Featured Publications
Progranulin Recruits HSP70 to β-Glucocerebrosidase and Is Therapeutic Against Gaucher Disease
Jian J, Tian Q, Hettinghouse A, Zhao S, Liu H, Wei J, Grunig G, Zhang W, Setchell K, Sun Y, Overkleeft H, Chan G, Liu C. Progranulin Recruits HSP70 to β-Glucocerebrosidase and Is Therapeutic Against Gaucher Disease. EBioMedicine 2016, 13: 212-224. PMID: 27789271, PMCID: PMC5264254, DOI: 10.1016/j.ebiom.2016.10.010.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsCell LineDisease Models, AnimalFibroblastsGaucher DiseaseGlucosylceramidaseHSP70 Heat-Shock ProteinsHumansIntercellular Signaling Peptides and ProteinsLysosome-Associated Membrane GlycoproteinsLysosomesMiceMice, KnockoutPhenotypeProgranulinsProtein AggregatesProtein BindingRecombinant ProteinsStress, PhysiologicalConceptsGaucher diseaseLysosomal storage diseaseStorage diseaseCommon lysosomal storage diseaseNew therapeutic interventionsΒ-glucocerebrosidaseProgranulin insufficiencyAnimal modelsTherapeutic interventionsDiseasePGRNDisease phenotypePatient fibroblastsGCaseComplex-associated proteinsLysosomal localizationHSP70Deficiency
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