HOMEPRODUCTSCOMPANYCONTACTFAQResearchDictionaryPharmaSign Up FREE or Login

Bis(monoacylglycero)phosphate: a secondary storage lipid in the gangliosidoses.

Abstract
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 β-galactosidase or β-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.
AuthorsZeynep Akgoc, Miguel Sena-Esteves, Douglas R Martin, Xianlin Han, Alessandra d'Azzo, Thomas N Seyfried
JournalJournal of lipid research (J Lipid Res) Vol. 56 Issue 5 Pg. 1006-13 (May 2015) ISSN: 1539-7262 [Electronic] United States
PMID25795792 (Publication Type: Journal Article, Research Support, N.I.H., Extramural, Research Support, Non-U.S. Gov't)
CopyrightCopyright © 2015 by the American Society for Biochemistry and Molecular Biology, Inc.
Chemical References
  • Lysophospholipids
  • Monoglycerides
  • bis(monoacylglyceryl)phosphate
Topics
  • Animals
  • Brain (metabolism)
  • Cat Diseases (metabolism)
  • Cats
  • Female
  • Gangliosidosis, GM1 (metabolism, veterinary)
  • Humans
  • Lipid Metabolism
  • Lysophospholipids (metabolism)
  • Male
  • Mice, 129 Strain
  • Mice, Knockout
  • Monoglycerides (metabolism)
  • Sandhoff Disease (metabolism, veterinary)
  • Ursidae

Join CureHunter, for free Research Interface BASIC access!

Take advantage of free CureHunter research engine access to explore the best drug and treatment options for any disease. Find out why thousands of doctors, pharma researchers and patient activists around the world use CureHunter every day.
Realize the full power of the drug-disease research graph!


Choose Username:
Email:
Password:
Verify Password:
Enter Code Shown: