J. F. O'brien, M. Cantz, and J. Spranger, Maroteaux-Lamy disease (mucopolysaccharidosis VI), subtype A: Deficiency of a N-acetylgalactosamine-4-sulfatase, Biochem Biophys Res Commun, vol.60, pp.1170-1177, 1974.

A. L. Fluharty, R. L. Stevens, D. L. Sanders, and H. Kihara, Arylsulfatase B deficiency in Maroteaux-Lamy syndrome cultured fibroblasts, Biochem Biophys Res Commun, vol.59, issue.74, pp.455-461, 1974.

E. F. Neufeld and J. Muenzer, The mucopolysaccharidoses. The Metabolic and Molecular Bases of Inherited Disease, pp.3421-3452, 2001.

V. Valayannopoulos, H. Nicely, P. Harmatz, S. Turbeville, and V. I. Mucopolysaccharidosis, Orphanet J Rare Dis, vol.5, p.20385007, 2010.

P. Harmatz and R. Shediac, Mucopolysaccharidosis VI: pathophysiology, diagnosis and treatment, Front Biosci, vol.22, pp.385-406, 2017.

L. A. Clarke, Pathogenesis of skeletal and connective tissue involvement in the mucopolysaccharidoses. Glycosaminoglycan storage is merely the instigator, Rheumatology (Oxford), vol.50, issue.5, pp.13-21, 2011.

L. A. Clarke and C. Hollak, The clinical spectrum and pathophysiology of skeletal complications in lysosomal storage disorders, Best practice & Research clinical endocrinology & Metabolism, vol.29, pp.219-235, 2015.

E. A. Braunlin, P. R. Harmatz, M. Scarpa, B. Furlanetto, C. Kampmann et al., Cardiac disease in patients with mucopolysaccharidosis. Presentation, diagnosis and management, J Inherit Metab Dis, vol.34, pp.1183-1197, 2011.

A. Golda, A. Jurecka, and A. Tylki-szymanska, Cardiovascular manifestations of mucopolysaccharidosis type VI (Maroteaux-Lamy syndrome), Int J Cardiol, vol.158, pp.6-11, 2012.

L. V. Fong, S. Menahem, J. E. Wraith, and C. W. Chow, Endocardial fibroelastosis in mucopolysaccharidosis type VI, Clin Cardiol, vol.10, pp.362-364, 1987.

J. L. Ashworth, S. Biswas, E. Wraith, and I. C. Lloyd, The ocular features of the mucopolysaccharidoses, Eye (Lond), vol.20, pp.553-563, 2006.

L. B. Cantor, J. A. Disseler, and F. M. Wilson, Glaucoma in the Maroteaux-Lamy syndrome, Am J Ophthalmol, vol.108, pp.426-430, 1989.

R. Giugliani, A. Federhen, F. Vairo, C. Vanzella, G. Pasqualim et al., Emerging drugs for the treatment of mucopolysaccharidoses, Expert Opin Emerg Drugs, vol.21, pp.9-26, 2016.

K. Sawamoto, M. Stapleton, C. J. Alméciga-díaz, A. J. Espejo-mojica, J. C. Losada et al., Therapeutic Options for Mucopolysaccharidoses: Current and Emerging Treatments, Drugs, vol.79, p.31209777, 2019.

P. Harmatz, C. B. Whitley, L. Waber, R. Pais, R. Steiner et al., Enzyme replacement therapy in mucopolysaccharidosis VI (Maroteaux-Lamy syndrome), J Pediatr, vol.144, pp.574-580, 2004.

M. J. Brunelli, Á. Atallah, and E. Da-silva, Enzyme replacement therapy with galsulfase for mucopolysaccharidosis type VI, Cochrane Database Syst Rev, vol.3, 2016.

C. R. Fenzl, K. Teramoto, and M. Moshirfar, Ocular manifestations and management recommendations of lysosomal storage disorders I: mucopolysaccharidoses, Clin Ophthalmol, vol.9, pp.1633-1644, 2015.

