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- W4361255683 abstract "Autosomal dominant polycystic kidney disease (ADPKD) is the most prevalent potentially lethal monogenic disorder. Mutations in the PKD1 gene, which encodes polycystin-1 (PC1), account for approximately 78% of cases. PC1 is a large 462-kDa protein that undergoes cleavage in its N and C-terminal domains. C-terminal cleavage produces fragments that translocate to mitochondria. We show that transgenic expression of a protein corresponding to the final 200 amino acid (aa) residues of PC1 in two Pkd1-KO orthologous murine models of ADPKD suppresses cystic phenotype and preserves renal function. This suppression depends upon an interaction between the C-terminal tail of PC1 and the mitochondrial enzyme Nicotinamide Nucleotide Transhydrogenase (NNT). This interaction modulates tubular/cyst cell proliferation, the metabolic profile, mitochondrial function, and the redox state. Together, these results suggest that a short fragment of PC1 is sufficient to suppress cystic phenotype and open the door to the exploration of gene therapy strategies for ADPKD." @default.
- W4361255683 created "2023-03-31" @default.
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- W4361255683 date "2023-03-30" @default.
- W4361255683 modified "2023-10-18" @default.
- W4361255683 title "The C-terminal tail of polycystin-1 suppresses cystic disease in a mitochondrial enzyme-dependent fashion" @default.
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- W4361255683 doi "https://doi.org/10.1038/s41467-023-37449-1" @default.
- W4361255683 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/36997516" @default.
- W4361255683 hasPublicationYear "2023" @default.
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