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Persistent reduction in sialylation of cerebral glycoproteins following postnatal inflammatory exposure.

Demina EP, Pierre WC, Nguyen ALA, Londono I, Reiz B, Zou C, Chakraberty R, Cairo CW, Pshezhetsky AV, Lodygensky GA.

J Neuroinflammation. 2018 Dec 5;15(1):336. doi: 10.1186/s12974-018-1367-2.


Selective inhibitors of human neuraminidase 1 (NEU1).

Guo T, Heon-Roberts R, Zou C, Zheng RB, Pshezhetsky AV, Cairo CW.

J Med Chem. 2018 Nov 20. doi: 10.1021/acs.jmedchem.8b01411. [Epub ahead of print]


Presence of aberrant epididymal tubules revealing undifferentiated epithelial cells and absence of spermatozoa in a combined neuraminidase-3 and -4 deficient adult mouse model.

Oliveira R, Hermo L, Pshezhetsky AV, Morales CR.

PLoS One. 2018 Oct 25;13(10):e0206173. doi: 10.1371/journal.pone.0206173. eCollection 2018.


Anatomical changes and pathophysiology of the brain in mucopolysaccharidosis disorders.

Bigger BW, Begley DJ, Virgintino D, Pshezhetsky AV.

Mol Genet Metab. 2018 Dec;125(4):322-331. doi: 10.1016/j.ymgme.2018.08.003. Epub 2018 Aug 10. Review.


Keeping it trim: roles of neuraminidases in CNS function.

Pshezhetsky AV, Ashmarina M.

Glycoconj J. 2018 Aug;35(4):375-386. doi: 10.1007/s10719-018-9837-4. Epub 2018 Aug 7. Review.


A novel adeno-associated virus capsid with enhanced neurotropism corrects a lysosomal transmembrane enzyme deficiency.

Tordo J, O'Leary C, Antunes ASLM, Palomar N, Aldrin-Kirk P, Basche M, Bennett A, D'Souza Z, Gleitz H, Godwin A, Holley RJ, Parker H, Liao AY, Rouse P, Youshani AS, Dridi L, Martins C, Levade T, Stacey KB, Davis DM, Dyer A, Clément N, Björklund T, Ali RR, Agbandje-McKenna M, Rahim AA, Pshezhetsky A, Waddington SN, Linden RM, Bigger BW, Henckaerts E.

Brain. 2018 Jul 1;141(7):2014-2031. doi: 10.1093/brain/awy126.


Neuraminidase 1 activates insulin receptor and reverses insulin resistance in obese mice.

Fougerat A, Pan X, Smutova V, Heveker N, Cairo CW, Issad T, Larrivée B, Medin JA, Pshezhetsky AV.

Mol Metab. 2018 Jun;12:76-88. doi: 10.1016/j.molmet.2018.03.017. Epub 2018 Apr 21.


Selective Inhibitors of Human Neuraminidase 3.

Guo T, Dätwyler P, Demina E, Richards MR, Ge P, Zou C, Zheng R, Fougerat A, Pshezhetsky AV, Ernst B, Cairo CW.

J Med Chem. 2018 Mar 8;61(5):1990-2008. doi: 10.1021/acs.jmedchem.7b01574. Epub 2018 Feb 21.


Neuraminidases 3 and 4 regulate neuronal function by catabolizing brain gangliosides.

Pan X, De Aragão CBP, Velasco-Martin JP, Priestman DA, Wu HY, Takahashi K, Yamaguchi K, Sturiale L, Garozzo D, Platt FM, Lamarche-Vane N, Morales CR, Miyagi T, Pshezhetsky AV.

FASEB J. 2017 Aug;31(8):3467-3483. doi: 10.1096/fj.201601299R. Epub 2017 Apr 25.


Atypical juvenile presentation of GM2 gangliosidosis AB in a patient compound-heterozygote for c.259G > T and c.164C > T mutations in the GM2A gene.

Martins C, Brunel-Guitton C, Lortie A, Gauvin F, Morales CR, Mitchell GA, Pshezhetsky AV.

