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10.1194/jlr.M057851 http://scihub22266oqcxt.onion/10.1194/jlr.M057851 C4409277!4409277 !25795792     free     free     free Warning: file_get_contents(https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&id=25795792 &cmd=llinks): Failed to open stream: HTTP request failed! 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Bis(monoacylglycero)phosphate: a secondary storage lipid in the gangliosidoses |pdf=|usr=}}{{25795792 }} gab.com Text Bis(monoacylglycero)phosphate: a secondary storage lipid in the gangliosidoses JO: J Lipid Res http://www.kidney.de/pget.php?&tw=1&pmid=25795792 #FOAvip Bis(monoacylglycero)phosphate (BMP) is a negatively charged glycerophospholipid with an unusual sn-1;sn-1' structural configuration. BMP is primarily enriched in endosomal/lysosomal membranes. BMP is thought to play a role in glycosphingolipid degradation and cholesterol transport. Elevated BMP levels have been found in many lysosomal storage diseases (LSDs), suggesting an association with lysosomal storage material. The gangliosidoses are a group of neurodegenerative LSDs involving the accumulation of either GM1 or GM2 gangliosides resulting from inherited deficiencies in ?-galactosidase or ?-hexosaminidase, respectively. Little information is available on BMP levels in gangliosidosis brain tissue. Our results showed that the content of BMP in brain was significantly greater in humans and in animals (mice, cats, American black bears) with either GM1 or GM2 ganglioside storage diseases, than in brains of normal subjects. The storage of BMP and ganglioside GM2 in brain were reduced similarly following adeno-associated viral-mediated gene therapy in Sandhoff disease mice. We also found that C22:6, C18:0, and C18:1 were the predominant BMP fatty acid species in gangliosidosis brains. The results show that BMP accumulates as a secondary storage material in the brain of a broad range of mammals with gangliosidoses. Twit Text FOAVip Bis(monoacylglycero)phosphate: a secondary storage lipid in the gangliosidoses ????J Lipid Res http://www.kidney.de/pget.php?&tw=1&pmid=25795792 #FOAvip Twit Text # Free Paper on # # # # # LINK http://www.kidney.de/pget.php?&tw=1&pmid=25795792 #FOAvip English Wikipedia <ref>{{Cite pmid|25795792 }}</ref> Bis(monoacylglycero)phosphate: a secondary storage lipid in the gangliosidoses #MMPMID25795792 Akgoc Z ; Sena-Esteves M ; Martin DR ; Han X ; d'Azzo A ; Seyfried TN J Lipid Res 2015[May]; 56 (5 ): 1006-13 PMID25795792 show ga Bis(monoacylglycero)phosphate (BMP) is a negatively charged glycerophospholipid with an unusual sn-1;sn-1' structural configuration. BMP is primarily enriched in endosomal/lysosomal membranes. BMP is thought to play a role in glycosphingolipid degradation and cholesterol transport. Elevated BMP levels have been found in many lysosomal storage diseases (LSDs), suggesting an association with lysosomal storage material. The gangliosidoses are a group of neurodegenerative LSDs involving the accumulation of either GM1 or GM2 gangliosides resulting from inherited deficiencies in ?-galactosidase or ?-hexosaminidase, respectively. Little information is available on BMP levels in gangliosidosis brain tissue. Our results showed that the content of BMP in brain was significantly greater in humans and in animals (mice, cats, American black bears) with either GM1 or GM2 ganglioside storage diseases, than in brains of normal subjects. The storage of BMP and ganglioside GM2 in brain were reduced similarly following adeno-associated viral-mediated gene therapy in Sandhoff disease mice. We also found that C22:6, C18:0, and C18:1 were the predominant BMP fatty acid species in gangliosidosis brains. The results show that BMP accumulates as a secondary storage material in the brain of a broad range of mammals with gangliosidoses. |Animals [MESH] |Brain/metabolism [MESH] |Cat Diseases/*metabolism [MESH] |Cats [MESH] |Female [MESH] |Gangliosidosis, GM1/metabolism/*veterinary [MESH] |Humans [MESH] |Lipid Metabolism [MESH] |Lysophospholipids/*metabolism [MESH] |Male [MESH] |Mice, 129 Strain [MESH] |Mice, Knockout [MESH] |Monoglycerides/*metabolism [MESH] |Sandhoff Disease/metabolism/*veterinary [MESH]Bis(monoacylglycero)phosphate: a secondary storage lipid in the gangli(epmc).pdf DeepDyve Pubget Overpricing