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The Bi-Functional Proteins:
DCoH and DCoHalpha DCoH and DCoHalpha are mammalian bi-functional proteins that act both as enzymes to dehydrate 4a-hydroxy-tetrahydrobiopterin and as coactivators of transcription by complexing with the transcription factor HNF1alpha. Mutations in these proteins have been associated with hyperphenylalaninemia and diabetes, respectively. HNF1alpha has further been implicated in development and carcinogenesis. Our goals are to 1) understand how the two functions of DCoH(alpha) are regulated, 2) determine the molecular mechanism of DCoH(alpha)-dependent coactivation of HNF1alpha-dependent transcription, and 3) ascertain how particular residue differences alter the functions of DCoH and DCoHalpha. Hevel, JM*, Steweart, JA, Gross, KL, & Ayling, JE (2006) Can the DCoHalpha Isozyme Compensate in Patients with 4a-Hydroxy-tetrahydrobiopterin Dehydratase/DCoH Deficiency? Molecular Genetics and Metabolism, 88:38-46 Hevel, J.M*., Pande, P., Viera-Oveson, S., Sudweeks, T., Jaffree, L.S. , Hansen, C.M., & Ayling, J.E. (2008) Determinants of Oligomerization of the Bifunctional Protein DCoHalpha and the Effect on its Transcriptional Coactivator and Enzymatic Activities. Archives in Biochemistry and Biophysics, in press |
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Moonlighting Proteins
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