Characterization of expression of the gene for human pterin carbinolamine dehydratase/dimerization cofactor of HNF1.

Pterin carbinolamine dehydratase/dimerization cofactor of HNF1 (PCD/DCoH) is a dual-function protein. In the cytoplasm it acts as a dehydratase in the regeneration of tetrahydrobiopterin, the cofactor for aromatic amino acid hydroxylases. In the nucleus, it functions as a dimerization cofactor of HNF1 and increases the transcriptional activity of HNF1. To deepen our understanding of this protein, we characterized its expression in human tissues and cells. Human PCD/DCoH was present predominantly in liver and kidney, with significant amounts in testis and ovary, trace amounts in lung, and undetectable levels in whole brain, heart, and spleen. It was expressed in all of the cells that were examined. Importantly, it was also present in the nucleus of HeLa cells, which lack HNF1, and in the cytoplasm of fibroblasts that have little or no tetrahydrobiopterin. The expression of human PCD/DCoH in the liver and nonhepatic cells was compared at both the mRNA and protein levels. Although the mRNA level in liver was...