Mol Genet Metab Rep. 2017 Apr 7;11:24-29. doi: 10.1016/j.ymgmr.2017.01.017. eCollection 2017 Jun.


Mice, double deficient in lysosomal serine carboxypeptidases Scpep1 and Cathepsin A develop the hyperproliferative vesicular corneal dystrophy and hypertrophic skin thickenings.

Pan X, Wang Y, Lübke T, Hinek A, Pshezhetsky AV.

PLoS One. 2017 Feb 24;12(2):e0172854. doi: 10.1371/journal.pone.0172854. eCollection 2017.


Loss of Cellular Sialidases Does Not Affect the Sialylation Status of the Prion Protein but Increases the Amounts of Its Proteolytic Fragment C1.

Katorcha E, Klimova N, Makarava N, Savtchenko R, Pan X, Annunziata I, Takahashi K, Miyagi T, Pshezhetsky AV, d'Azzo A, Baskakov IV.

PLoS One. 2015 Nov 16;10(11):e0143218. doi: 10.1371/journal.pone.0143218. eCollection 2015.


Novel Direct Assay for Acetyl-CoA:α-Glucosaminide N-Acetyltransferase Using BODIPY-Glucosamine as a Substrate.

Choi Y, Tuzikov AB, Ovchinnikova TV, Bovin NV, Pshezhetsky AV.

JIMD Rep. 2015 Oct 23. [Epub ahead of print]


Neuroinflammation, mitochondrial defects and neurodegeneration in mucopolysaccharidosis III type C mouse model.

Martins C, Hůlková H, Dridi L, Dormoy-Raclet V, Grigoryeva L, Choi Y, Langford-Smith A, Wilkinson FL, Ohmi K, DiCristo G, Hamel E, Ausseil J, Cheillan D, Moreau A, Svobodová E, Hájková Z, Tesařová M, Hansíková H, Bigger BW, Hrebícek M, Pshezhetsky AV.

Brain. 2015 Feb;138(Pt 2):336-55. doi: 10.1093/brain/awu355. Epub 2015 Jan 6. Erratum in: Brain. 2015 Jul;138(Pt 7):e366.


Screening for Pompe Disease in Specific At-Risk Populations with Sleep-Disordered Breathing.

Kaminska M, Noel F, Genge A, Melancon SB, Pshezhetsky A, Petrof BJ.

J Neuromuscul Dis. 2015;2(s1):S44. No abstract available.


Therapeutic strategies based on modified U1 snRNAs and chaperones for Sanfilippo C splicing mutations.

Matos L, Canals I, Dridi L, Choi Y, Prata MJ, Jordan P, Desviat LR, Pérez B, Pshezhetsky AV, Grinberg D, Alves S, Vilageliu L.

Orphanet J Rare Dis. 2014 Dec 10;9:180. doi: 10.1186/s13023-014-0180-y.


De novo mutations in moderate or severe intellectual disability.

Hamdan FF, Srour M, Capo-Chichi JM, Daoud H, Nassif C, Patry L, Massicotte C, Ambalavanan A, Spiegelman D, Diallo O, Henrion E, Dionne-Laporte A, Fougerat A, Pshezhetsky AV, Venkateswaran S, Rouleau GA, Michaud JL.

PLoS Genet. 2014 Oct 30;10(10):e1004772. doi: 10.1371/journal.pgen.1004772. eCollection 2014 Oct.


Mitochondrial damage and cholesterol storage in human hepatocellular carcinoma cells with silencing of UBIAD1 gene expression.

Morales CR, Grigoryeva LS, Pan X, Bruno L, Hickson G, Ngo MH, McMaster CR, Samuels ME, Pshezhetsky AV.

Mol Genet Metab Rep. 2014 Sep 19;1:407-411. eCollection 2014.


Structural basis for substrate specificity of mammalian neuraminidases.

Smutova V, Albohy A, Pan X, Korchagina E, Miyagi T, Bovin N, Cairo CW, Pshezhetsky AV.

