{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,21]],"date-time":"2025-11-21T05:59:58Z","timestamp":1763704798663,"version":"3.37.3"},"reference-count":43,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2012,11,28]],"date-time":"2012-11-28T00:00:00Z","timestamp":1354060800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"National Institute of Arthritis and Musculoskeletal and Skin Diseases\/National Institutes of Health","award":["5R01 AR051146","1R01 DK087185"],"award-info":[{"award-number":["5R01 AR051146","1R01 DK087185"]}]},{"DOI":"10.13039\/100013927","name":"National MPS Society","doi-asserted-by":"crossref","id":[{"id":"10.13039\/100013927","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Journal Orthopaedic Research"],"published-print":{"date-parts":[[2013,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Mucopolysaccharidosis (MPS) VI is an inherited lysosomal storage disorder resulting from deficiency of <jats:italic>N<\/jats:italic>\u2010acetylgalactosamine\u20104\u2010sulfatase activity and subsequent accumulation of incompletely degraded dermatan sulfate (DS) containing glycosaminoglycans (GAGs). Painful spinal deformities are commonly found in MPS VI patients. We characterized lumbar spine structure, composition, and biomechanics in a naturally occurring rat MPS VI model and evaluated the role of MMP\u201013, ADAMTS\u20105 and TNF\u2010\u03b1 in modulating the observed changes. MPS VI rats had discs with large vacuolated cells and sizable nuclear defects. MPS spine segments also had structural and functional changes suggestive of spinal instability, including decreased nuclear pressurization, increased joint laxity and increased disc height index. These functional changes were at least partly associated with elevated ADAMTS\u20105, MMP\u201013, and TNF\u2010\u03b1. Vertebral and endplate biomechanics were also affected by MPS VI with decreased failure load and stiffness. The discal and vertebral dysfunctions observed in MPS VI rats are likely to be associated with pathological spinal conditions, similar to those that afflict MPS patients. Our findings also suggest more broadly that abnormal accumulation of GAGs and the associated chronic pro\u2010inflammatory and catabolic cascade may also be a source of spinal dysfunction. \u00a9 2012 Orthopaedic Research Society. Published by Wiley Periodicals, Inc. 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