EC Number | Inhibitors | Comment | Organism | Structure |
---|---|---|---|---|
3.5.4.16 | additional information | asymmetric dimethylarginine, ADMA, decreases GCH1 protein, but not mRNA concentrations, in pulmonary arterial endothelial cells because of the ubiquitination and proteasome-dependent degradation of GCH1. Overexpression of CHIP potentiates, whereas a CHIP U-box domain mutant attenuates, ADMA-induced GCH1 degradation and reductions in cellular BH4 concentrations. L-Arginine acts antagonistic and restores the activities | Ovis aries |
EC Number | Organism | UniProt | Comment | Textmining |
---|---|---|---|---|
3.5.4.16 | Ovis aries | - |
lambs | - |
EC Number | Posttranslational Modification | Comment | Organism |
---|---|---|---|
3.5.4.16 | additional information | ubiquitination and proteasome-dependent degradation of GCH1. The CHIP-ubiquitin pathway modifies GCH1 in a lamb model of pulmonary hypertension secondary to increased pulmonary blood flow | Ovis aries |
EC Number | Source Tissue | Comment | Organism | Textmining |
---|---|---|---|---|
3.5.4.16 | lung | - |
Ovis aries | - |
3.5.4.16 | pulmonary artery endothelial cell | - |
Ovis aries | - |
EC Number | Synonyms | Comment | Organism |
---|---|---|---|
3.5.4.16 | GCH1 | - |
Ovis aries |
3.5.4.16 | guanosine-5'-triphosphate cyclohydrolase 1 | - |
Ovis aries |
EC Number | General Information | Comment | Organism |
---|---|---|---|
3.5.4.16 | metabolism | GCH1 is the rate-limiting enzyme in the generation of BH4 | Ovis aries |
3.5.4.16 | additional information | asymmetric dimethylarginine, ADMA, decreases GCH1 protein, but not mRNA concentrations, in pulmonary arterial endothelial cells because of the ubiquitination and proteasome-dependent degradation of GCH1. Hsp90-GCH1 interactions are reduced, whereas the association of GCH1 with Hsp70 and the C-terminus of Hsp70-interacting protein, i.e. CHIP, increases in the cells. In vivo Hsp90/GCH1 interactions are decreased, whereas GCH1-Hsp70 and GCH1-CHIP interactions and GCH1 ubiquitination are increased. L-Arginine acts antagonistic and restores the activities | Ovis aries |
3.5.4.16 | physiological function | GTP cyclohydrolase I interaction is required for degradation of the C-terminus of heat shock protein 70-interacting protein. GCH1 is a client protein for Hsp90. The U-box domain of CHIP is essential for ADMA-mediated GCH1 degradation in pulmonary arterial endothelial cells | Ovis aries |