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Items: 1 to 50 of 65

1.

Novel Vector Design and Hexosaminidase Variant Enabling Self-Complementary Adeno-Associated Virus for the Treatment of Tay-Sachs Disease.

Karumuthil-Melethil S, Nagabhushan Kalburgi S, Thompson P, Tropak M, Kaytor MD, Keimel JG, Mark BL, Mahuran D, Walia JS, Gray SJ.

Hum Gene Ther. 2016 Jul;27(7):509-21. doi: 10.1089/hum.2016.013.

2.

Construction of a hybrid β-hexosaminidase subunit capable of forming stable homodimers that hydrolyze GM2 ganglioside in vivo.

Tropak MB, Yonekawa S, Karumuthil-Melethil S, Thompson P, Wakarchuk W, Gray SJ, Walia JS, Mark BL, Mahuran D.

Mol Ther Methods Clin Dev. 2016 Mar 2;3:15057. doi: 10.1038/mtm.2015.57. eCollection 2016.

3.

Pyrimethamine Derivatives: Insight into Binding Mechanism and Improved Enhancement of Mutant β-N-acetylhexosaminidase Activity.

Tropak MB, Zhang J, Yonekawa S, Rigat BA, Aulakh VS, Smith MR, Hwang HJ, Ciufolini MA, Mahuran DJ.

J Med Chem. 2015 Jun 11;58(11):4483-93. doi: 10.1021/jm5017895. Epub 2015 Jun 2.

PMID:
25984755
4.

TMEM43 mutation p.S358L alters intercalated disc protein expression and reduces conduction velocity in arrhythmogenic right ventricular cardiomyopathy.

Siragam V, Cui X, Masse S, Ackerley C, Aafaqi S, Strandberg L, Tropak M, Fridman MD, Nanthakumar K, Liu J, Sun Y, Su B, Wang C, Liu X, Yan Y, Mendlowitz A, Hamilton RM.

PLoS One. 2014 Oct 24;9(10):e109128. doi: 10.1371/journal.pone.0109128. eCollection 2014.

5.

Synthesis of 1,5-dideoxy-1,5-iminoribitol C-glycosides through a nitrone-olefin cycloaddition domino strategy: identification of pharmacological chaperones of mutant human lysosomal β-galactosidase.

Siriwardena A, Sonawane DP, Bande OP, Markad PR, Yonekawa S, Tropak MB, Ghosh S, Chopade BA, Mahuran DJ, Dhavale DD.

J Org Chem. 2014 May 16;79(10):4398-404. doi: 10.1021/jo500328u. Epub 2014 Apr 28.

PMID:
24735108
6.

Transcutaneous electrical nerve stimulation as a novel method of remote preconditioning: in vitro validation in an animal model and first human observations.

Merlocco AC, Redington KL, Disenhouse T, Strantzas SC, Gladstone R, Wei C, Tropak MB, Manlhiot C, Li J, Redington AN.

Basic Res Cardiol. 2014 May;109(3):406. doi: 10.1007/s00395-014-0406-0. Epub 2014 Mar 7.

PMID:
24604614
7.

Remote cardioprotection by transfer of coronary effluent from ischemic preconditioned rabbit heart preserves mitochondrial integrity and function via adenosine receptor activation.

Leung CH, Wang L, Nielsen JM, Tropak MB, Fu YY, Kato H, Callahan J, Redington AN, Caldarone CA.

Cardiovasc Drugs Ther. 2014 Feb;28(1):7-17. doi: 10.1007/s10557-013-6489-2.

PMID:
24018748
8.

Interaction of δ and κ opioid receptors with adenosine A1 receptors mediates cardioprotection by remote ischemic preconditioning.

Surendra H, Diaz RJ, Harvey K, Tropak M, Callahan J, Hinek A, Hossain T, Redington A, Wilson GJ.

J Mol Cell Cardiol. 2013 Jul;60:142-50. doi: 10.1016/j.yjmcc.2013.04.010. Epub 2013 Apr 19.

PMID:
23608604
9.

Evaluation of N-nonyl-deoxygalactonojirimycin as a pharmacological chaperone for human GM1 gangliosidosis leads to identification of a feline model suitable for testing enzyme enhancement therapy.

Rigat BA, Tropak MB, Buttner J, Crushell E, Benedict D, Callahan JW, Martin DR, Mahuran DJ.

Mol Genet Metab. 2012 Sep;107(1-2):203-12. doi: 10.1016/j.ymgme.2012.06.007. Epub 2012 Jun 19.

10.

Pharmacological chaperones facilitate the post-ER transport of recombinant N370S mutant β-glucocerebrosidase in plant cells: evidence that N370S is a folding mutant.

Babajani G, Tropak MB, Mahuran DJ, Kermode AR.