PLoS One. 2014 Sep 15;9(9):e106320. doi: 10.1371/journal.pone.0106320. eCollection 2014.


Identification of Selective Nanomolar Inhibitors of the Human Neuraminidase, NEU4.

Albohy A, Zhang Y, Smutova V, Pshezhetsky AV, Cairo CW.

ACS Med Chem Lett. 2013 May 7;4(6):532-7. doi: 10.1021/ml400080t. eCollection 2013 Jun 13.


Serine carboxypeptidase SCPEP1 and Cathepsin A play complementary roles in regulation of vasoconstriction via inactivation of endothelin-1.

Pan X, Grigoryeva L, Seyrantepe V, Peng J, Kollmann K, Tremblay J, Lavoie JL, Hinek A, Lübke T, Pshezhetsky AV.

PLoS Genet. 2014 Feb 27;10(2):e1004146. doi: 10.1371/journal.pgen.1004146. eCollection 2014 Feb.


Elastin-derived peptides potentiate atherosclerosis through the immune Neu1-PI3Kγ pathway.

Gayral S, Garnotel R, Castaing-Berthou A, Blaise S, Fougerat A, Berge E, Montheil A, Malet N, Wymann MP, Maurice P, Debelle L, Martiny L, Martinez LO, Pshezhetsky AV, Duca L, Laffargue M.

Cardiovasc Res. 2014 Apr 1;102(1):118-27. doi: 10.1093/cvr/cvt336. Epub 2013 Dec 19.


Desialylation of surface receptors as a new dimension in cell signaling.

Pshezhetsky AV, Ashmarina LI.

Biochemistry (Mosc). 2013 Jul;78(7):736-45. doi: 10.1134/S0006297913070067. Review.


Identification of selective inhibitors for human neuraminidase isoenzymes using C4,C7-modified 2-deoxy-2,3-didehydro-N-acetylneuraminic acid (DANA) analogues.

Zhang Y, Albohy A, Zou Y, Smutova V, Pshezhetsky AV, Cairo CW.

J Med Chem. 2013 Apr 11;56(7):2948-58. doi: 10.1021/jm301892f. Epub 2013 Mar 26.


Positive regulation of insulin signaling by neuraminidase 1.

Dridi L, Seyrantepe V, Fougerat A, Pan X, Bonneil E, Thibault P, Moreau A, Mitchell GA, Heveker N, Cairo CW, Issad T, Hinek A, Pshezhetsky AV.

Diabetes. 2013 Jul;62(7):2338-46. doi: 10.2337/db12-1825. Epub 2013 Mar 21.


Metabolism of vertebrate amino sugars with N-glycolyl groups: resistance of α2-8-linked N-glycolylneuraminic acid to enzymatic cleavage.

Davies LR, Pearce OM, Tessier MB, Assar S, Smutova V, Pajunen M, Sumida M, Sato C, Kitajima K, Finne J, Gagneux P, Pshezhetsky A, Woods R, Varki A.

J Biol Chem. 2012 Aug 17;287(34):28917-31. doi: 10.1074/jbc.M112.365056. Epub 2012 Jun 12.


Caspase activation regulates the extracellular export of autophagic vacuoles.

Sirois I, Groleau J, Pallet N, Brassard N, Hamelin K, Londono I, Pshezhetsky AV, Bendayan M, Hébert MJ.

Autophagy. 2012 Jun;8(6):927-37. doi: 10.4161/auto.19768. Epub 2012 Jun 1.


Hyaluronidase 1 and β-hexosaminidase have redundant functions in hyaluronan and chondroitin sulfate degradation.

Gushulak L, Hemming R, Martin D, Seyrantepe V, Pshezhetsky A, Triggs-Raine B.

J Biol Chem. 2012 May 11;287(20):16689-97. doi: 10.1074/jbc.M112.350447. Epub 2012 Mar 26.


Global analysis of protein phosphorylation networks in insulin signaling by sequential enrichment of phosphoproteins and phosphopeptides.

