{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T01:45:29Z","timestamp":1791164729347,"version":"4.1.0"},"reference-count":45,"publisher":"Rockefeller University Press","issue":"3","content-domain":{"domain":["rupress.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2001,2,5]]},"abstract":"<jats:p>The Rieger syndrome is an autosomal dominant disease characterized by ocular, craniofacial, and umbilical defects. Patients have mutations in PITX2, a paired-bicoid homeobox gene, also involved in left\/right polarity determination. In this study we have identified a family of genes for enzymes responsible for hydroxylizing lysines in collagens as one group of likely cognate targets of PITX2 transcriptional regulation. The mouse procollagen lysyl hydroxylase (Plod)-2 gene was enriched for by chromatin precipitation using a PITX2\/Pitx2-specific antibody. Plod-2, as well as the human PLOD-1 promoters, contains multiple bicoid (PITX2) binding elements. We show these elements to bind PITX2 specifically in vitro. The PLOD-1 promoter induces the expression of a luciferase reporter gene in the presence of PITX2 in cotransfection experiments. The Rieger syndrome causing PITX2 mutant T68P fails to induce PLOD-1\u2013luciferase. Mutations and rearrangements in PLOD-1 are known to be prevalent in patients with Ehlers-Danlos syndrome, kyphoscoliosis type (type VI [EDVI]). Several of the same organ systems are involved in Rieger syndrome and EDVI.<\/jats:p>","DOI":"10.1083\/jcb.152.3.545","type":"journal-article","created":{"date-parts":[[2002,7,26]],"date-time":"2002-07-26T12:46:23Z","timestamp":1027687583000},"page":"545-552","update-policy":"https:\/\/doi.org\/10.1083\/jcb.crossmarkpolicy","source":"Crossref","is-referenced-by-count":64,"title":["Pitx2 Regulates Procollagen Lysyl Hydroxylase (Plod) Gene Expression"],"prefix":"10.1083","volume":"152","author":[{"given":"Tord A.","family":"Hjalt","sequence":"first","affiliation":[{"name":"aDepartment of Pediatrics, University of Iowa, Iowa City, Iowa 52242"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brad A.","family":"Amendt","sequence":"additional","affiliation":[{"name":"bDepartment of Biological Science, University of Tulsa, Tulsa, Oklahoma 74104"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeffrey C.","family":"Murray","sequence":"additional","affiliation":[{"name":"aDepartment of Pediatrics, University of Iowa, Iowa City, Iowa 52242"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"291","published-online":{"date-parts":[[2001,2,5]]},"reference":[{"key":"2023072211225682700_Amendtetal1998","doi-asserted-by":"crossref","first-page":"20066","DOI":"10.1074\/jbc.273.32.20066","article-title":"The molecular basis of Rieger syndrome","volume":"273","author":"Amendt","year":"1998","journal-title":"J. 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