{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T02:18:23Z","timestamp":1790734703553,"version":"4.1.0"},"reference-count":52,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2004,4,1]],"date-time":"2004-04-01T00:00:00Z","timestamp":1080777600000},"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 Cellular Biochemistry"],"published-print":{"date-parts":[[2004,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>To examine mechanisms by which reduced type V collagen causes weakened connective tissues in the Ehlers\u2013Danlos syndrome (EDS), we examined matrix deposition and collagen fibril morphology in long\u2010term dermal fibroblast cultures. EDS cells with COL5A1 haplo\u2010insufficiency deposited less than one\u2010half of hydroxyproline as collagen compared to control fibroblasts, though total collagen synthesis rates are near\u2010normal because type V collagen represents a small fraction of collagen synthesized. Cells from patients with osteogenesis imperfecta (OI) and haplo\u2010insufficiency for pro\u03b11(I) chains of type I collagen also incorporated about one\u2010half the collagen as controls, but this amount was proportional to their reduced rates of total collagen synthesis. Collagen fibril diameter was inversely proportional to type V\/type I collagen ratios (EDS\u2009&gt;\u2009control\u2009&gt;\u2009OI). However, a reduction of type V collagen, in the EDS derived cells, was associated with the assembly of significantly fewer fibrils compared to control and OI cells. These data indicate that in cell culture, the quantity of collagen fibrils deposited in matrix is highly sensitive to reduction in type V collagen, far out of proportion to type V collagen's contribution to collagen mass. \u00a9 2004 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/jcb.20024","type":"journal-article","created":{"date-parts":[[2004,4,1]],"date-time":"2004-04-01T22:00:47Z","timestamp":1080856847000},"page":"113-124","source":"Crossref","is-referenced-by-count":63,"title":["Reduced type I collagen utilization: A pathogenic mechanism in COL5A1 haplo\u2010insufficient Ehlers\u2013Danlos syndrome"],"prefix":"10.1002","volume":"92","author":[{"given":"Richard J.","family":"Wenstrup","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jane B.","family":"Florer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"William G.","family":"Cole","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marcia C.","family":"Willing","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David E.","family":"Birk","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2004,4]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.3109\/03008208609017469"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1093\/glycob\/cwf065"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1096\/fj.01-0848com"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.79.12.3838"},{"key":"e_1_2_6_6_1","first-page":"189","volume-title":"McKusick's heritable disorders of connective tissue","author":"Beighton P","year":"1992"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-8628(19980428)77:1<31::AID-AJMG8>3.0.CO;2-O"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/0076-6879(82)82074-0"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.99.6.2024"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.106.3.999"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1242\/jcs.95.4.649"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1097-0177(199703)208:3<291::AID-AJA1>3.0.CO;2-D"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(18)89655-7"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M011742200"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/1523-1747.ep12348978"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2230.1997.tb01055.x"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.141.5.1277"},{"key":"e_1_2_6_18_1","doi-asserted-by":"publisher","DOI":"10.1359\/jbmr.2002.17.7.1180"},{"key":"e_1_2_6_19_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.136.3.729"},{"key":"e_1_2_6_20_1","first-page":"547","article-title":"Mutations in the COL5A1 gene are causal in the Ehlers\u2013Danlos syndromes I and II","volume":"60","author":"De Paepe A","year":"1997","journal-title":"Am J Hum Genet"},{"key":"e_1_2_6_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0945-053X(00)00067-6"},{"key":"e_1_2_6_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0945-053X(05)80001-0"},{"key":"e_1_2_6_23_1","doi-asserted-by":"publisher","DOI":"10.1021\/bi00587a003"},{"key":"e_1_2_6_24_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M205398200"},{"key":"e_1_2_6_25_1","doi-asserted-by":"publisher","DOI":"10.1042\/bj3160001"},{"key":"e_1_2_6_26_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.121.5.1181"},{"key":"e_1_2_6_27_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(19)52451-6"},{"key":"e_1_2_6_28_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng850"},{"key":"e_1_2_6_29_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.135.5.1415"},{"key":"e_1_2_6_30_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/7.2.249"},{"key":"e_1_2_6_31_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1432-1033.1994.tb18815.x"},{"key":"e_1_2_6_32_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.33.11.940"},{"key":"e_1_2_6_33_1","doi-asserted-by":"publisher","DOI":"10.1016\/0167-4838(93)90099-D"},{"key":"e_1_2_6_34_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1432-1033.1994.00943.x"},{"key":"e_1_2_6_35_1","first-page":"1","volume-title":"Collagen: Volume II: Biochemistry and biomechanics","author":"Parry DA","year":"1988"},{"key":"e_1_2_6_36_1","doi-asserted-by":"publisher","DOI":"10.1021\/bi00782a009"},{"key":"e_1_2_6_37_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.35.10.846"},{"key":"e_1_2_6_38_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI112012"},{"key":"e_1_2_6_39_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa002939"},{"key":"e_1_2_6_40_1","doi-asserted-by":"publisher","DOI":"10.1086\/302933"},{"key":"e_1_2_6_41_1","doi-asserted-by":"publisher","DOI":"10.1002\/aja.1001750409"},{"key":"e_1_2_6_42_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00206058"},{"key":"e_1_2_6_43_1","first-page":"351","volume-title":"Connective tissue and its heritable disorders","author":"Steinmann B","year":"1993"},{"key":"e_1_2_6_44_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.274.14.9636"},{"key":"e_1_2_6_45_1","doi-asserted-by":"publisher","DOI":"10.1006\/geno.1995.9961"},{"key":"e_1_2_6_46_1","doi-asserted-by":"publisher","DOI":"10.1016\/0305-0491(95)02014-4"},{"key":"e_1_2_6_47_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng0796-361"},{"key":"e_1_2_6_48_1","first-page":"201","article-title":"Abnormal collagen fibril structure in the gravis form (type I) of Ehlers\u2013Danlos syndrome","volume":"40","author":"Vogel A","year":"1979","journal-title":"Lab Invest"},{"key":"e_1_2_6_49_1","doi-asserted-by":"publisher","DOI":"10.1111\/1523-1747.ep12259720"},{"key":"e_1_2_6_50_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/5.11.1733"},{"key":"e_1_2_6_51_1","doi-asserted-by":"publisher","DOI":"10.1086\/302930"},{"key":"e_1_2_6_52_1","first-page":"638","article-title":"Osteogenesis imperfecta type I: Molecular heterogeneity for COL1A1 null alleles of type I collagen","volume":"55","author":"Willing MC","year":"1994","journal-title":"Am J Hum Genet"},{"key":"e_1_2_6_53_1","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/30.3.173"}],"container-title":["Journal of Cellular Biochemistry"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fjcb.20024","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/jcb.20024","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,11,16]],"date-time":"2023-11-16T22:30:35Z","timestamp":1700173835000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/jcb.20024"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2004,4]]},"references-count":52,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2004,5]]}},"alternative-id":["10.1002\/jcb.20024"],"URL":"https:\/\/doi.org\/10.1002\/jcb.20024","archive":["Portico"],"relation":{},"ISSN":["0730-2312","1097-4644"],"issn-type":[{"value":"0730-2312","type":"print"},{"value":"1097-4644","type":"electronic"}],"subject":[],"published":{"date-parts":[[2004,4]]}}}