{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T06:45:04Z","timestamp":1791182704262,"version":"4.1.0"},"reference-count":25,"publisher":"Portland Press Ltd.","issue":"1","content-domain":{"domain":["portlandpress.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2008,4,1]]},"abstract":"<jats:p>BMP [bis(monoacylglycero)phosphate] is an acidic phospholipid and a structural isomer of PG (phosphatidylglycerol), consisting of lysophosphatidylglycerol with an additional fatty acid esterified to the glycerol head group. It is thought to be synthesized from PG in the endosomal\/lysosomal compartment and is found primarily in multivesicular bodies within the same compartment. In the present study, we investigated the effect of lysosomal storage on BMP in cultured fibroblasts from patients with eight different LSDs (lysosomal storage disorders) and plasma samples from patients with one of 20 LSDs. Using ESI-MS\/MS (electrospray ionization tandem MS), we were able to demonstrate either elevations or alterations in the individual species of BMP, but not of PG, in cultured fibroblasts. All affected cell lines, with the exception of Fabry disease, showed a loss of polyunsaturated BMP species relative to mono-unsaturated species, and this correlated with the literature reports of lysosomal dysfunction leading to elevations of glycosphingolipids and cholesterol in affected cells, processes thought to be critical to the pathogenesis of LSDs. Plasma samples from patients with LSDs involving storage in macrophages and\/or with hepatomegaly showed an elevation in the plasma concentration of the C18:1\/C18:1 species of BMP when compared with control plasmas, whereas disorders involving primarily the central nervous system pathology did not. These results suggest that the release of BMP is cell\/tissue-specific and that it may be useful as a biomarker for a subset of LSDs.<\/jats:p>","DOI":"10.1042\/bj20071043","type":"journal-article","created":{"date-parts":[[2008,3,14]],"date-time":"2008-03-14T17:04:46Z","timestamp":1205514286000},"page":"71-78","update-policy":"https:\/\/doi.org\/10.1042\/crossmark_policy","source":"Crossref","is-referenced-by-count":92,"title":["Effect of lysosomal storage on bis(monoacylglycero)phosphate"],"prefix":"10.1042","volume":"411","author":[{"given":"Peter\u00a0J.","family":"Meikle","sequence":"first","affiliation":[{"name":"Lysosomal Diseases Research Unit, Department of Genetic Medicine, Children Youth and Women's Health Service, North Adelaide, SA 5006, Australia"},{"name":"Department of Paediatrics, University of Adelaide, Adelaide, SA 5005, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephen","family":"Duplock","sequence":"additional","affiliation":[{"name":"Lysosomal Diseases Research Unit, Department of Genetic Medicine, Children Youth and Women's Health Service, North Adelaide, SA 5006, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Blacklock","sequence":"additional","affiliation":[{"name":"Lysosomal Diseases Research Unit, Department of Genetic Medicine, Children Youth and Women's Health Service, North Adelaide, SA 5006, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Phillip\u00a0D.","family":"Whitfield","sequence":"additional","affiliation":[{"name":"Lysosomal Diseases Research Unit, Department of Genetic Medicine, Children Youth and Women's Health Service, North Adelaide, SA 5006, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gemma","family":"Macintosh","sequence":"additional","affiliation":[{"name":"Lysosomal Diseases Research Unit, Department of Genetic Medicine, Children Youth and Women's Health Service, North Adelaide, SA 5006, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John\u00a0J.","family":"Hopwood","sequence":"additional","affiliation":[{"name":"Lysosomal Diseases Research Unit, Department of Genetic Medicine, Children Youth and Women's Health Service, North Adelaide, SA 5006, Australia"},{"name":"Department of Paediatrics, University of Adelaide, Adelaide, SA 5005, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"Fuller","sequence":"additional","affiliation":[{"name":"Lysosomal Diseases Research Unit, Department of Genetic Medicine, Children Youth and Women's Health Service, North Adelaide, SA 5006, Australia"},{"name":"Department of Paediatrics, University of Adelaide, Adelaide, SA 5005, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"288","published-online":{"date-parts":[[2008,3,13]]},"reference":[{"key":"2021112215135552900_B1","doi-asserted-by":"crossref","first-page":"65","DOI":"10.1016\/S1095-6433(01)00306-3","article-title":"A comparison of the molecular species compositions of mammalian lung surfactant phospholipids","volume":"129","author":"Postle","year":"2001","journal-title":"Comp. Biochem. Physiol. A Mol. Integr. Physiol."