleculesExpression of CHI3L1Serum CHI3L1Serum levelsPGRN levelsTherapeutic effectClinical biomarkersPatientsPotential biomarkersNull miceCHI3L1ProgranulinElevated levelsBiomarkersWhole-genome microarraysDiseaseCHIT1Genome microarraysNovel regulatorImmunohistochemistryLevelsMice
2017
Progranulin acts as a shared chaperone and regulates multiple lysosomal enzymes
Jian J, Hettinghouse A, Liu C. Progranulin acts as a shared chaperone and regulates multiple lysosomal enzymes. Genes & Diseases 2017, 4: 125-126. PMID: 28944282, PMCID: PMC5609500, DOI: 10.1016/j.gendis.2017.05.001.Peer-Reviewed Original ResearchMultiple lysosomal enzymesLysosomal enzymesE domainLysosomal enzyme cathepsin DLysosomal storage diseaseLysosomal enzyme β-glucocerebrosidaseEnzyme cathepsin DC-terminusChaperonesMolecular mechanismsChaperone moleculesEnzyme β-glucocerebrosidaseNeuronal ceroid lipofuscinosisStorage diseaseGaucher diseaseNull miceEnzymeCeroid lipofuscinosisCathepsin DGene variantsMutationsΒ-glucocerebrosidaseRole of progranulinImportant roleProgranulin
2016
Progranulin inhibits expression and release of chemokines CXCL9 and CXCL10 in a TNFR1 dependent manner
Mundra J, Jian J, Bhagat P, Liu C. Progranulin inhibits expression and release of chemokines CXCL9 and CXCL10 in a TNFR1 dependent manner. Scientific Reports 2016, 6: 21115. PMID: 26892362, PMCID: PMC4759551, DOI: 10.1038/srep21115.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsCD4-Positive T-LymphocytesChemokine CXCL10Chemokine CXCL9Cluster AnalysisDermatitis, ContactGene Expression ProfilingGene Expression RegulationIntercellular Signaling Peptides and ProteinsMacrophagesMiceMice, KnockoutProgranulinsReceptors, Tumor Necrosis Factor, Type IRecombinant ProteinsConceptsWild-type B6 micePGRN KO miceRecombinant PGRN proteinTNFR1-dependent mannerSeverity of inflammationInflammatory bowel diseaseAnti-inflammatory functionalityCXCL9 expressionTreg populationGene array analysisBowel diseaseDermatitis modelRheumatoid arthritisChemokine expressionChemokines CXCL9Autoimmune diseasesB6 micePGRN functionCXCL10 expressionKO miceT cellsPleiotrophic growth factorsImmune systemProgranulinNull mice