GTP cyclohydrolase I

GCH1
Available structures PDB Ortholog search: PDBe RCSB List of PDB id codes 1FB1
Identifiers
Aliases	GCH1, DYT14, DYT5, DYT5a, GCH, GTP-CH-1, GTPCH1, HPABH4B, GTP cyclohydrolase 1
External IDs	MGI: 95675 HomoloGene: 132 GeneCards: GCH1
Genetically Related Diseases
obesity, rheumatoid arthritis
Gene ontology Molecular function • nucleotide binding • calcium ion binding • protein homodimerization activity • zinc ion binding • GTP binding • metal ion binding • GTP-dependent protein binding • protein binding • catalytic activity • coenzyme binding • hydrolase activity • GTP cyclohydrolase I activity • translation initiation factor binding • mitogen-activated protein kinase binding Cellular component • cytoplasm • cytosol • nuclear membrane • nucleoplasm • protein complex • cytoplasmic vesicle • nucleus • neuron projection terminus Biological process • nitric oxide biosynthetic process • response to interferon-gamma • neuromuscular process controlling posture • protein heterooligomerization • protein complex assembly • negative regulation of blood pressure • dopamine biosynthetic process • regulation of lung blood pressure • regulation of nitric-oxide synthase activity • vasodilation • pteridine-containing compound biosynthetic process • 7,8-dihydroneopterin 3'-triphosphate biosynthetic process • dihydrobiopterin metabolic process • regulation of blood pressure • response to tumor necrosis factor • tetrahydrofolate biosynthetic process • response to lipopolysaccharide • positive regulation of nitric-oxide synthase activity • response to pain • tetrahydrobiopterin biosynthetic process • metabolic process • protein homooligomerization • regulation of removal of superoxide radicals • positive regulation of heart rate Sources:Amigo / QuickGO
RNA expression pattern
More reference expression data
Orthologs
Species	Human	Mouse
Entrez	2643	14528
Ensembl	ENSG00000131979	ENSMUSG00000037580
UniProt	P30793	Q05915
RefSeq (mRNA)	NM_000161 NM_001024024 NM_001024070 NM_001024071	NM_008102
RefSeq (protein)	NP_000152 NP_001019195 NP_001019241 NP_001019242	NP_032128.1 NP_032128
Location (UCSC)	Chr 14: 54.84 – 54.9 Mb	Chr 14: 47.15 – 47.19 Mb
PubMed search

The chemical reaction performed by GTPCH. The important carbons relative to the transformation are numbered for reference.
Identifiers
EC number	3.5.4.16
CAS number	37289-19-3
Databases
IntEnz	IntEnz view
BRENDA	BRENDA entry
ExPASy	NiceZyme view
KEGG	KEGG entry
MetaCyc	metabolic pathway
PRIAM	profile
PDB structures	RCSB PDB PDBe PDBsum
Gene Ontology	AmiGO / EGO
Search PMC articles PubMed articles NCBI proteins

1FB1

2643

14528

ENSG00000131979

ENSMUSG00000037580

P30793

Q05915

NM_000161
NM_001024024
NM_001024070
NM_001024071

NM_008102

NP_000152
NP_001019195
NP_001019241
NP_001019242

NP_032128.1
NP_032128

GTP cyclohydrolase I (GTPCH) (EC 3.5.4.16) is a member of the GTP cyclohydrolase family of enzymes. GTPCH is part of the folate and biopterin biosynthesis pathways. It is responsible for the hydrolysis of guanosine triphosphate (GTP) to form 7,8-dihydroneopterin triphosphate (7,8-DHNP-3'-TP, 7,8-NH₂-3'-TP).