{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"submitter":["Lea WA"],"funding":["NIDDK NIH HHS"],"pagination":["1500"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC6992733"],"repository":["biostudies-literature"],"omics_type":["Unknown"],"volume":["10(1)"],"pubmed_abstract":["The polycystin-1 (PC1), polycystin-2 (PC2) and fibrocystin proteins, the respective products of the PKD1, PKD2 and PKHD1 genes, are abundant in urinary exosome-like vesicles (ELVs) where they form the polycystin complex (PCC). ELVs are 100 nm diameter membrane vesicles shed into the urine by the cells lining the nephron. Using MS/MS analysis of ELVs from individuals with PKD1 mutations and controls, we show that in addition to the well-described GPS/GAIN cleavage event in PC1 at 3048 aa and the proprotein convertase cleavage (PPC) event in fibrocystin at 3616 aa, there are multiple other cleavage events in these proteins. The C-terminal 11 transmembrane portion of PC1 undergoes three cleavage events in vivo. The absence of peptides from the C-terminal cytoplasmic tail of fibrocystin implie"],"journal":["Scientific reports"],"pubmed_title":["Analysis of the polycystin complex (PCC) in human urinary exosome-like vesicles (ELVs)."],"pmcid":["PMC6992733"],"funding_grant_id":["P30 DK090868","P30 DK106912","RC1 DK086161","R01 DK080688"],"pubmed_authors":["Johnson KL","McCormick DJ","Zelenchuk L","McGreal K","Ward CJ","Madden BJ","Parnell SC","Charlesworth MC","Hogan MC","Lea WA","Sharma M"],"additional_accession":[]},"is_claimable":false,"name":"Analysis of the polycystin complex (PCC) in human urinary exosome-like vesicles (ELVs).","description":"The polycystin-1 (PC1), polycystin-2 (PC2) and fibrocystin proteins, the respective products of the PKD1, PKD2 and PKHD1 genes, are abundant in urinary exosome-like vesicles (ELVs) where they form the polycystin complex (PCC). ELVs are 100 nm diameter membrane vesicles shed into the urine by the cells lining the nephron. Using MS/MS analysis of ELVs from individuals with PKD1 mutations and controls, we show that in addition to the well-described GPS/GAIN cleavage event in PC1 at 3048 aa and the proprotein convertase cleavage (PPC) event in fibrocystin at 3616 aa, there are multiple other cleavage events in these proteins. The C-terminal 11 transmembrane portion of PC1 undergoes three cleavage events in vivo. The absence of peptides from the C-terminal cytoplasmic tail of fibrocystin implie","dates":{"release":"2020-01-01T00:00:00Z","publication":"2020 Jan","modification":"2026-05-07T21:53:31.498Z","creation":"2020-05-22T09:29:22Z"},"accession":"S-EPMC6992733","cross_references":{"pubmed":["32001768"],"doi":["10.1038/s41598-020-58087-3"]}}