2023
The C-terminal tail of polycystin-1 suppresses cystic disease in a mitochondrial enzyme-dependent fashion
Onuchic L, Padovano V, Schena G, Rajendran V, Dong K, Shi X, Pandya R, Rai V, Gresko N, Ahmed O, Lam T, Wang W, Shen H, Somlo S, Caplan M. The C-terminal tail of polycystin-1 suppresses cystic disease in a mitochondrial enzyme-dependent fashion. Nature Communications 2023, 14: 1790. PMID: 36997516, PMCID: PMC10063565, DOI: 10.1038/s41467-023-37449-1.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsDisease Models, AnimalHumansKidneyMiceMitochondrial ProteinsNADP Transhydrogenase, AB-SpecificPolycystic Kidney, Autosomal DominantTRPP Cation ChannelsConceptsPolycystin-1Nicotinamide nucleotide transhydrogenaseTerminal tailCystic phenotypeAutosomal dominant polycystic kidney diseaseCyst cell proliferationC-terminal domainAmino acid residuesLethal monogenic disorderC-terminal cleavageNucleotide transhydrogenaseAcid residuesMitochondrial functionTransgenic expressionPKD1 geneRedox stateShort fragmentsCell proliferationMonogenic disordersDominant polycystic kidney diseasePolycystic kidney diseaseGene therapy strategiesProteinPhenotypeFragments
2017
Comparative Analysis of Mitochondrial N-Termini from Mouse, Human, and Yeast*
Calvo SE, Julien O, Clauser KR, Shen H, Kamer KJ, Wells JA, Mootha VK. Comparative Analysis of Mitochondrial N-Termini from Mouse, Human, and Yeast*. Molecular & Cellular Proteomics 2017, 16: 512-523. PMID: 28122942, PMCID: PMC5383775, DOI: 10.1074/mcp.m116.063818.Peer-Reviewed Original ResearchConceptsN-terminusMitochondrial proteinsCleavage eventsMature mitochondrial proteinN-terminal presequenceNovel protein isoformsN-end ruleAmino acidsMultiple cleavage eventsMitochondrial processingNuclear genomeHuman U937 cellsMature proteinMitochondrial peptidasesYeast proteinsProtein isoformsSequence levelCanonical motifsIndividual proteinsPresequenceDistinct enzymesPolypeptide cleavageCleavage siteYeastProtein