Fedjaev M, Parmar A, Xu Y, Vyetrogon K, Difalco MR, Ashmarina M, Nifant'ev I, Posner BI, Pshezhetsky AV.

Mol Biosyst. 2012 Apr;8(5):1461-71. doi: 10.1039/c2mb05440j. Epub 2012 Feb 23.


Spectral optical coherence tomography in a patient with type I sialidosis.

Michalewska Z, Gajos A, Michalewski J, Nawrocki J, Pshezhetsky AV, Bogucki A.

Med Sci Monit. 2011 Oct;17(10):CS129-31.


Where catabolism meets signalling: neuraminidase 1 as a modulator of cell receptors.

Pshezhetsky AV, Hinek A.

Glycoconj J. 2011 Oct;28(7):441-52. doi: 10.1007/s10719-011-9350-5. Epub 2011 Sep 20. Review.


Caspase-3-dependent export of TCTP: a novel pathway for antiapoptotic intercellular communication.

Sirois I, Raymond MA, Brassard N, Cailhier JF, Fedjaev M, Hamelin K, Londono I, Bendayan M, Pshezhetsky AV, Hébert MJ.

Cell Death Differ. 2011 Mar;18(3):549-62. doi: 10.1038/cdd.2010.126. Epub 2010 Oct 22.


Mice doubly-deficient in lysosomal hexosaminidase A and neuraminidase 4 show epileptic crises and rapid neuronal loss.

Seyrantepe V, Lema P, Caqueret A, Dridi L, Bel Hadj S, Carpentier S, Boucher F, Levade T, Carmant L, Gravel RA, Hamel E, Vachon P, Di Cristo G, Michaud JL, Morales CR, Pshezhetsky AV.

PLoS Genet. 2010 Sep 16;6(9):e1001118. doi: 10.1371/journal.pgen.1001118.


Analysis of the biogenesis of heparan sulfate acetyl-CoA:alpha-glucosaminide N-acetyltransferase provides insights into the mechanism underlying its complete deficiency in mucopolysaccharidosis IIIC.

Durand S, Feldhammer M, Bonneil E, Thibault P, Pshezhetsky AV.

J Biol Chem. 2010 Oct 8;285(41):31233-42. doi: 10.1074/jbc.M110.141150. Epub 2010 Jul 22.


Epidermal growth factor and perlecan fragments produced by apoptotic endothelial cells co-ordinately activate ERK1/2-dependent antiapoptotic pathways in mesenchymal stem cells.

Soulez M, Sirois I, Brassard N, Raymond MA, Nicodème F, Noiseux N, Durocher Y, Pshezhetsky AV, Hébert MJ.

Stem Cells. 2010 Apr;28(4):810-20. doi: 10.1002/stem.403.


Desialylation of insulin receptors and IGF-1 receptors by neuraminidase-1 controls the net proliferative response of L6 myoblasts to insulin.

Arabkhari M, Bunda S, Wang Y, Wang A, Pshezhetsky AV, Hinek A.

Glycobiology. 2010 May;20(5):603-16. doi: 10.1093/glycob/cwq010. Epub 2010 Jan 24.


The stoichiometry of protein phosphorylation in adipocyte lipid droplets: analysis by N-terminal isotope tagging and enzymatic dephosphorylation.

Kanshin E, Wang S, Ashmarina L, Fedjaev M, Nifant'ev I, Mitchell GA, Pshezhetsky AV.

Proteomics. 2009 Nov;9(22):5067-77. doi: 10.1002/pmic.200800861.


Regulation of phagocytosis in macrophages by neuraminidase 1.

Seyrantepe V, Iannello A, Liang F, Kanshin E, Jayanth P, Samarani S, Szewczuk MR, Ahmad A, Pshezhetsky AV.

J Biol Chem. 2010 Jan 1;285(1):206-15. doi: 10.1074/jbc.M109.055475. Epub 2009 Nov 4.


Protein misfolding as an underlying molecular defect in mucopolysaccharidosis III type C.

Feldhammer M, Durand S, Pshezhetsky AV.

