Creative BioMart to Present at IMMUNOLOGY2024™|May 3-7, 2024|Booth #512 Learn More

GCH1

Official Full Name

GTP cyclohydrolase 1
Overview

This gene encodes a member of the GTP cyclohydrolase family. The encoded protein is the first and rate-limiting enzyme;in tetrahydrobiopterin (BH4) biosynthesis, catalyzing the conversion of GTP into 7,8-dihydroneopterin triphosphate.;BH4 is an essential cofactor required by aromatic amino acid hydroxylases as well as nitric oxide synthases. Mutations;in this gene are associated with malignant hyperphenylalaninemia and dopa-responsive dystonia. Several alternatively;spliced transcript variants encoding different isoforms have been described; however, not all variants give rise to a;functional enzyme.
Synonyms

GCH1; GTP cyclohydrolase 1; dystonia 14 , DYT5, DYT14, GCH; dopa responsive dystonia; DYT5a; GTPCH1; GTP-CH-I; dystonia 14; GTP cyclohydrolase I; guanosine 5-triphosphate cyclohydrolase I; GCH; DYT5; DYT14; HPABH4B; GTP-CH-1;

Type

Recombinant Proteins
Cell & Tissue Lysates
Protein Pre-coupled Magnetic Beads

Species

Chicken
Human
Mouse
Rat
Zebrafish

Source

E.coli
HEK293
In Vitro Cell Free System
Mammalian Cell
Wheat Germ

Tag

GST
His
His (Fc)
Avi
N/A
N

Species	Cat.#	Product name	Source (Host)	Tag	Protein Length	Price
Human	GCH1-28280TH	Recombinant Human GCH1	Wheat Germ	N/A	251 amino acids	Inquiry
Human	GCH1-28283TH	Recombinant Human GCH1	Wheat Germ	N/A	89 amino acids	Inquiry
Human	GCH1-470H	Recombinant Human GCH1	Mammalian Cell	His		Inquiry
Human	GCH1-801H	Recombinant Human GCH1 Protein, His-tagged	E.coli	His		Inquiry
Human	GCH1-5988HCL	Recombinant Human GCH1 293 Cell Lysate	HEK293	N/A		Inquiry
Human	GCH1-4794H	Recombinant Human GCH1 Protein, GST-tagged	Wheat Germ	GST		Inquiry
Human	GCH1-183HF	Recombinant Full Length Human GCH1 Protein	In Vitro Cell Free System		251 amino acids	Inquiry
Human	GCH1-3192H	Recombinant Human GCH1 protein, His-tagged	E.coli	His	1-250 aa	$319 / 25ug
Human	GCH1-3024H	Recombinant Human GCH1 Protein, His (Fc)-Avi-tagged	HEK293	His (Fc)-Avi		Inquiry
Human	GCH1-2197H	Recombinant Human GCH1 Protein, His-tagged	E.coli	N-His	Ala16-Ser250	Inquiry
Human	GCH1-5170HF	Recombinant Full Length Human GCH1 Protein, GST-tagged	In Vitro Cell Free System	GST	250 amino acids	Inquiry
Human	GCH1-3024H-B	Recombinant Human GCH1 Protein Pre-coupled Magnetic Beads	HEK293			Inquiry
Mouse	GCH1-6263M	Recombinant Mouse GCH1 Protein	Mammalian Cell	His		Inquiry
Mouse	GCH1-3504M-B	Recombinant Mouse GCH1 Protein Pre-coupled Magnetic Beads	HEK293			Inquiry
Mouse	GCH1-3504M	Recombinant Mouse GCH1 Protein, His (Fc)-Avi-tagged	HEK293	His (Fc)-Avi		Inquiry
Rat	GCH1-2489R	Recombinant Rat GCH1 Protein	Mammalian Cell	His		Inquiry
Rat	GCH1-2145R-B	Recombinant Rat GCH1 Protein Pre-coupled Magnetic Beads	HEK293			Inquiry
Rat	GCH1-2145R	Recombinant Rat GCH1 Protein, His (Fc)-Avi-tagged	HEK293	His (Fc)-Avi		Inquiry
Zebrafish	GCH1-7210Z	Recombinant Zebrafish GCH1	Mammalian Cell	His		Inquiry
Chicken	GCH1-6776C	Recombinant Chicken GCH1	Mammalian Cell	His		Inquiry

Involved Pathway
Protein Function
Interacting Protein
GCH1 Related Articles

GCH1 involved in several pathways and played different roles in them. We selected most pathways GCH1 participated on our site, such as Folate biosynthesis, Metabolic pathways, which may be useful for your reference. Also, other proteins which involved in the same pathway with GCH1 were listed below. Creative BioMart supplied nearly all the proteins listed, you can search them on our site.

Pathway Name	Pathway Related Protein
Folate biosynthesis	PTS;QDPR;ALPI.1;DHFRL1;QDPRA;ALPI;GCH2;FPGS;GGH
Metabolic pathways	PGP;CERS4;AGMAT;ALDH3A2;UQCR11;DHRS3B;DCT;URAD;ALOX8

GCH1 has several biochemical functions, for example, GTP binding, GTP cyclohydrolase I activity, NOT GTP cyclohydrolase I activity. Some of the functions are cooperated with other proteins, some of the functions could acted by GCH1 itself. We selected most functions GCH1 had, and list some proteins which have the same functions with GCH1. You can find most of the proteins on our site.

Function	Related Protein
GTP binding	RHOC;GNAV1;RIT2;RAB41;GLUD1;MXC;RAB14;DIRAS2;SEPT7B
GTP cyclohydrolase I activity	GCH1;GCH2
NOT GTP cyclohydrolase I activity
GTP-dependent protein binding	EEA1;RAB32;RASGRP4;RAB5B;HPS6;ARFIP2;GCH1;RAB38;PPIAL4A
calcium ion binding	NOTCH4;DST;RCV1;EML1;RASGRP1;PLCD3A;PKD2;BGLAP2;ADAM8
coenzyme binding	GCH1;HMGCR;ATAT1;NDUFA9A;NDUFA9;TSTA3;ACAT1;HMGCRA;TDH2
protein binding	SKA1;LMO2;C12orf43;TSEN54;NAV1;MTAP;RHOV;UBE2D4;LOX
protein homodimerization activity	MCL1;TRIM9;MTUS2;PRTFDC1;PVRL1A;LHPP;MAFA;NCR3;ABCD1
zinc ion binding	DPP3;PEG10;P2RX2;RNF150;ALPI;HHIP;CA6;ZDHHC22;MMP10

GCH1 has direct interactions with proteins and molecules. Those interactions were detected by several methods such as yeast two hybrid, co-IP, pull-down and so on. We selected proteins and molecules interacted with GCH1 here. Most of them are supplied by our site. Hope this information will be useful for your research of GCH1.

YWHAZ; AHSA1; OSBPL9; HSPA8; GCHFR; SP3; RLF; GIT2; CDC37; CNRIP1

Douglas, G; Hale, AB; et al. A requirement for Gch1 and tetrahydrobiopterin in embryonic development. DEVELOPMENTAL BIOLOGY 399:129-138(2015).

Kim, JI; Choi, JK; et al. A novel missense mutation in GCH1 gene in a Korean family with Segawa disease. BRAIN & DEVELOPMENT 37:359-361(2015).