FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W3185955495> ?p ?o ?g. }

• W3185955495 endingPage "1591" @default.
• W3185955495 startingPage "1576" @default.
• W3185955495 abstract "Key Points Although fatty acid (FA) oxidation defects have been reported in polycystic kidney disease (PKD), no studies have examined whether peroxisomes contribute to the abnormalities. We investigated peroxisome biogenesis and FA metabolism in autosomal dominant PKD models and tested whether polycystin-1 colocalized with peroxisome proteins. Our studies show that loss of Pkd1 does not disrupt peroxisome biogenesis nor peroxisome-dependent FA metabolism. Background Multiple studies of tissue and cell samples from patients and preclinical models of autosomal dominant polycystic kidney disease report abnormal mitochondrial function and morphology and suggest metabolic reprogramming is an intrinsic feature of this disease. Peroxisomes interact with mitochondria physically and functionally, and congenital peroxisome biogenesis disorders can cause various phenotypes, including mitochondrial defects, metabolic abnormalities, and renal cysts. We hypothesized that a peroxisomal defect might contribute to the metabolic and mitochondrial impairments observed in autosomal dominant polycystic kidney disease. Methods Using control and Pkd1−/− kidney epithelial cells, we investigated peroxisome abundance, biogenesis, and morphology by immunoblotting, immunofluorescence, and live cell imaging of peroxisome-related proteins and assayed peroxisomal specific β -oxidation. We further analyzed fatty acid composition by mass spectrometry in kidneys of Pkd1fl/fl;Ksp-Cre mice. We also evaluated peroxisome lipid metabolism in published metabolomics datasets of Pkd1 mutant cells and kidneys. Lastly, we investigated if the C terminus or full-length polycystin-1 colocalize with peroxisome markers by imaging studies. Results Peroxisome abundance, morphology, and peroxisome-related protein expression in Pkd1−/− cells were normal, suggesting preserved peroxisome biogenesis. Peroxisomal β -oxidation was not impaired in Pkd1−/− cells, and there was no obvious accumulation of very-long-chain fatty acids in kidneys of mutant mice. Reanalysis of published datasets provide little evidence of peroxisomal abnormalities in independent sets of Pkd1 mutant cells and cystic kidneys, and provide further evidence of mitochondrial fatty acid oxidation defects. Imaging studies with either full-length polycystin-1 or its C terminus, a fragment previously shown to go to the mitochondria, showed minimal colocalization with peroxisome markers restricted to putative mitochondrion-peroxisome contact sites. Conclusions Our studies showed that loss of Pkd1 does not disrupt peroxisome biogenesis nor peroxisome-dependent fatty acid metabolism." @default.
• W3185955495 created "2021-08-02" @default.
• W3185955495 creator A5013106852 @default.
• W3185955495 creator A5016691060 @default.
• W3185955495 creator A5019589330 @default.
• W3185955495 creator A5033822711 @default.
• W3185955495 creator A5068056560 @default.
• W3185955495 creator A5071899808 @default.
• W3185955495 creator A5084317555 @default.
• W3185955495 date "2021-10-01" @default.
• W3185955495 modified "2023-10-18" @default.
• W3185955495 title "Pkd1 Mutation Has No Apparent Effects on Peroxisome Structure or Lipid Metabolism" @default.
• W3185955495 cites W1522739755 @default.
• W3185955495 cites W157502360 @default.
• W3185955495 cites W1582860366 @default.
• W3185955495 cites W1645771708 @default.
• W3185955495 cites W1838356724 @default.
• W3185955495 cites W184565012 @default.
• W3185955495 cites W1966289658 @default.
• W3185955495 cites W1979157121 @default.
• W3185955495 cites W1996116737 @default.
• W3185955495 cites W2000489985 @default.
• W3185955495 cites W2009917506 @default.
• W3185955495 cites W2017759854 @default.
• W3185955495 cites W2064298973 @default.
• W3185955495 cites W2079801694 @default.
