2022
Dynamic quality control machinery that operates across compartmental borders mediates the degradation of mammalian nuclear membrane proteins
Tsai P, Cameron C, Forni M, Wasko R, Naughton B, Horsley V, Gerstein M, Schlieker C. Dynamic quality control machinery that operates across compartmental borders mediates the degradation of mammalian nuclear membrane proteins. Cell Reports 2022, 41: 111675. PMID: 36417855, PMCID: PMC9827541, DOI: 10.1016/j.celrep.2022.111675.Peer-Reviewed Original ResearchConceptsProtein turnoverCellular quality control systemNuclear membrane proteinsQuality control machineryDistinct cellular compartmentsNuclear envelope proteinsGenetic screenProtein homeostasisUbiquitin ligasesControl machineryMembrane proteinsCellular compartmentsEnzyme Ube2g2Quality control systemEndoplasmic reticulumHuman diseasesEfficient biosynthesisHRD1RNF5Disease variantsTMEM33Envelope proteinSubstrate levelsDisease etiologyModel substrate
2019
An unbiased approach de-livers unexpected insight into torsin biology
Prophet SM, Schlieker C. An unbiased approach de-livers unexpected insight into torsin biology. Journal Of Clinical Investigation 2019, 129: 4576-4579. PMID: 31589164, PMCID: PMC6819095, DOI: 10.1172/jci132442.Peer-Reviewed Original ResearchConceptsFatty liver diseaseHepatic lipid metabolismLiver diseaseNonneural tissuesLipid metabolismBrain functionConditional deletionCongenital disorderDisease etiologySecretion defectHuman pathologiesMammalian liverPolypeptide 1Future investigationsSteatohepatitisUnbiased approachDysfunctionVLDLEtiologyMutationsDiseaseMiceLiverNeuronsPathology