J. R. Toomey, M. A. Abboud, R. E. Valocik, P. F. Koster, C. L. Burns-kurtis et al., A comparison of the beta-D-xyloside, odiparcil, to warfarin in a rat model of venous thrombosis, J Thromb Haemost, vol.4, p.16961606, 2006.

L. Galligani, J. Hopwood, N. B. Schwartz, and A. Dorfman, Stimulation of synthesis of free chondroitin sulfate chains by beta-D-xylosides in cultured cells, J Biol Chem, vol.250, pp.5400-5406, 1975.

N. B. Schwartz, Effect of beta-xylosides on synthesis of chondroitin sulfate proteoglycan, chondroitin sulfate chains, and core protein, J Biol Chem, vol.252, pp.6316-6321, 1977.

F. N. Lugemwa, A. K. Sarkar, and J. D. Esko, Unusual beta-D-xylosides that prime glycosaminoglycans in animal cells, J Biol Chem, vol.271, pp.19159-19165, 1996.

J. S. Chua and B. Kuberan, Synthetic Xylosides. Probing the Glycosaminoglycan Biosynthetic Machinery for Biomedical Applications, Acc Chem Res, vol.50, pp.2693-2705, 2017.

K. Prydz and K. T. Dalen, Synthesis and sorting of proteoglycans, J Cell Sci, vol.113, issue.2, pp.193-205, 2000.

N. B. Schwartz, L. Rodén, and A. Dorfman, Biosynthesis of chondroitin sulfate: interaction between xylosyltransferase and galactosyltransferase, Biochem Biophys Res Commun, vol.56, pp.717-724, 1974.

P. J. Masson, D. Coup, J. Millet, and N. L. Brown, The effect of the beta-D-xyloside naroparcil on circulating plasma glycosaminoglycans. An explanation for its known antithrombotic activity in the rabbit, J Biol Chem, vol.270, pp.2662-2668, 1995.

, Transforming Growth Factor-?1-Induced Fibrotic Phenotype in Lung Fibroblasts, PLoS ONE, vol.11, p.146499, 2016.

M. M. Curtain and L. R. Donahue, A mutation in the Arsb gene; a mouse model that resembles Maroteaux-Lamy syndrome, 2009.

S. Pohl, A. Angermann, A. Jeschke, G. Hendrickx, T. A. Yorgan et al., The Lysosomal Protein Arylsulfatase B Is a Key Enzyme Involved in Skeletal Turnover, J Bone Miner Res, vol.33, pp.2186-2201, 2018.

V. J. Coulson-thomas and T. F. Gesteira, Dimethylmethylene Blue Assay (DMMB), vol.4, p.1236, 2014.

Z. Avnur and B. Geiger, Immunocytochemical localization of native chondroitin-sulfate in tissues and cultured cells using specific monoclonal antibody, Cell, vol.38, issue.84, p.6435883, 1984.

M. Geetha-habib, S. C. Campbell, and N. B. Schwartz, Subcellular localization of the synthesis and glycosylation of chondroitin sulfate proteoglycan core protein, J Biol Chem, vol.259, pp.7300-7310, 1984.

P. Stanley, Golgi glycosylation, Cold Spring Harb Perspect Biol, 2011.

T. Mikami and H. Kitagawa, Biosynthesis and function of chondroitin sulfate, Biochim Biophys Acta, vol.1830, pp.4719-4733, 2013.

M. Evers, P. Saftig, P. Schmidt, A. Hafner, D. B. Mcloghlin et al., Targeted disruption of the arylsulfatase B gene results in mice resembling the phenotype of mucopolysaccharidosis VI, Proc Natl Acad Sci U S A, vol.93, pp.8214-8219, 1996.

O. F. Strauch, J. Stypmann, T. Reinheckel, E. Martinez, W. Haverkamp et al., Cardiac and ocular pathologies in a mouse model of mucopolysaccharidosis type VI, Pediatr Res, vol.54, pp.701-708, 2003.

J. D. Nuttall, L. K. Brumfield, N. L. Fazzalari, J. J. Hopwood, and S. Byers, Histomorphometric analysis of the tibial growth plate in a feline model of mucopolysaccharidosis type VI, Calcif Tissue Int, vol.65, pp.47-52, 1999.