},{"key":"2021112215135552900_B2","doi-asserted-by":"crossref","first-page":"13995","DOI":"10.1021\/bi961164o","article-title":"Transacylase and phospholipases in the synthesis of bis(monoacylglycero)phosphate","volume":"35","author":"Amidon","year":"1996","journal-title":"Biochemistry"},{"key":"2021112215135552900_B3","doi-asserted-by":"crossref","first-page":"277","DOI":"10.1016\/S1388-1981(99)00021-9","article-title":"Transacylase formation of bis(monoacylglycerol)phosphate","volume":"1437","author":"Heravi","year":"1999","journal-title":"Biochim. Biophys. Acta"},{"key":"2021112215135552900_B4","doi-asserted-by":"crossref","first-page":"32157","DOI":"10.1074\/jbc.M202838200","article-title":"Separation and characterization of late endosomal membrane domains","volume":"277","author":"Kobayashi","year":"2002","journal-title":"J. Biol. Chem."},{"key":"2021112215135552900_B5","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1146\/annurev.cellbio.21.122303.120013","article-title":"Principles of lysosomal membrane digestion: stimulation of sphingolipid degradation by sphingolipid activator proteins and anionic lysosomal lipids","volume":"21","author":"Kolter","year":"2005","journal-title":"Annu. Rev. Cell Dev. Biol."},{"key":"2021112215135552900_B6","doi-asserted-by":"crossref","first-page":"113","DOI":"10.1038\/10084","article-title":"Late endosomal membranes rich in lysobisphosphatidic acid regulate cholesterol transport","volume":"1","author":"Kobayashi","year":"1999","journal-title":"Nat. Cell Biol."},{"key":"2021112215135552900_B7","first-page":"79","article-title":"Order from disorder, corralling cholesterol with chaotic lipids. The role of polyunsaturated lipids in membrane raft formation","volume":"132","author":"Wassall","year":"2004","journal-title":"Chem. Phys. Lipids"},{"key":"2021112215135552900_B8","doi-asserted-by":"crossref","first-page":"433","DOI":"10.1016\/j.semcdb.2004.03.002","article-title":"Secondary accumulation of gangliosides in lysosomal storage disorders","volume":"15","author":"Walkley","year":"2004","journal-title":"Semin. Cell Dev. Biol."},{"key":"2021112215135552900_B9","doi-asserted-by":"crossref","first-page":"415","DOI":"10.1002\/cne.20355","article-title":"Differential subcellular localization of cholesterol, gangliosides, and glycosaminoglycans in murine models of mucopolysaccharide storage disorders","volume":"480","author":"McGlynn","year":"2004","journal-title":"J. Comp. Neurol."},{"key":"2021112215135552900_B10","doi-asserted-by":"crossref","first-page":"239","DOI":"10.1006\/mgme.2000.3139","article-title":"Metabolic studies of glycosphingolipid accumulation in mucopolysaccharidosis IIID","volume":"72","author":"Liour","year":"2001","journal-title":"Mol. Genet. Metab."},{"key":"2021112215135552900_B11","doi-asserted-by":"crossref","first-page":"257","DOI":"10.5694\/j.1326-5377.1982.tb132293.x","article-title":"Enzymatic diagnosis of the mucopolysaccharidoses: experience of 96 cases diagnosed in a five-year period","volume":"1","author":"Hopwood","year":"1982","journal-title":"Med. J. Aust."},{"key":"2021112215135552900_B12","doi-asserted-by":"crossref","first-page":"265","DOI":"10.1016\/S0021-9258(19)52451-6","article-title":"Protein measurement with the Folin phenol reagent","volume":"193","author":"Lowry","year":"1951","journal-title":"J. Biol. Chem."},{"key":"2021112215135552900_B13","doi-asserted-by":"crossref","first-page":"1345","DOI":"10.1172\/JCI103182","article-title":"The distribution and chemical composition of ultracentrifugally separated lipoproteins in human serum","volume":"34","author":"Havel","year":"1955","journal-title":"J. Clin. Invest."},{"key":"2021112215135552900_B14","doi-asserted-by":"crossref","first-page":"161","DOI":"10.1042\/bj20031594","article-title":"Mast cell- and dendritic cell-derived exosomes display a specific lipid composition and an unusual membrane organization","volume":"380","author":"Laulagnier","year":"2004","journal-title":"Biochem. J."},{"key":"2021112215135552900_B15","doi-asserted-by":"crossref","first-page":"497","DOI":"10.1016\/S0021-9258(18)64849-5","article-title":"A simple method for the isolation and purification of total lipids from animal tissues","volume":"226","author":"Folch","year":"1957","journal-title":"J. Biol. Chem."