Mol Genet Metab. 2012 Jul;106(3):323-9. doi: 10.1016/j.ymgme.2012.04.018. Epub 2012 Apr 26.

11.

Exercise-induced cardioprotection is mediated by a bloodborne, transferable factor.

Michelsen MM, Støttrup NB, Schmidt MR, Løfgren B, Jensen RV, Tropak M, St-Michel EJ, Redington AN, Bøtker HE.

Basic Res Cardiol. 2012 May;107(3):260. doi: 10.1007/s00395-012-0260-x. Epub 2012 Mar 18.

PMID:
22426795
12.

Rapid assembly of a library of lipophilic iminosugars via the thiol-ene reaction yields promising pharmacological chaperones for the treatment of Gaucher disease.

Goddard-Borger ED, Tropak MB, Yonekawa S, Tysoe C, Mahuran DJ, Withers SG.

J Med Chem. 2012 Mar 22;55(6):2737-45. doi: 10.1021/jm201633y. Epub 2012 Mar 6.

13.

Remote cardioprotection by direct peripheral nerve stimulation and topical capsaicin is mediated by circulating humoral factors.

Redington KL, Disenhouse T, Strantzas SC, Gladstone R, Wei C, Tropak MB, Dai X, Manlhiot C, Li J, Redington AN.

Basic Res Cardiol. 2012 Mar;107(2):241. doi: 10.1007/s00395-011-0241-5. Epub 2012 Jan 10.

PMID:
22231674
14.

Production of active human glucocerebrosidase in seeds of Arabidopsis thaliana complex-glycan-deficient (cgl) plants.

He X, Galpin JD, Tropak MB, Mahuran D, Haselhorst T, von Itzstein M, Kolarich D, Packer NH, Miao Y, Jiang L, Grabowski GA, Clarke LA, Kermode AR.

Glycobiology. 2012 Apr;22(4):492-503. doi: 10.1093/glycob/cwr157. Epub 2011 Nov 7.

15.

Tailoring the specificity and reactivity of a mechanism-based inactivator of glucocerebrosidase for potential therapeutic applications.

Rempel BP, Tropak MB, Mahuran DJ, Withers SG.

Angew Chem Int Ed Engl. 2011 Oct 24;50(44):10381-3. doi: 10.1002/anie.201103924. Epub 2011 Sep 13. No abstract available.

PMID:
21915968
16.

Synthesis, kinetic evaluation and cell-based analysis of C-alkylated isofagomines as chaperones of β-glucocerebrosidase.

Hill T, Tropak MB, Mahuran D, Withers SG.

Chembiochem. 2011 Sep 19;12(14):2151-4. doi: 10.1002/cbic.201100332. Epub 2011 Jul 12. No abstract available.

PMID:
21751327
17.

1-Deoxy-D-galactonojirimycins with dansyl capped N-substituents as β-galactosidase inhibitors and potential probes for GM1 gangliosidosis affected cell lines.

Fröhlich RF, Furneaux RH, Mahuran DJ, Saf R, Stütz AE, Tropak MB, Wicki J, Withers SG, Wrodnigg TM.

Carbohydr Res. 2011 Sep 6;346(12):1592-8. doi: 10.1016/j.carres.2011.05.010. Epub 2011 May 24.

18.

Potent neuroprotection induced by remote preconditioning in a rat model of neonatal cerebral hypoxic-ischemic injury.

Tropak MB, Shi H, Li J, Dai X, Redington AN, Askalan R.

J Thorac Cardiovasc Surg. 2011 Jul;142(1):233-5. doi: 10.1016/j.jtcvs.2011.04.003. Epub 2011 May 8. No abstract available.

19.

Crystal structure of β-hexosaminidase B in complex with pyrimethamine, a potential pharmacological chaperone.

Bateman KS, Cherney MM, Mahuran DJ, Tropak M, James MN.

J Med Chem. 2011 Mar 10;54(5):1421-9. doi: 10.1021/jm101443u. Epub 2011 Jan 25.

20.

Remote preconditioning provides potent cardioprotection via PI3K/Akt activation and is associated with nuclear accumulation of β-catenin.

Li J, Xuan W, Yan R, Tropak MB, Jean-St-Michel E, Liang W, Gladstone R, Backx PH, Kharbanda RK, Redington AN.

Clin Sci (Lond). 2011 May;120(10):451-62. doi: 10.1042/CS20100466.

PMID:
21143191
21.

Remote preconditioning improves maximal performance in highly trained athletes.

Jean-St-Michel E, Manlhiot C, Li J, Tropak M, Michelsen MM, Schmidt MR, McCrindle BW, Wells GD, Redington AN.

Med Sci Sports Exerc. 2011 Jul;43(7):1280-6. doi: 10.1249/MSS.0b013e318206845d.

PMID:
21131871
22.

