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
2008
Inhibition of ADAMTS-7 and ADAMTS-12 degradation of cartilage oligomeric matrix protein by alpha-2-macroglobulin
Luan Y, Kong L, Howell D, Ilalov K, Fajardo M, Bai X, Di Cesare P, Goldring M, Abramson S, Liu C. Inhibition of ADAMTS-7 and ADAMTS-12 degradation of cartilage oligomeric matrix protein by alpha-2-macroglobulin. Osteoarthritis And Cartilage 2008, 16: 1413-1420. PMID: 18485748, PMCID: PMC2574789, DOI: 10.1016/j.joca.2008.03.017.Peer-Reviewed Original ResearchConceptsCartilage oligomeric matrix proteinADAMTS-7ADAMTS-12COMP degradationOA patientsOligomeric matrix proteinIL-1betaTNF-alphaHuman chondrocytesCartilage explantsCultured cartilage explantsFirst endogenous inhibitorsArthritic patientsTumor necrosisMatrix proteinsCOMP fragmentsPatientsEndogenous inhibitorDependent mannerOsteoarthritisADAMTS familyAntibodiesInhibitionCartilageChondrocytes
2006
ADAMTS-12 Associates with and Degrades Cartilage Oligomeric Matrix Protein*
Liu C, Kong W, Xu K, Luan Y, Ilalov K, Sehgal B, Yu S, Howell R, Di Cesare P. ADAMTS-12 Associates with and Degrades Cartilage Oligomeric Matrix Protein*. Journal Of Biological Chemistry 2006, 281: 15800-15808. PMID: 16611630, PMCID: PMC1483932, DOI: 10.1074/jbc.m513433200.Peer-Reviewed Original ResearchConceptsCartilage oligomeric matrix proteinADAMTS-12Matrix proteinsOligomeric matrix proteinCOMP degradationNoncollagenous matrix componentsSynovium of patientsProgression of arthritisEpidermal growth factor-like repeat domainRepeat domainExtracellular matrix breakdownFunctional domainsArticular cartilageMolecular mechanismsArthritis patientsRheumatoid arthritisSynovial fluidMatrix breakdownArthritisMatrix componentsDegradative fragmentsPatientsSynoviumProteinNormal cartilage