FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W2290606973> ?p ?o ?g. }

• W2290606973 endingPage "1590" @default.
• W2290606973 startingPage "1579" @default.
• W2290606973 abstract "Zellweger syndrome and related disorders represent a group of lethal, genetically heterogeneous diseases. These peroxisome biogenesis disorders (PBDs) are characterized by defective peroxisomal matrix protein import and comprise at least 10 complementation groups. The genes defective in seven of these groups and more than 90% of PBD patients are now known. Here we examine the distribution of peroxisomal membrane proteins in fibroblasts from PBD patients representing the seven complementation groups for which the mutant gene is known. Peroxisomes were detected in all PBD cells, indicating that the ability to form a minimal peroxisomal structure is not blocked in these mutants. We also observed that peroxisome abundance was reduced fivefold in PBD cells that are defective in the PEX1, PEX5, PEX12, PEX6, PEX10, and PEX2 genes. These cell lines all display a defect in the import of proteins with the type-1 peroxisomal targeting signal (PTS1). In contrast, peroxisome abundance was unaffected in cells that are mutated in PEX7 and are defective only in the import of proteins with the type-2 peroxisomal targeting signal. Interestingly, a fivefold reduction in peroxisome abundance was also observed for cells lacking either of two PTS1-targeted peroxisomal beta-oxidation enzymes, acyl-CoA oxidase and 2-enoyl-CoA hydratase/D-3-hydroxyacyl-CoA dehydrogenase. These results indicate that reduced peroxisome abundance in PBD cells may be caused by their inability to import these PTS1-containing enzymes. Furthermore, the fact that peroxisome abundance is influenced by peroxisomal 105-oxidation activities suggests that there may be metabolic control of peroxisome abundance." @default.
• W2290606973 created "2016-06-24" @default.
• W2290606973 creator A5003907612 @default.
• W2290606973 creator A5016156307 @default.
• W2290606973 creator A5025089980 @default.
• W2290606973 creator A5042584066 @default.
• W2290606973 creator A5059798104 @default.
• W2290606973 creator A5066547264 @default.
• W2290606973 creator A5091858941 @default.
• W2290606973 date "1999-05-15" @default.
• W2290606973 modified "2023-10-14" @default.
• W2290606973 title "Metabolic control of peroxisome abundance" @default.
• W2290606973 cites W119049379 @default.
• W2290606973 cites W1606277396 @default.
• W2290606973 cites W1645771708 @default.
• W2290606973 cites W1826524642 @default.
• W2290606973 cites W1877409741 @default.
• W2290606973 cites W1905481237 @default.
• W2290606973 cites W1973400291 @default.
• W2290606973 cites W1987049546 @default.
• W2290606973 cites W1994238431 @default.
• W2290606973 cites W1998485639 @default.
• W2290606973 cites W2000489985 @default.
• W2290606973 cites W2006120283 @default.
• W2290606973 cites W2009917506 @default.
• W2290606973 cites W2016626029 @default.
• W2290606973 cites W2018171395 @default.
• W2290606973 cites W2019872550 @default.
• W2290606973 cites W2021461751 @default.
• W2290606973 cites W2025104020 @default.
• W2290606973 cites W2028043438 @default.
• W2290606973 cites W2033150452 @default.
• W2290606973 cites W2037167329 @default.
• W2290606973 cites W2057579672 @default.
• W2290606973 cites W2060401928 @default.
• W2290606973 cites W2065260095 @default.
• W2290606973 cites W2067747241 @default.
• W2290606973 cites W2071649496 @default.
• W2290606973 cites W2073972553 @default.
• W2290606973 cites W2078142538 @default.
• W2290606973 cites W2082573660 @default.
• W2290606973 cites W2083543206 @default.
• W2290606973 cites W2084938165 @default.
• W2290606973 cites W2087303310 @default.
• W2290606973 cites W2092362067 @default.
• W2290606973 cites W2094238059 @default.
• W2290606973 cites W2112107786 @default.
• W2290606973 cites W2118530103 @default.
• W2290606973 cites W2119598920 @default.
• W2290606973 cites W2119635227 @default.
• W2290606973 cites W2123214397 @default.
• W2290606973 cites W2130944611 @default.
• W2290606973 cites W2131521418 @default.
• W2290606973 cites W2151810319 @default.
• W2290606973 cites W2154782659 @default.
• W2290606973 cites W2155628638 @default.
• W2290606973 cites W2156432834 @default.
• W2290606973 cites W2157110784 @default.
• W2290606973 cites W2158795732 @default.
• W2290606973 cites W2160700542 @default.
• W2290606973 cites W2162421551 @default.
• W2290606973 cites W2167246280 @default.
• W2290606973 cites W2186252650 @default.
• W2290606973 cites W223368339 @default.
• W2290606973 doi "https://doi.org/10.1242/jcs.112.10.1579" @default.
• W2290606973 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/10212151" @default.
• W2290606973 hasPublicationYear "1999" @default.
• W2290606973 type Work @default.
• W2290606973 sameAs 2290606973 @default.
• W2290606973 citedByCount "129" @default.
• W2290606973 countsByYear W22906069732012 @default.
• W2290606973 countsByYear W22906069732013 @default.
• W2290606973 countsByYear W22906069732014 @default.
• W2290606973 countsByYear W22906069732015 @default.
• W2290606973 countsByYear W22906069732016 @default.
• W2290606973 countsByYear W22906069732017 @default.
• W2290606973 countsByYear W22906069732018 @default.
• W2290606973 countsByYear W22906069732020 @default.
• W2290606973 countsByYear W22906069732021 @default.
• W2290606973 countsByYear W22906069732022 @default.
• W2290606973 countsByYear W22906069732023 @default.
• W2290606973 crossrefType "journal-article" @default.
• W2290606973 hasAuthorship W2290606973A5003907612 @default.
• W2290606973 hasAuthorship W2290606973A5016156307 @default.
• W2290606973 hasAuthorship W2290606973A5025089980 @default.
• W2290606973 hasAuthorship W2290606973A5042584066 @default.
• W2290606973 hasAuthorship W2290606973A5059798104 @default.
• W2290606973 hasAuthorship W2290606973A5066547264 @default.
• W2290606973 hasAuthorship W2290606973A5091858941 @default.
• W2290606973 hasConcept C104317684 @default.
• W2290606973 hasConcept C12296475 @default.
• W2290606973 hasConcept C127078168 @default.
• W2290606973 hasConcept C143065580 @default.
• W2290606973 hasConcept C188082640 @default.
• W2290606973 hasConcept C205668238 @default.
• W2290606973 hasConcept C2777485865 @default.
• W2290606973 hasConcept C2781317408 @default.
• W2290606973 hasConcept C55493867 @default.

• next