Identification of drug candidates which increase cytochrome c oxidase activity in deficient patient fibroblasts.

Maj M, Sriskandarajah N, Hung V, Browne I, Shah B, Weadge A, Jamieson NL, Tropak M, Cameron JM, Addis JB, Robinson BH.

Mitochondrion. 2011 Mar;11(2):264-72. doi: 10.1016/j.mito.2010.10.002. Epub 2010 Nov 2.

PMID:
21050896
23.

An open-label Phase I/II clinical trial of pyrimethamine for the treatment of patients affected with chronic GM2 gangliosidosis (Tay-Sachs or Sandhoff variants).

Clarke JT, Mahuran DJ, Sathe S, Kolodny EH, Rigat BA, Raiman JA, Tropak MB.

Mol Genet Metab. 2011 Jan;102(1):6-12. doi: 10.1016/j.ymgme.2010.09.004. Epub 2010 Sep 17.

24.

Pretreatment with the nitric oxide donor SNAP or nerve transection blocks humoral preconditioning by remote limb ischemia or intra-arterial adenosine.

Steensrud T, Li J, Dai X, Manlhiot C, Kharbanda RK, Tropak M, Redington A.

Am J Physiol Heart Circ Physiol. 2010 Nov;299(5):H1598-603. doi: 10.1152/ajpheart.00396.2010. Epub 2010 Aug 27.

25.

Fluorous iminoalditols: a new family of glycosidase inhibitors and pharmacological chaperones.

Schitter G, Steiner AJ, Pototschnig G, Scheucher E, Thonhofer M, Tarling CA, Withers SG, Fantur K, Paschke E, Mahuran DJ, Rigat BA, Tropak MB, Illaszewicz C, Saf R, Stütz AE, Wrodnigg TM.

Chembiochem. 2010 Sep 24;11(14):2026-33. doi: 10.1002/cbic.201000192.

26.

Synthesis of lipophilic 1-deoxygalactonojirimycin derivatives as D-galactosidase inhibitors.

Schitter G, Scheucher E, Steiner AJ, Stütz AE, Thonhofer M, Tarling CA, Withers SG, Wicki J, Fantur K, Paschke E, Mahuran DJ, Rigat BA, Tropak M, Wrodnigg TM.

Beilstein J Org Chem. 2010 Mar 1;6:21. doi: 10.3762/bjoc.6.21.

27.

1-Deoxynojirimycins with dansyl capped N-substituents as probes for Morbus Gaucher affected cell lines.

Fröhlich RF, Furneaux RH, Mahuran DJ, Rigat BA, Stütz AE, Tropak MB, Wicki J, Withers SG, Wrodnigg TM.

Carbohydr Res. 2010 Jul 2;345(10):1371-6. doi: 10.1016/j.carres.2010.04.015. Epub 2010 Apr 24.

28.

A sensitive fluorescence-based assay for monitoring GM2 ganglioside hydrolysis in live patient cells and their lysates.

Tropak MB, Bukovac SW, Rigat BA, Yonekawa S, Wakarchuk W, Mahuran DJ.

Glycobiology. 2010 Mar;20(3):356-65. doi: 10.1093/glycob/cwp183. Epub 2009 Nov 16.

29.

Identification and characterization of ambroxol as an enzyme enhancement agent for Gaucher disease.

Maegawa GH, Tropak MB, Buttner JD, Rigat BA, Fuller M, Pandit D, Tang L, Kornhaber GJ, Hamuro Y, Clarke JT, Mahuran DJ.

J Biol Chem. 2009 Aug 28;284(35):23502-16. doi: 10.1074/jbc.M109.012393. Epub 2009 Jul 3.

30.

2-Acetamino-1,2-dideoxynojirimycin-lysine hybrids as hexosaminidase inhibitors.

Steiner AJ, Schitter G, Stütz AE, Wrodnigg TM, Tarling CA, Withers SG, Mahuran DJ, Tropak MB.

Tetrahedron Asymmetry. 2009 May 1;20(6-8):832-835.

31.

Transient limb ischaemia remotely preconditions through a humoral mechanism acting directly on the myocardium: evidence suggesting cross-species protection.

Shimizu M, Tropak M, Diaz RJ, Suto F, Surendra H, Kuzmin E, Li J, Gross G, Wilson GJ, Callahan J, Redington AN.

Clin Sci (Lond). 2009 Aug 3;117(5):191-200. doi: 10.1042/CS20080523.

PMID:
19175358
32.

1-Deoxygalactonojirimycin-lysine hybrids as potent D-galactosidase inhibitors.

Steiner AJ, Schitter G, Stütz AE, Wrodnigg TM, Tarling CA, Withers SG, Fantur K, Mahuran D, Paschke E, Tropak M.

