{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2023,10,24]],"date-time":"2023-10-24T14:21:57Z","timestamp":1698157317988},"reference-count":0,"publisher":"Portland Press Ltd.","issue":"2","content-domain":{"domain":["portlandpress.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[1989,1,15]]},"abstract":"<jats:p>Fibroblasts from two lethal variants of osteogenesis imperfecta were shown to synthesize increased amounts of type IV procollagen. Previous studies established that one of these variants had a non-functional allele for the pro alpha 2 chain of type I procollagen, whereas the other pro alpha 2(I) allele contained a mutation leading to synthesis of shortened pro alpha 2(I) chains. In the two variants, the relative level of mRNA for pro alpha 1(IV) was 31 and 42% of the level of mRNA for pro alpha 1(I) chains. A value of less than 2% was found for a third lethal and four non-lethal variants of osteogenesis imperfecta. Immunofluorescent staining of fibroblasts from the two variants synthesizing increased amounts of type IV procollagen indicated that a homogeneous population of cells synthesized both type IV and type I procollagen. The results suggest that mutations in the type I procollagen genes that result in osteogenesis imperfecta can be associated with increased expression of the genes for type IV procollagen.<\/jats:p>","DOI":"10.1042\/bj2570439","type":"journal-article","created":{"date-parts":[[2015,8,10]],"date-time":"2015-08-10T21:12:59Z","timestamp":1439241179000},"page":"439-445","update-policy":"http:\/\/dx.doi.org\/10.1042\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Increased expression of the gene for the pro<i>\u03b1<\/i> 1(IV) chain of basement-membrane procollagen in cultured skin fibroblasts from two variants of osteogenesis imperfecta"],"prefix":"10.1042","volume":"257","author":[{"given":"T","family":"Pihlajaniemi","sequence":"first","affiliation":[{"name":"Collagen Research Unit, Biocenter and Department of Medical Biochemistry, University of Oulu, Kajaanintie 52 A, 90220 Oulu, Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J","family":"McKeon","sequence":"additional","affiliation":[{"name":"Department of Biochemistry, UMDNJ-Rutgers Medical School, Piscataway, NJ 08854, U.S.A."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S","family":"Gay","sequence":"additional","affiliation":[{"name":"Department of Medicine, University of Alabama, Birmingham, AL 35294, U.S.A."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R","family":"Gay","sequence":"additional","affiliation":[{"name":"Department of Medicine, University of Alabama, Birmingham, AL 35294, U.S.A."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"W J","family":"de Wet","sequence":"additional","affiliation":[{"name":"Department of Biochemistry, University of Potchefstroom, Potchefstroom, South Africa"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J C","family":"Myers","sequence":"additional","affiliation":[{"name":"Departments of Medicine and Human Genetics, and Connective Tissue Research Institute, University of Pennsylvania, Philadelphia, PA 19104, U.S.A."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"D J","family":"Prockop","sequence":"additional","affiliation":[{"name":"Jefferson Institute of Molecular Medicine, Jefferson Medical College, Philadelphia, PA, U.S.A."}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"288","container-title":["Biochemical Journal"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/portlandpress.com\/biochemj\/article-pdf\/257\/2\/439\/594954\/bj2570439.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/portlandpress.com\/biochemj\/article-pdf\/257\/2\/439\/594954\/bj2570439.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,11,25]],"date-time":"2021-11-25T19:24:54Z","timestamp":1637868294000},"score":1,"resource":{"primary":{"URL":"https:\/\/portlandpress.com\/biochemj\/article\/257\/2\/439\/83844\/Increased-expression-of-the-gene-for-the-pro-1-IV"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1989,1,15]]},"references-count":0,"journal-issue":{"issue":"2","published-print":{"date-parts":[[1989,1,15]]}},"URL":"https:\/\/doi.org\/10.1042\/bj2570439","relation":{},"ISSN":["0264-6021","1470-8728"],"issn-type":[{"value":"0264-6021","type":"print"},{"value":"1470-8728","type":"electronic"}],"subject":[],"published":{"date-parts":[[1989,1,15]]}}}