J. Alroy, G. O. Freden, V. Goyal, S. S. Raghavan, and K. L. Schunk, Morphology of leukocytes from cats affected with alpha-mannosidosis and mucopolysaccharidosis VI (MPS VI), Vet Pathol, vol.26, pp.294-302, 1989.

G. Anderson, V. V. Smith, M. Malone, and N. J. Sebire, Blood film examination for vacuolated lymphocytes in the diagnosis of metabolic disorders; retrospective experience of more than 2,500 cases from a single centre, J Clin Pathol, vol.58, pp.1305-1310, 2005.

P. Masson, J. Theveniaux, D. Coup, T. Gré-goire, M. Vaillot et al., Further studies on the mechanism for the antithrombotic effects of naroparcil, an orally active thioxyloside compound, Thromb Haemost, vol.81, pp.945-950, 1999.

A. L. Myers, V. V. Upreti, M. Khurana, and N. D. Eddington, Characterization of total plasma glycosaminoglycan levels in healthy volunteers following oral administration of a novel antithrombotic odiparcil with aspirin or enoxaparin, J Clin Pharmacol, vol.48, pp.1158-1170, 2008.

K. Mani, M. Belting, U. Ellervik, N. Falk, G. Svensson et al., Tumor attenuation by 2(6-hydroxynaphthyl)-beta-D-xylopyranoside requires priming of heparan sulfate and nuclear targeting of the products, Glycobiology, vol.14, pp.387-397, 2004.

A. Persson, E. Tykesson, G. Westergren-thorsson, . Malmströ-m-a, U. Ellervik et al., Xyloside-primed Chondroitin Sulfate/Dermatan Sulfate from Breast Carcinoma Cells with a Defined Disaccharide Composition Has Cytotoxic Effects in Vitro, J Biol Chem, vol.291, pp.14871-14882, 2016.

J. S. Chua, V. M. Tran, M. Kalita, M. V. Quintero, O. Antelope et al., A glycan-based approach to therapeutic angiogenesis, PLoS ONE, vol.12, p.182301, 2017.

L. C. Smith-thomas, J. Stevens, J. Fok-seang, A. Faissner, J. H. Rogers et al., Increased axon regeneration in astrocytes grown in the presence of proteoglycan synthesis inhibitors, J Cell Sci, vol.108, pp.1307-1315, 1995.

J. Muto, N. N. Naidu, K. Yamasaki, N. Pineau, L. Breton et al., Exogenous addition of a C-xylopyranoside derivative stimulates keratinocyte dermatan sulfate synthesis and promotes migration, PLoS ONE, vol.6, 2011.

C. Chuang, H. Lin, T. Wang, C. Tsai, H. Liu et al., A modified liquid chromatography/tandem mass spectrometry method for predominant disaccharide units of urinary glycosaminoglycans in patients with mucopolysaccharidoses, Orphanet J Rare Dis, vol.9, p.25178307, 2014.

H. Zhang, T. Wood, S. P. Young, and D. S. Millington, A straightforward, quantitative ultra-performance liquid chromatography-tandem mass spectrometric method for heparan sulfate, dermatan sulfate and chondroitin sulfate in urine: an improved clinical screening test for the mucopolysaccharidoses, Mol Genet Metab, vol.114, pp.123-128, 2015.

J. Alroy and J. A. Lyons, Lysosomal Storage Diseases, Journal of Inborn Errors of Metabolism and Screening, vol.2, pp.1-20, 2014.

G. Baldo, F. Poswar, A. Federhen, C. Bittar, G. R. Bender et al., Enzyme Replacement Therapy With Elosulfase Alfa Decreases Storage of Glycosaminoglycan in White Blood Cells of Patients With Morquio A Syndrome, Journal of Inborn Errors of Metabolism and Screening, vol.3, pp.1-3, 2015.

D. A. Brooks, B. M. King, A. C. Crawley, S. Byers, and J. J. Hopwood, Enzyme replacement therapy in Mucopolysaccharidosis VI: evidence for immune responses and altered efficacy of treatment in animal models, Biochim Biophys Acta, vol.1361, pp.36-43, 1997.

