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Items: 1 to 20 of 113

1.

Selective action of the iminosugar isofagomine, a pharmacological chaperone for mutant forms of acid-beta-glucosidase.

Steet R, Chung S, Lee WS, Pine CW, Do H, Kornfeld S.

Biochem Pharmacol. 2007 May 1;73(9):1376-83. Epub 2006 Dec 15.

2.

The iminosugar isofagomine increases the activity of N370S mutant acid beta-glucosidase in Gaucher fibroblasts by several mechanisms.

Steet RA, Chung S, Wustman B, Powe A, Do H, Kornfeld SA.

Proc Natl Acad Sci U S A. 2006 Sep 12;103(37):13813-8. Epub 2006 Aug 31.

3.

The pharmacological chaperone isofagomine increases the activity of the Gaucher disease L444P mutant form of beta-glucosidase.

Khanna R, Benjamin ER, Pellegrino L, Schilling A, Rigat BA, Soska R, Nafar H, Ranes BE, Feng J, Lun Y, Powe AC, Palling DJ, Wustman BA, Schiffmann R, Mahuran DJ, Lockhart DJ, Valenzano KJ.

FEBS J. 2010 Apr;277(7):1618-38. doi: 10.1111/j.1742-4658.2010.07588.x. Epub 2010 Feb 10.

4.
5.

Ex vivo and in vivo effects of isofagomine on acid β-glucosidase variants and substrate levels in Gaucher disease.

Sun Y, Liou B, Xu YH, Quinn B, Zhang W, Hamler R, Setchell KD, Grabowski GA.

J Biol Chem. 2012 Feb 3;287(6):4275-87. doi: 10.1074/jbc.M111.280016. Epub 2011 Dec 13.

6.

Miglustat (NB-DNJ) works as a chaperone for mutated acid beta-glucosidase in cells transfected with several Gaucher disease mutations.

Alfonso P, Pampín S, Estrada J, Rodríguez-Rey JC, Giraldo P, Sancho J, Pocoví M.

Blood Cells Mol Dis. 2005 Sep-Oct;35(2):268-76.

PMID:
16039881
7.

Isofagomine induced stabilization of glucocerebrosidase.

Kornhaber GJ, Tropak MB, Maegawa GH, Tuske SJ, Coales SJ, Mahuran DJ, Hamuro Y.

Chembiochem. 2008 Nov 3;9(16):2643-9. doi: 10.1002/cbic.200800249.

8.

Selective chaperone effect of aminocyclitol derivatives on G202R and other mutant glucocerebrosidases causing Gaucher disease.

Serra-Vinardell J, Díaz L, Gutiérrez-de Terán H, Sánchez-Ollé G, Bujons J, Michelakakis H, Mavridou I, Aerts JM, Delgado A, Grinberg D, Vilageliu L, Casas J.

Int J Biochem Cell Biol. 2014 Sep;54:245-54. doi: 10.1016/j.biocel.2014.07.017. Epub 2014 Jul 30.

PMID:
25084554
9.

Structure of acid beta-glucosidase with pharmacological chaperone provides insight into Gaucher disease.

Lieberman RL, Wustman BA, Huertas P, Powe AC Jr, Pine CW, Khanna R, Schlossmacher MG, Ringe D, Petsko GA.

Nat Chem Biol. 2007 Feb;3(2):101-7. Epub 2006 Dec 24.

PMID:
17187079
10.

Chaperone activity of bicyclic nojirimycin analogues for Gaucher mutations in comparison with N-(n-nonyl)deoxynojirimycin.

Luan Z, Higaki K, Aguilar-Moncayo M, Ninomiya H, Ohno K, García-Moreno MI, Ortiz Mellet C, García Fernández JM, Suzuki Y.

Chembiochem. 2009 Nov 23;10(17):2780-92. doi: 10.1002/cbic.200900442.

PMID:
19830760
11.
12.

Alpha-1-C-octyl-1-deoxynojirimycin as a pharmacological chaperone for Gaucher disease.

Yu L, Ikeda K, Kato A, Adachi I, Godin G, Compain P, Martin O, Asano N.

Bioorg Med Chem. 2006 Dec 1;14(23):7736-44. Epub 2006 Aug 21.

PMID:
16919960
13.

Chemical chaperones increase the cellular activity of N370S beta -glucosidase: a therapeutic strategy for Gaucher disease.

Sawkar AR, Cheng WC, Beutler E, Wong CH, Balch WE, Kelly JW.

Proc Natl Acad Sci U S A. 2002 Nov 26;99(24):15428-33. Epub 2002 Nov 14.

14.

Isofagomine increases lysosomal delivery of exogenous glucocerebrosidase.

Shen JS, Edwards NJ, Hong YB, Murray GJ.

Biochem Biophys Res Commun. 2008 May 16;369(4):1071-5. doi: 10.1016/j.bbrc.2008.02.125. Epub 2008 Mar 6.

15.

Crystal structures of complexes of N-butyl- and N-nonyl-deoxynojirimycin bound to acid beta-glucosidase: insights into the mechanism of chemical chaperone action in Gaucher disease.

Brumshtein B, Greenblatt HM, Butters TD, Shaaltiel Y, Aviezer D, Silman I, Futerman AH, Sussman JL.

J Biol Chem. 2007 Sep 28;282(39):29052-8. Epub 2007 Jul 31.

16.

N-butyldeoxygalactonojirimycin inhibits glycolipid biosynthesis but does not affect N-linked oligosaccharide processing.

Platt FM, Neises GR, Karlsson GB, Dwek RA, Butters TD.

J Biol Chem. 1994 Oct 28;269(43):27108-14.

17.

N-butyldeoxynojirimycin is a novel inhibitor of glycolipid biosynthesis.

Platt FM, Neises GR, Dwek RA, Butters TD.

J Biol Chem. 1994 Mar 18;269(11):8362-5.

18.

A Fluorescent sp2-iminosugar with pharmacological chaperone activity for gaucher disease: synthesis and intracellular distribution studies.

Luan Z, Higaki K, Aguilar-Moncayo M, Li L, Ninomiya H, Nanba E, Ohno K, García-Moreno MI, Ortiz Mellet C, García Fernández JM, Suzuki Y.

Chembiochem. 2010 Nov 22;11(17):2453-64. doi: 10.1002/cbic.201000323.

PMID:
21064079
19.

Promising results of the chaperone effect caused by imino sugars and aminocyclitol derivatives on mutant glucocerebrosidases causing Gaucher disease.

Sánchez-Ollé G, Duque J, Egido-Gabás M, Casas J, Lluch M, Chabás A, Grinberg D, Vilageliu L.

Blood Cells Mol Dis. 2009 Mar-Apr;42(2):159-66. doi: 10.1016/j.bcmd.2008.11.002. Epub 2009 Jan 22.

PMID:
19167250
20.

Effects of pH and iminosugar pharmacological chaperones on lysosomal glycosidase structure and stability.

Lieberman RL, D'aquino JA, Ringe D, Petsko GA.

Biochemistry. 2009 Jun 9;48(22):4816-27. doi: 10.1021/bi9002265.

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