FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W2076878049> ?p ?o ?g. }

• W2076878049 endingPage "619" @default.
• W2076878049 startingPage "610" @default.
• W2076878049 abstract "Peroxisomes are subcellular organelles that function in multiple anabolic and catabolic processes, including β-oxidation of very-long-chain fatty acids (VLCFA) and biosynthesis of ether phospholipids. Peroxisomal disorders caused by defects in peroxisome biogenesis or peroxisomal β-oxidation manifest as severe neural disorders of the central nervous system. Abnormal peroxisomal metabolism is thought to be responsible for the clinical symptoms of these diseases, but their molecular pathogenesis remains to be elucidated. We performed lipidomic analysis to identify aberrant metabolites in fibroblasts from patients with Zellweger syndrome (ZS), acyl-CoA oxidase1 (AOx) deficiency, D-bifunctional protein (D-BP) and X-linked adrenoleukodystrophy (X-ALD), as well as in peroxisome-deficient Chinese hamster ovary cell mutants. In cells deficient in peroxisomal biogenesis, plasmenylethanolamine was remarkably reduced and phosphatidylethanolamine was increased. Marked accumulation of very-long-chain saturated fatty acid and monounsaturated fatty acids in phosphatidylcholine was observed in all mutant cells. Very-long-chain polyunsaturated fatty acid (VLC-PUFA) levels were significantly elevated, whilst phospholipids containing docosahexaenoic acid (DHA, C22:6n-3) were reduced in fibroblasts from patients with ZS, AOx deficiency, and D-BP deficiency, but not in fibroblasts from an X-ALD patient. Because patients with AOx deficiency suffer from more severe symptoms than those with X-ALD, accumulation of VLC-PUFA and/or reduction of DHA may be associated with the severity of peroxisomal diseases." @default.
• W2076878049 created "2016-06-24" @default.
• W2076878049 creator A5029383741 @default.
• W2076878049 creator A5029449740 @default.
• W2076878049 creator A5053489078 @default.
• W2076878049 creator A5054959537 @default.
• W2076878049 creator A5079327408 @default.
• W2076878049 date "2014-04-01" @default.
• W2076878049 modified "2023-10-16" @default.
• W2076878049 title "Very-long-chain polyunsaturated fatty acids accumulate in phosphatidylcholine of fibroblasts from patients with Zellweger syndrome and acyl-CoA oxidase1 deficiency" @default.
• W2076878049 cites W1538386443 @default.
• W2076878049 cites W1602972089 @default.
• W2076878049 cites W1683037353 @default.
• W2076878049 cites W1966285776 @default.
• W2076878049 cites W1967818306 @default.
• W2076878049 cites W1969920697 @default.
• W2076878049 cites W1971817019 @default.
• W2076878049 cites W1980380307 @default.
• W2076878049 cites W1987601583 @default.
• W2076878049 cites W1988211561 @default.
• W2076878049 cites W1991080827 @default.
• W2076878049 cites W1994613052 @default.
• W2076878049 cites W1996044489 @default.
• W2076878049 cites W1999442060 @default.
• W2076878049 cites W1999919904 @default.
• W2076878049 cites W2001954239 @default.
• W2076878049 cites W2005966972 @default.
• W2076878049 cites W2006841196 @default.
• W2076878049 cites W2009192911 @default.
• W2076878049 cites W2014581910 @default.
• W2076878049 cites W2014664955 @default.
• W2076878049 cites W2015577087 @default.
• W2076878049 cites W2020253195 @default.
• W2076878049 cites W2027110836 @default.
• W2076878049 cites W2032527505 @default.
• W2076878049 cites W2036275900 @default.
• W2076878049 cites W2044558839 @default.
• W2076878049 cites W2047277779 @default.
• W2076878049 cites W2057729851 @default.
• W2076878049 cites W2064245163 @default.
• W2076878049 cites W2066289643 @default.
• W2076878049 cites W2067275984 @default.
• W2076878049 cites W2072066566 @default.
• W2076878049 cites W2079213310 @default.
• W2076878049 cites W2079801694 @default.
• W2076878049 cites W2081869877 @default.
• W2076878049 cites W2087303310 @default.
• W2076878049 cites W2095777272 @default.
• W2076878049 cites W2100174362 @default.
• W2076878049 cites W2101378834 @default.
• W2076878049 cites W2101641567 @default.
• W2076878049 cites W2111220794 @default.
• W2076878049 cites W2112798765 @default.
• W2076878049 cites W2119068792 @default.
• W2076878049 cites W2125775483 @default.
• W2076878049 cites W2126108038 @default.
• W2076878049 cites W2135684554 @default.
• W2076878049 cites W2138312139 @default.
• W2076878049 cites W2143790242 @default.
• W2076878049 cites W2143942115 @default.
• W2076878049 cites W2147536089 @default.
• W2076878049 cites W2148246150 @default.
• W2076878049 cites W2159466612 @default.
• W2076878049 cites W2167326901 @default.
• W2076878049 cites W2187003249 @default.
• W2076878049 doi "https://doi.org/10.1016/j.bbalip.2014.01.001" @default.
• W2076878049 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/24418004" @default.
• W2076878049 hasPublicationYear "2014" @default.
• W2076878049 type Work @default.
• W2076878049 sameAs 2076878049 @default.
• W2076878049 citedByCount "47" @default.
• W2076878049 countsByYear W20768780492015 @default.
• W2076878049 countsByYear W20768780492016 @default.
• W2076878049 countsByYear W20768780492017 @default.
• W2076878049 countsByYear W20768780492018 @default.
• W2076878049 countsByYear W20768780492019 @default.
• W2076878049 countsByYear W20768780492020 @default.
• W2076878049 countsByYear W20768780492021 @default.
• W2076878049 countsByYear W20768780492022 @default.
• W2076878049 countsByYear W20768780492023 @default.
• W2076878049 crossrefType "journal-article" @default.
• W2076878049 hasAuthorship W2076878049A5029383741 @default.
• W2076878049 hasAuthorship W2076878049A5029449740 @default.
• W2076878049 hasAuthorship W2076878049A5053489078 @default.
• W2076878049 hasAuthorship W2076878049A5054959537 @default.
• W2076878049 hasAuthorship W2076878049A5079327408 @default.
• W2076878049 hasConcept C104317684 @default.
• W2076878049 hasConcept C126322002 @default.
• W2076878049 hasConcept C127078168 @default.
• W2076878049 hasConcept C134018914 @default.
• W2076878049 hasConcept C19038510 @default.
• W2076878049 hasConcept C2777150068 @default.
• W2076878049 hasConcept C2777171753 @default.
• W2076878049 hasConcept C2777485865 @default.
• W2076878049 hasConcept C2777565452 @default.
• W2076878049 hasConcept C2778918659 @default.
• W2076878049 hasConcept C2779611823 @default.
• W2076878049 hasConcept C2781317408 @default.

• next