},{"key":"2021112215135552900_B16","doi-asserted-by":"crossref","first-page":"317","DOI":"10.1016\/j.ab.2004.04.004","article-title":"Toward fingerprinting cellular lipidomes directly from biological samples by two-dimensional electrospray ionization mass spectrometry","volume":"330","author":"Han","year":"2004","journal-title":"Anal. Biochem."},{"key":"2021112215135552900_B17","doi-asserted-by":"crossref","first-page":"330","DOI":"10.1016\/0005-2760(92)90063-2","article-title":"Bis(monoacylglycero)phosphate from PC12 cells, a phospholipid that can comigrate with phosphatidic acid: molecular species analysis by fast atom bombardment mass spectrometry","volume":"1125","author":"Holbrook","year":"1992","journal-title":"Biochim. Biophys. Acta"},{"key":"2021112215135552900_B18","doi-asserted-by":"crossref","first-page":"1051","DOI":"10.1046\/j.1471-4159.2003.01602.x","article-title":"Analysis of phospholipid molecular species in brains from patients with infantile and juvenile neuronal-ceroid lipofuscinosis using liquid chromatography\u2013electrospray ionization mass spectrometry","volume":"84","author":"Kakela","year":"2003","journal-title":"J. Neurochem."},{"key":"2021112215135552900_B19","doi-asserted-by":"crossref","first-page":"179","DOI":"10.1006\/abio.1999.4452","article-title":"Electrospray\/tandem mass spectrometry for quantitative analysis of lipid remodeling in essential fatty acid deficient mice","volume":"279","author":"Duffin","year":"2000","journal-title":"Anal. Biochem."},{"key":"2021112215135552900_B20","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1007\/BF01813904","article-title":"Enzyme activities and phospholipid storage patterns in brain and spleen samples from Niemann\u2013Pick disease variants: a comparison of neuropathic and non-neuropathic forms","volume":"9","author":"Besley","year":"1986","journal-title":"J. Inherit. Metab. Dis."},{"key":"2021112215135552900_B21","doi-asserted-by":"crossref","first-page":"536","DOI":"10.1111\/j.1365-2990.2005.00675.x","article-title":"Abnormal neuronal metabolism and storage in mucopolysaccharidosis type\u00a0VI (Maroteaux\u2013Lamy) disease","volume":"31","author":"Walkley","year":"2005","journal-title":"Neuropathol. Appl. Neurobiol."},{"key":"2021112215135552900_B22","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1016\/S0014-5793(00)01417-4","article-title":"Further studies on the reconstitution of glucosylceramidase activity by Sap C and anionic phospholipids","volume":"472","author":"Salvioli","year":"2000","journal-title":"FEBS Lett."},{"key":"2021112215135552900_B23","doi-asserted-by":"crossref","first-page":"11358","DOI":"10.1073\/pnas.96.20.11358","article-title":"Cholesterol efflux to apolipoprotein AI involves endocytosis and resecretion in a calcium-dependent pathway","volume":"96","author":"Takahashi","year":"1999","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"key":"2021112215135552900_B24","doi-asserted-by":"crossref","first-page":"1221","DOI":"10.1161\/01.RES.0000170946.56981.5c","article-title":"New insights into the regulation of HDL metabolism and reverse cholesterol transport","volume":"96","author":"Lewis","year":"2005","journal-title":"Circ. Res."},{"key":"2021112215135552900_B25","doi-asserted-by":"crossref","first-page":"1952","DOI":"10.1016\/S0022-2275(20)32356-7","article-title":"Involvement of caveolin-1 in cholesterol enrichment of high density lipoprotein during its assembly by apolipoprotein and THP-1 cells","volume":"41","author":"Arakawa","year":"2000","journal-title":"J. Lipid Res."}],"container-title":["Biochemical Journal"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/portlandpress.com\/biochemj\/article-pdf\/411\/1\/71\/651775\/bj4110071.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/portlandpress.com\/biochemj\/article-pdf\/411\/1\/71\/651775\/bj4110071.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,11,22]],"date-time":"2021-11-22T20:18:44Z","timestamp":1637612324000},"score":1,"resource":{"primary":{"URL":"https:\/\/portlandpress.com\/biochemj\/article\/411\/1\/71\/44650\/Effect-of-lysosomal-storage-on-bis-monoacylglycero"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2008,3,13]]},"references-count":25,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2008,4,1]]}},"URL":"https:\/\/doi.org\/10.1042\/bj20071043","relation":{},"ISSN":["0264-6021","1470-8728"],"issn-type":[{"value":"0264-6021","type":"print"},{"value":"1470-8728","type":"electronic"}],"subject":[],"published":{"date-parts":[[2008,3,13]]}}}