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10.1007/s10545-017-0076-9 http://scihub22266oqcxt.onion/10.1007/s10545-017-0076-9 C5959964!5959964!28849344     free     free     free Warning: file_get_contents(https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&id=28849344&cmd=llinks): Failed to open stream: HTTP request failed! 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Functional characterisation of peroxisomal ?-oxidation disorders in fibroblasts using lipidomics |pdf=|usr=}}{{28849344}} gab.com Text Functional characterisation of peroxisomal -oxidation disorders in fibroblasts using lipidomics JO: J Inherit Metab Dis http://www.kidney.de/pget.php?&tw=1&pmid=28849344 #FOAvip Peroxisomes play an important role in a variety of metabolic pathways, including the ?- and ?-oxidation of fatty acids, and the biosynthesis of ether phospholipids. Single peroxisomal enzyme deficiencies (PEDs) are a group of peroxisomal disorders in which either a peroxisomal matrix enzyme or a peroxisomal membrane transporter protein is deficient. To investigate the functional consequences of specific enzyme deficiencies on the lipidome, we performed lipidomics using cultured skin fibroblasts with different defects in the ?-oxidation of very long-chain fatty acids, including ABCD1- (ALD), acyl-CoA oxidase 1 (ACOX1)-, D-bifunctional protein (DBP)-, and acyl-CoA binding domain containing protein 5 (ACBD5)-deficient cell lines. Ultra-high performance liquid chromatography coupled with high-resolution mass spectrometry revealed characteristic changes in the phospholipid composition in fibroblasts with different fatty acid ?-oxidation defects. Remarkably, we found that ether phospholipids, including plasmalogens, were decreased. We defined specific phospholipid ratios reflecting the different enzyme defects, which can be used to discriminate the PED fibroblasts from healthy control cells.Electronic supplementary material: The online version of this article (doi:10.1007/s10545-017-0076-9) contains supplementary material, which is available to authorized users. Twit Text FOAVip Functional characterisation of peroxisomal -oxidation disorders in fibroblasts using lipidomics ????J Inherit Metab Dis http://www.kidney.de/pget.php?&tw=1&pmid=28849344 #FOAvip Twit Text # Free Paper on # # # # # LINK http://www.kidney.de/pget.php?&tw=1&pmid=28849344 #FOAvip English Wikipedia <ref>{{Cite pmid|28849344}}</ref> Functional characterisation of peroxisomal ?-oxidation disorders in fibroblasts using lipidomics #MMPMID28849344 Herzog K; Pras-Raves ML; Ferdinandusse S; Vervaart MAT; Luyf ACM; van Kampen AHC; Wanders RJA; Waterham HR; Vaz FM J Inherit Metab Dis 2018[]; 41 (3): 479-87 PMID28849344show ga Peroxisomes play an important role in a variety of metabolic pathways, including the ?- and ?-oxidation of fatty acids, and the biosynthesis of ether phospholipids. Single peroxisomal enzyme deficiencies (PEDs) are a group of peroxisomal disorders in which either a peroxisomal matrix enzyme or a peroxisomal membrane transporter protein is deficient. To investigate the functional consequences of specific enzyme deficiencies on the lipidome, we performed lipidomics using cultured skin fibroblasts with different defects in the ?-oxidation of very long-chain fatty acids, including ABCD1- (ALD), acyl-CoA oxidase 1 (ACOX1)-, D-bifunctional protein (DBP)-, and acyl-CoA binding domain containing protein 5 (ACBD5)-deficient cell lines. Ultra-high performance liquid chromatography coupled with high-resolution mass spectrometry revealed characteristic changes in the phospholipid composition in fibroblasts with different fatty acid ?-oxidation defects. Remarkably, we found that ether phospholipids, including plasmalogens, were decreased. We defined specific phospholipid ratios reflecting the different enzyme defects, which can be used to discriminate the PED fibroblasts from healthy control cells.Electronic supplementary material: The online version of this article (doi:10.1007/s10545-017-0076-9) contains supplementary material, which is available to authorized users. äFunctional characterisation of peroxisomal ?-oxidation disorders in fi(epmc).pdf DeepDyve Pubget Overpricing