Bioorg Med Chem. 2008 Dec 15;16(24):10216-20. doi: 10.1016/j.bmc.2008.10.054. Epub 2008 Oct 26.

33.

Identification of pharmacological chaperones for Gaucher disease and characterization of their effects on beta-glucocerebrosidase by hydrogen/deuterium exchange mass spectrometry.

Tropak MB, Kornhaber GJ, Rigat BA, Maegawa GH, Buttner JD, Blanchard JE, Murphy C, Tuske SJ, Coales SJ, Hamuro Y, Brown ED, Mahuran DJ.

Chembiochem. 2008 Nov 3;9(16):2650-62. doi: 10.1002/cbic.200800304.

34.

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.

35.

Remote ischemic preconditioning elaborates a transferable blood-borne effector that protects mitochondrial structure and function and preserves myocardial performance after neonatal cardioplegic arrest.

Wang L, Oka N, Tropak M, Callahan J, Lee J, Wilson G, Redington A, Caldarone CA.

J Thorac Cardiovasc Surg. 2008 Aug;136(2):335-42. doi: 10.1016/j.jtcvs.2007.12.055. Epub 2008 Jun 2.

37.

High-throughput screening for human lysosomal beta-N-Acetyl hexosaminidase inhibitors acting as pharmacological chaperones.

Tropak MB, Blanchard JE, Withers SG, Brown ED, Mahuran D.

Chem Biol. 2007 Feb;14(2):153-64.

38.

Pyrimethamine as a potential pharmacological chaperone for late-onset forms of GM2 gangliosidosis.

Maegawa GH, Tropak M, Buttner J, Stockley T, Kok F, Clarke JT, Mahuran DJ.

J Biol Chem. 2007 Mar 23;282(12):9150-61. Epub 2007 Jan 21.

39.

The natural history of juvenile or subacute GM2 gangliosidosis: 21 new cases and literature review of 134 previously reported.

Maegawa GH, Stockley T, Tropak M, Banwell B, Blaser S, Kok F, Giugliani R, Mahuran D, Clarke JT.

Pediatrics. 2006 Nov;118(5):e1550-62. Epub 2006 Oct 2. Review. Erratum in: Pediatrics. 2007 Oct;120(4):936.

40.

Identification of the gene encoding the enzyme deficient in mucopolysaccharidosis IIIC (Sanfilippo disease type C).

Fan X, Zhang H, Zhang S, Bagshaw RD, Tropak MB, Callahan JW, Mahuran DJ.

Am J Hum Genet. 2006 Oct;79(4):738-44. Epub 2006 Aug 23.

41.

Comparison of HCMV IE and EF-1 promoters for the stable expression of beta-subunit of hexosaminidase in CHO cell lines.

Sinici I, Zarghooni M, Tropak MB, Mahuran DJ, Ozkara HA.

Biochem Genet. 2006 Apr;44(3-4):173-80. Epub 2006 Apr 28. No abstract available.

43.

An alpha-subunit loop structure is required for GM2 activator protein binding by beta-hexosaminidase A.

Zarghooni M, Bukovac S, Tropak M, Callahan J, Mahuran D.

Biochem Biophys Res Commun. 2004 Nov 19;324(3):1048-52.

44.

Pharmacological enhancement of beta-hexosaminidase activity in fibroblasts from adult Tay-Sachs and Sandhoff Patients.

Tropak MB, Reid SP, Guiral M, Withers SG, Mahuran D.

J Biol Chem. 2004 Apr 2;279(14):13478-87. Epub 2004 Jan 14.

45.

Cloning and molecular characterization of two splice variants of a new putative member of the Siglec-3-like subgroup of Siglecs.

Foussias G, Taylor SM, Yousef GM, Tropak MB, Ordon MH, Diamandis EP.

Biochem Biophys Res Commun. 2001 Jun 22;284(4):887-99.

PMID:
11409877
46.

Myelin-associated glycoprotein binding to gangliosides. Structural specificity and functional implications.

Schnaar RL, Collins BE, Wright LP, Kiso M, Tropak MB, Roder JC, Crocker PR.

Ann N Y Acad Sci. 1998 Jun 19;845:92-105. Review.

PMID:
9668345
48.
49.

Cytotoxicity of B72.3XOKT3 bispecific antibody recognizing human colon cancer.

Shpitz B, Stern H, Sandhu JS, Reilley RM, Tropak MB, Jansz G, Xu J, Gallinger S.

J Surg Res. 1996 Feb 15;61(1):134-8.

PMID:
8769955
50.

Conservation of functionally important epitopes on myelin associated glycoprotein (MAG).

Tropak MB, Jansz GF, Abramow-Newerly W, Roder JC.

Comp Biochem Physiol B Biochem Mol Biol. 1995 Oct;112(2):345-54.

PMID:
7584863

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