• W3185955495 cites W2087804239 @default.
• W3185955495 cites W2089042234 @default.
• W3185955495 cites W2099540110 @default.
• W3185955495 cites W2107211482 @default.
• W3185955495 cites W2111220794 @default.
• W3185955495 cites W2111222260 @default.
• W3185955495 cites W2116926723 @default.
• W3185955495 cites W2117395827 @default.
• W3185955495 cites W2133073099 @default.
• W3185955495 cites W2135440453 @default.
• W3185955495 cites W2151833520 @default.
• W3185955495 cites W2160867187 @default.
• W3185955495 cites W2169651469 @default.
• W3185955495 cites W2184489830 @default.
• W3185955495 cites W2273525420 @default.
• W3185955495 cites W2290606973 @default.
• W3185955495 cites W2552102609 @default.
• W3185955495 cites W2585343016 @default.
• W3185955495 cites W2588834240 @default.
• W3185955495 cites W2605752950 @default.
• W3185955495 cites W2617326069 @default.
• W3185955495 cites W2746883268 @default.
• W3185955495 cites W2762281899 @default.
• W3185955495 cites W2793765428 @default.
• W3185955495 cites W2800857545 @default.
• W3185955495 cites W2900484912 @default.
• W3185955495 cites W2944176841 @default.
• W3185955495 cites W2951565157 @default.
• W3185955495 cites W2972128634 @default.
• W3185955495 cites W2981186198 @default.
• W3185955495 cites W3014284520 @default.
• W3185955495 cites W3024891235 @default.
• W3185955495 doi "https://doi.org/10.34067/kid.0000962021" @default.
• W3185955495 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/35372986" @default.
• W3185955495 hasPublicationYear "2021" @default.
• W3185955495 type Work @default.
• W3185955495 sameAs 3185955495 @default.
• W3185955495 citedByCount "2" @default.
• W3185955495 countsByYear W31859554952022 @default.
• W3185955495 countsByYear W31859554952023 @default.
• W3185955495 crossrefType "journal-article" @default.
• W3185955495 hasAuthorship W3185955495A5013106852 @default.
• W3185955495 hasAuthorship W3185955495A5016691060 @default.
• W3185955495 hasAuthorship W3185955495A5019589330 @default.
• W3185955495 hasAuthorship W3185955495A5033822711 @default.
• W3185955495 hasAuthorship W3185955495A5068056560 @default.
• W3185955495 hasAuthorship W3185955495A5071899808 @default.
• W3185955495 hasAuthorship W3185955495A5084317555 @default.
• W3185955495 hasBestOaLocation W31859554951 @default.
• W3185955495 hasConcept C100773827 @default.
• W3185955495 hasConcept C104317684 @default.
• W3185955495 hasConcept C127078168 @default.
• W3185955495 hasConcept C130241916 @default.
• W3185955495 hasConcept C131934819 @default.
• W3185955495 hasConcept C134018914 @default.
• W3185955495 hasConcept C170493617 @default.
• W3185955495 hasConcept C25095133 @default.
• W3185955495 hasConcept C2776266639 @default.
• W3185955495 hasConcept C2777229759 @default.
• W3185955495 hasConcept C2777485865 @default.
• W3185955495 hasConcept C2780091579 @default.
• W3185955495 hasConcept C28859421 @default.
• W3185955495 hasConcept C4733338 @default.
• W3185955495 hasConcept C55493867 @default.
• W3185955495 hasConcept C86803240 @default.
• W3185955495 hasConcept C95444343 @default.
• W3185955495 hasConceptScore W3185955495C100773827 @default.
• W3185955495 hasConceptScore W3185955495C104317684 @default.
• W3185955495 hasConceptScore W3185955495C127078168 @default.
• W3185955495 hasConceptScore W3185955495C130241916 @default.
• W3185955495 hasConceptScore W3185955495C131934819 @default.
• W3185955495 hasConceptScore W3185955495C134018914 @default.

• next