The paired-class homeobox genes PRX1 and PRX2 are necessary for craniofacial and limb development and are expressed in similar patterns in the cranial mesenchyme, limb buds, axial mesoderm and branchial arches. These proteins exhibit different patterns of expression, however, in heart and brain tissue. Specifically, PRX1 is expressed in the endocardial cusions, semilunar and atrioventricular valves, whereas PRX2 is initially expressed in a diffuse myocardial pattern and is later expressed in the ventricular septum. Furthermore, PRX2 is never expressed in the brain, whereas PRX1 is expressed in the ventral hypothalamus and in the telencephalon. Murine mutants lacking PRX1 function demonstrate skeletal defects in the skull, limbs, and vertebral column. Mice lacking functional PRX2 alone do not demonstrate skeletal abnormalities, however, PRX1/PRX2 double mutants demonstrate novel abnormalities that are not visualized with the PRX1-deficient mice. Transcripts of neither PRX1 nor PRX2 are detected in normal adult rat pulmonary arteries, however vascular disease induces PRX gene expression wherein they co-localize to sites of Tenascin-C expression.View more
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