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Items: 1 to 20 of 106

1.

Selective reduction of bis(monoacylglycero)phosphate ameliorates the storage burden in a THP-1 macrophage model of Gaucher disease.

Hein LK, Duplock S, Fuller M.

J Lipid Res. 2013 Jun;54(6):1691-7. doi: 10.1194/jlr.M038232. Epub 2013 Apr 6.

2.

Lipid composition of membrane rafts, isolated with and without detergent, from the spleen of a mouse model of Gaucher disease.

Hattersley KJ, Hein LK, Fuller M.

Biochem Biophys Res Commun. 2013 Dec 6;442(1-2):62-7. doi: 10.1016/j.bbrc.2013.11.009. Epub 2013 Nov 9.

PMID:
24220330
3.

Secondary sphingolipid accumulation in a macrophage model of Gaucher disease.

Hein LK, Meikle PJ, Hopwood JJ, Fuller M.

Mol Genet Metab. 2007 Dec;92(4):336-45. Epub 2007 Sep 18.

PMID:
17881272
4.

Lipid composition of microdomains is altered in a cell model of Gaucher disease.

Hein LK, Duplock S, Hopwood JJ, Fuller M.

J Lipid Res. 2008 Aug;49(8):1725-34. doi: 10.1194/jlr.M800092-JLR200. Epub 2008 Apr 21.

5.

Selective normalisation of regional brain bis(monoacylglycero)phosphate in the mucopolysaccharidosis 1 (Hurler) mouse.

Saville JT, Lehmann RJ, Derrick-Roberts AL, Fuller M.

Exp Neurol. 2016 Mar;277:68-75. doi: 10.1016/j.expneurol.2015.12.012. Epub 2015 Dec 19.

PMID:
26710715
6.

Metabolism of phosphatidylglycerol and bis(monoacylglycero)-phosphate in macrophage subcellular fractions.

Waite M, King L, Thornburg T, Osthoff G, Thuren TY.

J Biol Chem. 1990 Dec 15;265(35):21720-6.

7.
9.

Hsp70 stabilizes lysosomes and reverts Niemann-Pick disease-associated lysosomal pathology.

Kirkegaard T, Roth AG, Petersen NH, Mahalka AK, Olsen OD, Moilanen I, Zylicz A, Knudsen J, Sandhoff K, Arenz C, Kinnunen PK, Nylandsted J, Jäättelä M.

Nature. 2010 Jan 28;463(7280):549-53. doi: 10.1038/nature08710.

PMID:
20111001
10.

Biological function of the cellular lipid BMP-BMP as a key activator for cholesterol sorting and membrane digestion.

Gallala HD, Sandhoff K.

Neurochem Res. 2011 Sep;36(9):1594-600. doi: 10.1007/s11064-010-0337-6. Epub 2010 Dec 7. Review.

PMID:
21136156
11.

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

Akgoc Z, Sena-Esteves M, Martin DR, Han X, d'Azzo A, Seyfried TN.

J Lipid Res. 2015 May;56(5):1006-13. doi: 10.1194/jlr.M057851. Epub 2015 Mar 20.

12.

α/β Hydrolase Domain-containing 6 (ABHD6) Degrades the Late Endosomal/Lysosomal Lipid Bis(monoacylglycero)phosphate.

Pribasnig MA, Mrak I, Grabner GF, Taschler U, Knittelfelder O, Scherz B, Eichmann TO, Heier C, Grumet L, Kowaliuk J, Romauch M, Holler S, Anderl F, Wolinski H, Lass A, Breinbauer R, Marsche G, Brown JM, Zimmermann R.

J Biol Chem. 2015 Dec 11;290(50):29869-81. doi: 10.1074/jbc.M115.669168. Epub 2015 Oct 21.

13.

Selective decrease of bis(monoacylglycero)phosphate content in macrophages by high supplementation with docosahexaenoic acid.

Bouvier J, Zemski Berry KA, Hullin-Matsuda F, Makino A, Michaud S, Geloën A, Murphy RC, Kobayashi T, Lagarde M, Delton-Vandenbroucke I.

J Lipid Res. 2009 Feb;50(2):243-55. doi: 10.1194/jlr.M800300-JLR200. Epub 2008 Sep 22.

14.

AAV8-mediated expression of glucocerebrosidase ameliorates the storage pathology in the visceral organs of a mouse model of Gaucher disease.

McEachern KA, Nietupski JB, Chuang WL, Armentano D, Johnson J, Hutto E, Grabowski GA, Cheng SH, Marshall J.

J Gene Med. 2006 Jun;8(6):719-29.

PMID:
16528760
15.

Sphingolipids and lysosomal pathologies.

Schulze H, Sandhoff K.

Biochim Biophys Acta. 2014 May;1841(5):799-810. doi: 10.1016/j.bbalip.2013.10.015. Epub 2013 Oct 31. Review.

PMID:
24184515
17.

Macrophage models of Gaucher disease for evaluating disease pathogenesis and candidate drugs.

Aflaki E, Stubblefield BK, Maniwang E, Lopez G, Moaven N, Goldin E, Marugan J, Patnaik S, Dutra A, Southall N, Zheng W, Tayebi N, Sidransky E.

Sci Transl Med. 2014 Jun 11;6(240):240ra73. doi: 10.1126/scitranslmed.3008659.

18.

Correlation between enzyme activity and substrate storage in a cell culture model system for Gaucher disease.

Schueler UH, Kolter T, Kaneski CR, Zirzow GC, Sandhoff K, Brady RO.

J Inherit Metab Dis. 2004;27(5):649-58.

PMID:
15669681
19.

Effect of lysosomal storage on bis(monoacylglycero)phosphate.

Meikle PJ, Duplock S, Blacklock D, Whitfield PD, Macintosh G, Hopwood JJ, Fuller M.

Biochem J. 2008 Apr 1;411(1):71-8.

PMID:
18052935
20.

Bis(monoacylglycero)phosphate as a Macrophage Enriched Phospholipid.

Akgoc Z, Iosim S, Seyfried TN.

Lipids. 2015 Sep;50(9):907-12. doi: 10.1007/s11745-015-4045-5. Epub 2015 Jul 24.

PMID:
26205346

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