PLoS One. 2009 Oct 13;4(10):e7434. doi: 10.1371/journal.pone.0007434.


Neu1 desialylation of sialyl alpha-2,3-linked beta-galactosyl residues of TOLL-like receptor 4 is essential for receptor activation and cellular signaling.

Amith SR, Jayanth P, Franchuk S, Finlay T, Seyrantepe V, Beyaert R, Pshezhetsky AV, Szewczuk MR.

Cell Signal. 2010 Feb;22(2):314-24. doi: 10.1016/j.cellsig.2009.09.038. Epub 2009 Sep 29.


Caspase-3-mediated secretion of connective tissue growth factor by apoptotic endothelial cells promotes fibrosis.

Laplante P, Sirois I, Raymond MA, Kokta V, Béliveau A, Prat A, Pshezhetsky AV, Hébert MJ.

Cell Death Differ. 2010 Feb;17(2):291-303. doi: 10.1038/cdd.2009.124. Epub 2009 Sep 4.


Sanfilippo syndrome type C: mutation spectrum in the heparan sulfate acetyl-CoA: alpha-glucosaminide N-acetyltransferase (HGSNAT) gene.

Feldhammer M, Durand S, Mrázová L, Boucher RM, Laframboise R, Steinfeld R, Wraith JE, Michelakakis H, van Diggelen OP, Hrebícek M, Kmoch S, Pshezhetsky AV.

Hum Mutat. 2009 Jun;30(6):918-25. doi: 10.1002/humu.20986. Review.


Serine carboxypeptidases in regulation of vasoconstriction and elastogenesis.

Pshezhetsky AV, Hinek A.

Trends Cardiovasc Med. 2009 Jan;19(1):11-7. doi: 10.1016/j.tcm.2009.03.002. Review.


Dependence of pathogen molecule-induced toll-like receptor activation and cell function on Neu1 sialidase.

Amith SR, Jayanth P, Franchuk S, Siddiqui S, Seyrantepe V, Gee K, Basta S, Beyaert R, Pshezhetsky AV, Szewczuk MR.

Glycoconj J. 2009 Dec;26(9):1197-212. doi: 10.1007/s10719-009-9239-8.


Caspase-3 activation triggers extracellular cathepsin L release and endorepellin proteolysis.

Cailhier JF, Sirois I, Laplante P, Lepage S, Raymond MA, Brassard N, Prat A, Iozzo RV, Pshezhetsky AV, Hébert MJ.

J Biol Chem. 2008 Oct 3;283(40):27220-9. doi: 10.1074/jbc.M801164200. Epub 2008 Jul 24.


Development of a novel noncompetitive antagonist of IL-1 receptor.

Quiniou C, Sapieha P, Lahaie I, Hou X, Brault S, Beauchamp M, Leduc M, Rihakova L, Joyal JS, Nadeau S, Heveker N, Lubell W, Sennlaub F, Gobeil F Jr, Miller G, Pshezhetsky AV, Chemtob S.

J Immunol. 2008 May 15;180(10):6977-87.


Enzymatic activity of lysosomal carboxypeptidase (cathepsin) A is required for proper elastic fiber formation and inactivation of endothelin-1.

Seyrantepe V, Hinek A, Peng J, Fedjaev M, Ernest S, Kadota Y, Canuel M, Itoh K, Morales CR, Lavoie J, Tremblay J, Pshezhetsky AV.

Circulation. 2008 Apr 15;117(15):1973-81. doi: 10.1161/CIRCULATIONAHA.107.733212. Epub 2008 Apr 7.


Mice deficient in Neu4 sialidase exhibit abnormal ganglioside catabolism and lysosomal storage.

Seyrantepe V, Canuel M, Carpentier S, Landry K, Durand S, Liang F, Zeng J, Caqueret A, Gravel RA, Marchesini S, Zwingmann C, Michaud J, Morales CR, Levade T, Pshezhetsky AV.

Hum Mol Genet. 2008 Jun 1;17(11):1556-68. doi: 10.1093/hmg/ddn043. Epub 2008 Feb 11.


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