2023
ER chaperones use a protein folding and quality control glyco-code
Guay K, Ke H, Canniff N, George G, Eyles S, Mariappan M, Contessa J, Gershenson A, Gierasch L, Hebert D. ER chaperones use a protein folding and quality control glyco-code. Molecular Cell 2023, 83: 4524-4537.e5. PMID: 38052210, PMCID: PMC10790639, DOI: 10.1016/j.molcel.2023.11.006.Peer-Reviewed Original Research
2020
A cut above (and below): Protein cleavage in the regulation of polycystin trafficking and signaling
Padovano V, Mistry K, Merrick D, Gresko N, Caplan MJ. A cut above (and below): Protein cleavage in the regulation of polycystin trafficking and signaling. Cellular Signalling 2020, 72: 109634. PMID: 32283256, PMCID: PMC7269866, DOI: 10.1016/j.cellsig.2020.109634.Peer-Reviewed Original ResearchConceptsPolycystin-1Polycystin proteinsG proteinsPolycystin-1 proteinProtein maturationTerminal tailObligate stepBiological pathwaysProtein cleavagePhysiological functionsProteolytic siteProteinPathological consequencesAutosomal dominant polycystic kidney diseaseTraffickingDominant polycystic kidney diseasePolycystic kidney diseasePrimary functionCleavageRegulationMaturationGenesMitochondriaValuable insightsPathway
2019
ALG9 Mutation Carriers Develop Kidney and Liver Cysts
Besse W, Chang AR, Luo JZ, Triffo WJ, Moore BS, Gulati A, Hartzel DN, Mane S, Center R, Torres VE, Somlo S, Mirshahi T. ALG9 Mutation Carriers Develop Kidney and Liver Cysts. Journal Of The American Society Of Nephrology 2019, 30: 2091-2102. PMID: 31395617, PMCID: PMC6830805, DOI: 10.1681/asn.2019030298.Peer-Reviewed Original ResearchConceptsProteins polycystin-1Autosomal dominant polycystic kidney diseaseDisease genesRare loss-of-function variantsN-glycan precursorsNovel disease genesLoss-of-function variantsEndoplasmic reticulum lumenLoss-of-function mutationsMonogenic kidney diseaseWhole-exome sequencingGenotype-phenotype correlationProtein biogenesisProtein maturationReticulum lumenPolycystin-1Endoplasmic reticulumGene productsPopulation-based cohortCell-based assaysPhenotypic characterizationPolycystic phenotypeMutation carrier stateDefective glycosylationDominant polycystic kidney disease
2004
Carbohydrates act as sorting determinants in ER-associated degradation of tyrosinase
Svedine S, Wang T, Halaban R, Hebert DN. Carbohydrates act as sorting determinants in ER-associated degradation of tyrosinase. Journal Of Cell Science 2004, 117: 2937-2949. PMID: 15161941, DOI: 10.1242/jcs.01154.Peer-Reviewed Original ResearchMeSH KeywordsAdenosine TriphosphateAnimalsCalnexinCarbohydrate MetabolismCells, CulturedEndoplasmic ReticulumEndoplasmic Reticulum Chaperone BiPGlucoseHeat-Shock ProteinsMannoseMelanocytesMiceMolecular ChaperonesMonophenol MonooxygenaseMutationProteasome Endopeptidase ComplexProtein Disulfide-IsomerasesProtein TransportConceptsLectin chaperonesMutant tyrosinaseEndoplasmic reticulum (ER) quality control machineryQuality control machineryProtein disulfide isomeraseDegradation of tyrosinaseERAD substratesChaperone interactionsNon-native substratesER organizationProtein maturationER retentionER lumenDisulfide isomeraseAberrant proteinsProteasomal degradationGlucose trimmingProtein degradationProtein aggregatesTyrosinase degradationSubsequent degradationChaperonesIntact melanocytesMaturation processProteasome
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