{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T02:45:12Z","timestamp":1792723512232,"version":"4.5.0"},"reference-count":34,"publisher":"American Physiological Society","issue":"1","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American Journal of Physiology-Heart and Circulatory Physiology"],"published-print":{"date-parts":[[2011,1]]},"abstract":"<jats:p> Cardiac dysfunction is a common cause of death among pediatric patients with mutations in the lysosomal hydrolase \u03b1-l-iduronidase ( IDUA) gene, which causes mucopolysaccharidosis type I (MPS-I). The purpose of this study was to analyze adrenergic regulation of cardiac hemodynamic function in MPS-I. An analysis of murine heart function was performed using conductance micromanometry to assess in vivo cardiac hemodynamics. Although MPS-I ( IDUA<jats:sup> \u2212\/\u2212<\/jats:sup>) mice were able to maintain normal cardiac output and ejection fraction at baseline, this cohort had significantly compromised systolic and diastolic function compared with IDUA<jats:sup> +\/\u2212<\/jats:sup> control mice. During dobutamine infusion MPS-I mice did not significantly increase cardiac output from baseline, indicative of blunted cardiac reserve. Autonomic tone, measured functionally by \u03b2-blockade, indicated that MPS-I mice required catecholaminergic stimulation to maintain baseline hemodynamics. Survival analysis showed mortality only among MPS-I mice. Linear regression analysis revealed that heightened end-systolic volume in the resting heart is significantly correlated with susceptibility to mortality in MPS-I hearts. This study reveals that cardiac remodeling in the pathology of MPS-I involves heightened adrenergic tone at the expense of cardiac reserve with cardiac decompensation predicted on the basis of increased baseline systolic volumes. <\/jats:p>","DOI":"10.1152\/ajpheart.00774.2010","type":"journal-article","created":{"date-parts":[[2010,11,13]],"date-time":"2010-11-13T04:17:44Z","timestamp":1289621864000},"page":"H356-H365","source":"Crossref","is-referenced-by-count":6,"title":["Cardiac disease in mucopolysaccharidosis type I attributed to catecholaminergic and hemodynamic deficiencies"],"prefix":"10.1152","volume":"300","author":[{"given":"Nathan J.","family":"Palpant","sequence":"first","affiliation":[{"name":"Departments of 1Integrative Biology and Physiology and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fikru B.","family":"Bedada","sequence":"additional","affiliation":[{"name":"Departments of 1Integrative Biology and Physiology 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