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Names & Taxonomy

Uniprot ID:: P30793
Entry Name:: GCH1_HUMAN
Status:: reviewed
Protein Names:: GTP cyclohydrolase 1 (EC 3.5.4.16) (GTP cyclohydrolase I) (GTP-CH-I)
Gene Names:: GCH1 DYT5 GCH
Gene Names Primary:: GCH1
Organism:: Homo sapiens (Human)

Structure

Length:: 250
Sequence:: MEKGPVRAPAEKPRGARCSNGFPERDPPRPGPSRPAEKPPRPEAKSAQPADGWKGERPRSEEDNELNLPNLAAAYSSILSSLGENPQRQGLLKTPWRAASAMQFFTKGYQETISDVLNDAIFDEDHDEMVIVKDIDMFSMCEHHLVPFVGKVHIGYLPNKQVLGLSKLARIVEIYSRRLQVQERLTKQIAVAITEALRPAGVGVVVEATHMCMVMRGVQKMNSKTVTSTMLGVFREDPKTREEFLTLIRS
Proteomes:: UP000005640

Subcellular location

Subcellular Location:: Cytoplasm

Function

Function:: Positively regulates nitric oxide synthesis in umbilical vein endothelial cells (HUVECs). May be involved in dopamine synthesis. May modify pain sensitivity and persistence. Isoform GCH-1 is the functional enzyme, the potential function of the enzymatically inactive isoforms remains unknown.
Pathway:: Cofactor biosynthesis; 7,8-dihydroneopterin triphosphate biosynthesis; 7,8-dihydroneopterin triphosphate from GTP: step 1/1.
Catalytic Activity:: GTP + H(2)O = formate + 2-amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)-dihydropteridine triphosphate.
Kinetics:: BIOPHYSICOCHEMICAL PROPERTIES: Kinetic parameters: KM=116 uM for GTP
Enzyme Regulation:: ENZYME REGULATION: GTP shows a positive allosteric effect, and tetrahydrobiopterin inhibits the enzyme activity. Zinc is required for catalytic activity. Inhibited by Mg(2+).
Cross Reference Drug Bank:: DB02325
Gene Ontology Go:: cytoplasm
cytoplasmic vesicle
cytosol
nuclear membrane
nucleoplasm
nucleus
protein complex
calcium ion binding
coenzyme binding
GTP binding
GTP cyclohydrolase I activity
protein homodimerization activity
zinc ion binding
7,8-dihydroneopterin 3'-triphosphate biosynthetic process
dopamine biosynthetic process
negative regulation of blood pressure
neuromuscular process controlling posture
nitric oxide biosynthetic process
nitric oxide metabolic process
positive regulation of nitric-oxide synthase activity
protein heterooligomerization
protein homooligomerization
pteridine-containing compound biosynthetic process
regulation of blood pressure
regulation of lung blood pressure
regulation of nitric-oxide synthase activity
regulation of removal of superoxide radicals
response to interferon-gamma
response to lipopolysaccharide
response to pain
response to tumor necrosis factor
small molecule metabolic process
tetrahydrobiopterin biosynthetic process
tetrahydrofolate biosynthetic process
vasodilation
Gene Ontology Biological Process:: 7,8-dihydroneopterin 3'-triphosphate biosynthetic process
dopamine biosynthetic process
negative regulation of blood pressure
neuromuscular process controlling posture
nitric oxide biosynthetic process
nitric oxide metabolic process
positive regulation of nitric-oxide synthase activity
protein heterooligomerization
protein homooligomerization
pteridine-containing compound biosynthetic process
regulation of blood pressure
regulation of lung blood pressure
regulation of nitric-oxide synthase activity
regulation of removal of superoxide radicals
response to interferon-gamma
response to lipopolysaccharide
response to pain
response to tumor necrosis factor
small molecule metabolic process
tetrahydrobiopterin biosynthetic process
tetrahydrofolate biosynthetic process
vasodilation
Gene Ontology Molecular Function:: calcium ion binding
coenzyme binding
GTP binding
GTP cyclohydrolase I activity
protein homodimerization activity
zinc ion binding
Gene Ontology Cellular Component:: cytoplasm
cytoplasmic vesicle
cytosol
nuclear membrane
nucleoplasm
nucleus
protein complex
Keywords:: 3D-structure
Allosteric enzyme
Alternative splicing
Complete proteome
Cytoplasm
Disease mutation
Dystonia
GTP-binding
Hydrolase
Metal-binding
Nucleotide-binding
Nucleus
Parkinsonism
Phenylketonuria
Phosphoprotein
Polymorphism
Reference proteome
Tetrahydrobiopterin biosynthesis
Zinc
Interacts With:: O95433; P63104

Names & Taxonomy

Structure

Subcellular location

Function

Publication