{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2023,10,23]],"date-time":"2023-10-23T11:47:18Z","timestamp":1698061638373},"reference-count":16,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[1984,1,11]],"date-time":"1984-01-11T00:00:00Z","timestamp":442627200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[1984,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Aspartylglycosaminuria (AGU) is a lysosomal storage disorder of glycoprotein degradation characterized by severe mental retardation and connective tissue alterations. We have previously described low collagen production in skin fibroblast cultures from AGU patients. In the present work we showed that the urinary excretion of hydroxyproline (total, non\u2010dialysable and free hydroxyproline as indicators of collagen metabolism) was reduced in young AGU patients in comparison with age\u2010matched controls. In adult patients no significant difference was detected. The results support the view that reduced collagen production is associated with the connective tissue abnormalities in this disorder.<\/jats:p>","DOI":"10.1007\/bf01801767","type":"journal-article","created":{"date-parts":[[2005,6,27]],"date-time":"2005-06-27T23:54:31Z","timestamp":1119916471000},"page":"117-121","source":"Crossref","is-referenced-by-count":4,"title":["Metabolism of collagen in aspartylglycosaminuria: Urinary excretion of hydroxyproline"],"prefix":"10.1002","volume":"7","author":[{"given":"K.","family":"N\u00e4nt\u00f6\u2010Salonen","sequence":"first","affiliation":[{"name":"Department of Medical Chemistry University of Turku  Turku 52 20520 Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S.","family":"Autio","sequence":"additional","affiliation":[{"name":"Rinnekoti Institution for the Mentally Retarded  Espoo 98 02980 Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"E.","family":"H\u00e4r\u00f6","sequence":"additional","affiliation":[{"name":"Tahkokangas Central Institution for the Mentally Retarded  Oulu 25 90250 Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"T.","family":"Kivim\u00e4ki","sequence":"additional","affiliation":[{"name":"Ylinen Central Institution for the Mentally Retarded  Ylinen 34130 Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S. \u2010L.","family":"Koskela","sequence":"additional","affiliation":[{"name":"Kolpene Central Institution for the Mentally Retarded  Rovaniemi 44 96440 Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"V.","family":"N\u00e4nt\u00f6","sequence":"additional","affiliation":[{"name":"Department of Clinical Chemistry University of Turku  Turku 52 20520 Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R.","family":"Penttinen","sequence":"additional","affiliation":[{"name":"Department of Medical Chemistry University of Turku  Turku 52 20520 Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[1984,1,11]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/978-3-642-51582-8_5"},{"key":"e_1_2_1_3_1","first-page":"1","article-title":"Aspartylglycosaminuria. 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Rapola J.andPenttinen R.Abnormal collagen fibrils in aspartylglycosaminuria: altered dermal ultrastructure in a glycoprotein storage disorder.Lab. 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