{"id":103,"date":"2022-09-26T19:47:48","date_gmt":"2022-09-26T19:47:48","guid":{"rendered":"https:\/\/naglazymehcp-dev-001.azurewebsites.net\/en-us\/naglazyme\/?page_id=103"},"modified":"2023-05-24T11:03:27","modified_gmt":"2023-05-24T11:03:27","slug":"biochemical-basis-of-mps-vi","status":"publish","type":"page","link":"https:\/\/hcp.biomarin.com\/en-us\/naglazyme\/biochemical-basis-of-mps-vi\/","title":{"rendered":"The Biochemical Basis of MPS VI"},"content":{"rendered":"<div id=\"acf-block-6421cb7d5fc07\" class=\"block wrapped-content block-tight-top block-tight-bottom\">\n    <div class=\"wrapper\">\n        <div class=\"inner-wrapper\">\n                \n<div id=\"acf-block-6421cb7d5fe4b\" class=\"block-wysiwyg\">\n            <h1>The Biochemical Basis of MPS VI<\/h1>\n<p>MPS VI is caused by an inherited deficiency in the enzyme N\u2010acetylgalactosamine\u20104\u2010sulfatase (arylsulfatase B or ASB).<sup>1 <\/sup><\/p>\n<p>ASB regulates the catabolism of a glycosaminoglycan (GAG) called dermatan sulfate and is one of 5 enzymes necessary for stepwise degradation of dermatan sulfate.<sup>1<\/sup><\/p>\n<ul>\n<li>GAGs have important roles in cellular structure and cell interactions, especially in connective tissue and its extracellular matrix<sup>2<\/sup><\/li>\n<\/ul>\n<h2>GAG accumulation in MPS VI<\/h2>\n<p>In the absence of ASB, partially degraded GAGs accumulate in cell lysosomes.<sup>3<\/sup><\/p>\n    <\/div>\n        <\/div>\n    <\/div>\n<\/div>\n\n<div id=\"acf-block-6421cb7d5fe84\" class=\"block split-content equal-bias bottom-border block-tight-top block-tight-bottom\" data-muted-autoplay=\"false\">\n\t<div class=\"wrapper\">\n\t\t<div class=\"inner-wrapper\">\n\t\t                                                                <div class=\"content-block first-block\">\n                                                                                                                                                                                                                                                                                    \n                                    \n                                                                                                                \n                                    \n                                    <figure>\n                                        <div class=\"image image-rounded\">\n                                                                                            <img decoding=\"async\" class=\"\" src=\"https:\/\/hcp.biomarin.com\/en-us\/naglazyme\/wp-content\/uploads\/sites\/2\/2022\/10\/1-1a-HealthyCell.png?v=0.33\" alt=\"healthy cell\" \/>                                                                                    <\/div>\n                                                                                    <figcaption><p>Healthy cell<\/p>\n<\/figcaption>\n                                                                            <\/figure>\n                                                                                    <\/div>\n                    \n                                            <div class=\"content-block second-block\">\n                                                                                                                                                                                                                                                                                    \n                                    \n                                                                                                                \n                                    \n                                    <figure>\n                                        <div class=\"image image-rounded\">\n                                                                                            <img decoding=\"async\" class=\"\" src=\"https:\/\/hcp.biomarin.com\/en-us\/naglazyme\/wp-content\/uploads\/sites\/2\/2022\/10\/1-1b-MPS-Cell-Excess-Gag.png?v=0.33\" alt=\"MPS cell excess Gag\" \/>                                                                                    <\/div>\n                                                                                    <figcaption><p>Lysosomal storage disorder cell full of GAGs<\/p>\n<\/figcaption>\n                                                                            <\/figure>\n                                                                                    <\/div>\n                                                \t\t<\/div>\n\t<\/div>\n<\/div>\n\n<div id=\"acf-block-6421cb7d5fe9c\" class=\"block wrapped-content block-tight-top block-tight-bottom\">\n    <div class=\"wrapper\">\n        <div class=\"inner-wrapper\">\n                \n<div id=\"acf-block-6421cb7d5fec0\" class=\"block-wysiwyg\">\n            <ul>\n<li>Replete with excess GAGs, lysosomes crowd the nucleus and other critical organelles, engorging the cell<\/li>\n<li>This leads to cellular malfunction, multisystemic organ damage, and an extensive range of symptoms<sup>1,4<\/sup><\/li>\n<\/ul>\n<p>GAGs are a major component of connective tissues and organs throughout the body, including vessel linings, bone marrow, heart, lung, liver, and spleen stroma. As a result, MPS VI affects many organ systems.<sup>4,5<\/sup><\/p>\n<p><a class=\"nxt_page\" href=\"https:\/\/hcp.biomarin.com\/en-us\/naglazyme\/clinical-manifestations-of-mps-vi\/\">Learn more about the different ways MPS VI affects organ systems \u00bb<\/a><\/p>\n    <\/div>\n        <\/div>\n    <\/div>\n<\/div>\n\n<div id=\"acf-block-6421cb7d60152\" class=\"block references block-tight-top block-tight-bottom\">\n    <div class=\"wrapper\">\n\t\t<div class=\"inner-wrapper\">\n\t\t    \t\t\t    <h4>References:\n<\/h4>\n\t\t\t\t\t\t                <ol>\n                                                                                                                        <li><span>Neufeld EF, Muenzer J. The mucopolysaccharidoses. In: Scriver CR, Beaud<em>et al<\/em>, Sly WS, <em>et al<\/em>, eds. <em>The Metabolic and Molecular Bases of Inherited Disease<\/em>. 8th ed. New York, NY: McGraw\u2010Hill; 2001:3421\u20103452.\n<\/span><\/li>\n                                                                                                                                                <li><span>Aquino R S et al. <em>Academic Press 2010<\/em>; 93: 373-394.\n<\/span><\/li>\n                                                                                                                                                <li><span>Swiedler SJ, Beck M, Bajbouj M, <em>et al<\/em>. Threshold effect of urinary glycosaminoglycans and the walk test as indicators of disease progression in a survey of subjects with mucopolysaccharidosis VI (Maroteaux\u2010Lamy syndrome). <em>Am J Med Genet A<\/em>. 2005;134A(2):144-150.\n<\/span><\/li>\n                                                                                                                                                <li><span>Akyol MU, Alden TD, Amartino H, <em>et al<\/em>. Recommendations for the management of MPS VI: systematic evidence- and consensus-based guidance. <em>Orphanet J Rare Dis.<\/em> 2019;14(1):118.\n<\/span><\/li>\n                                                                                                                                                <li><span>MPS VI https:\/\/medlineplus.gov\/genetics\/condition\/mucopolysaccharidosis-type-vi\/ Accessed May 2023\n<\/span><\/li>\n                                                            <\/ol>\n\t\t\t\t\t<\/div>\n\t<\/div>\n<\/div>","protected":false},"excerpt":{"rendered":"","protected":false},"author":4,"featured_media":0,"parent":0,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","meta":{"_acf_changed":false,"inline_featured_image":false,"footnotes":""},"class_list":["post-103","page","type-page","status-publish","hentry"],"acf":[],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v27.4 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>Naglazyme | HCP Biochemical Basis of MPS VI<\/title>\n<meta name=\"description\" content=\"The biochemical basis of MPS VI. 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