H. Debiec, V. Valayannopoulos, O. Boyer, N. El-l-h, P. Callard et al., Allo-immune membranous nephropathy and recombinant aryl sulfatase replacement therapy: a need for tolerance induction therapy, J Am Soc Nephrol, vol.25, pp.675-680, 2014.
URL : https://hal.archives-ouvertes.fr/inserm-02191546

A. C. Crawley, D. A. Brooks, V. J. Muller, B. A. Petersen, E. L. Isaac et al., Enzyme replacement therapy in a feline model of Maroteaux-Lamy syndrome, J Clin Invest, vol.97, pp.1864-1873, 1996.

G. Hendrickx, T. Danyukova, A. Baranowsky, T. Rolvien, A. Angermann et al., Enzyme replacement therapy in mice lacking arylsulfatase B targets bone remodeling cells, but not chondrocytes, Hum Mol Genet, 2020.

E. D. Kakkis, E. Schuchman, X. He, Q. Wan, S. Kania et al., Enzyme replacement therapy in feline mucopolysaccharidosis I, Mol Genet Metab, vol.72, pp.199-208, 2001.

P. S. Kishnani, P. I. Dickson, L. Muldowney, J. J. Lee, A. Rosenberg et al., Immune response to enzyme replacement therapies in lysosomal storage diseases and the role of immune tolerance induction, Mol Genet Metab, vol.117, pp.66-83, 2016.

C. M. Simonaro, M. Angelo, M. E. Haskins, and E. H. Schuchman, Joint and bone disease in mucopolysaccharidoses VI and VII: identification of new therapeutic targets and biomarkers using animal models, Pediatr Res, vol.57, pp.701-707, 2005.

S. Wilson, S. Hashamiyan, L. Clarke, P. Saftig, J. Mort et al., Glycosaminoglycan-mediated loss of cathepsin K collagenolytic activity in MPS I contributes to osteoclast and growth plate abnormalities, Am J Pathol, vol.175, pp.2053-2062, 2009.

G. Baldo, A. Tavares, E. Gonzalez, E. Poletto, F. Q. Mayer et al., Progressive heart disease in mucopolysaccharidosis type I mice may be mediated by increased cathepsin B activity, Cardiovasc Pathol, vol.27, pp.45-50, 2017.

C. M. Simonaro, Y. Ge, E. Eliyahu, X. He, K. J. Jepsen et al., Involvement of the Toll-like receptor 4 pathway and use of TNF-alpha antagonists for treatment of the mucopolysaccharidoses, Proc Natl Acad Sci U S A, vol.107, pp.222-227, 2010.

E. Eliyahu, T. Wolfson, Y. Ge, K. J. Jepsen, E. H. Schuchman et al., Anti-TNF-alpha therapy enhances the effects of enzyme replacement therapy in rats with mucopolysaccharidosis type VI, PLoS ONE, vol.6, 2011.

T. Alliston, Chondroitin sulfate and growth factor signaling in the skeleton. Possible links to MPS VI, J Pediatr Rehabil Med, vol.3, pp.129-138, 2010.

E. Vassal-stermann, A. Duranton, A. F. Black, G. Azadiguian, J. Demaude et al., A New C-Xyloside induces modifications of GAG expression, structure and functional properties, PLoS ONE, vol.7, 2012.
URL : https://hal.archives-ouvertes.fr/hal-00871190

A. Persson, U. Ellervik, and K. Mani, Fine-tuning the structure of glycosaminoglycans in living cells using xylosides, Glycobiology, vol.28, pp.499-511, 2018.

D. R. Randall, K. E. Colobong, H. Hemmelgarn, G. B. Sinclair, H. E. Thomas et al., Heparin cofactor IIthrombin complex: a biomarker of MPS disease, Mol Genet Metab, vol.94, pp.456-461, 2008.

A. Persson, G. Toledo, A. Vorontsov, E. Nasir, W. Willén et al., LC-MS/MS characterization of xyloside-primed glycosaminoglycans with cytotoxic properties reveals structural diversity and novel glycan modifications, J Biol Chem, 2018.