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Published online 5 February 2001. doi:10.1083/jcb.152.3.545
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© The Rockefeller University Press, 0021-9525/2001//545 $5.00
The Journal of Cell Biology, Volume 152, Number 3, , 2001 545-552

Original Article

Pitx2 Regulates Procollagen Lysyl Hydroxylase (Plod) Gene Expression

: Implications for the Pathology of Rieger Syndrome



Tord A. Hjalta, Brad A. Amendtb, and Jeffrey C. Murraya

a Department of Pediatrics, University of Iowa, Iowa City, Iowa 52242
b Department of Biological Science, University of Tulsa, Tulsa, Oklahoma 74104
University of Iowa, Department of Pediatrics, 140 EMRB, Iowa City, IA 52242.(319) 335-6970(319) 335-6897

jeff-murray{at}uiowa.edu

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–luciferase. 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.

Key Words: PITX2 • PLOD • Rieger • Ehlers-Danlos • promoter

© 2001 The Rockefeller University Press

Abbreviations used in this paper: BAC, bacterial artificial chromosome; CMV, cytomegalovirus; EDVI, Ehlers-Danlos syndrome type VI; EMSA, electrophoretic mobility shift assay; PLOD, procollagen lysyl hydroxylase; RT, reverse transcription; TK, thymidine kinase.


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