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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 Deprecated: Implicit conversion from float 231.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 231.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 231.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 231.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 231.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 231.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 231.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Warning: imagejpeg(C:\Inetpub\vhosts\kidney.de\httpdocs\phplern\28849344 .jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117       J+Inherit+Metab+Dis 2018 ; 41 (3 ): 479-487 Nephropedia Template TP {{tp|p=28849344 |t=2018. 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. 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[May]; 41 (3 ): 479-487 PMID28849344 show 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. |Case-Control Studies [MESH] |Cells, Cultured [MESH] |Chromatography, High Pressure Liquid/methods [MESH] |Fatty Acids/metabolism [MESH] |Fibroblasts/*chemistry/*metabolism [MESH] |Humans [MESH] |Lipid Metabolism [MESH] |Lipids/*analysis [MESH] |Mass Spectrometry/methods [MESH] |Metabolomics/*methods [MESH] |Oxidation-Reduction [MESH] |Peroxisomal Disorders/*diagnosis/metabolism [MESH] |Peroxisomes/metabolism [MESH]Functional characterisation of peroxisomal ?-oxidation disorders in fi(epmc).pdf DeepDyve Pubget Overpricing