GCH1
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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
Recombinant Proteins
- Human
- Rat
- Mouse
- Chicken
- Zebrafish
- E.coli
- Mammalian Cells
- Wheat Germ
- HEK293
- In Vitro Cell Free System
- His
- Non
- His&Fc&Avi
- GST
| Cat.# | Product name | Source (Host) | Species | Tag | Protein Length | Price |
|---|---|---|---|---|---|---|
| GCH1-3192H | Recombinant Human GCH1 protein, His-tagged | E.coli | Human | His | 1-250 aa | |
| GCH1-2489R | Recombinant Rat GCH1 Protein | Mammalian Cells | Rat | His |
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| GCH1-28280TH | Recombinant Human GCH1 | Wheat Germ | Human | Non | 251 amino acids |
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| GCH1-28283TH | Recombinant Human GCH1 | Wheat Germ | Human | Non | 89 amino acids |
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| GCH1-470H | Recombinant Human GCH1 | Mammalian Cells | Human | His |
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| GCH1-6263M | Recombinant Mouse GCH1 Protein | Mammalian Cells | Mouse | His |
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| GCH1-6776C | Recombinant Chicken GCH1 | Mammalian Cells | Chicken | His |
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| GCH1-7210Z | Recombinant Zebrafish GCH1 | Mammalian Cells | Zebrafish | His |
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| GCH1-801H | Recombinant Human GCH1 Protein, His-tagged | E.coli | Human | His |
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| GCH1-5988HCL | Recombinant Human GCH1 293 Cell Lysate | HEK293 | Human | Non |
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| GCH1-183HF | Recombinant Full Length Human GCH1 Protein | In Vitro Cell Free System | Human | Full L. 251 amino acids |
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| GCH1-2145R | Recombinant Rat GCH1 Protein, His (Fc)-Avi-tagged | HEK293 | Rat | His&Fc&Avi |
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| GCH1-2145R-B | Recombinant Rat GCH1 Protein Pre-coupled Magnetic Beads | HEK293 | Rat |
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| GCH1-2197H | Recombinant Human GCH1 Protein, His-tagged | E.coli | Human | His | Ala16-Ser250 |
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| GCH1-3024H | Recombinant Human GCH1 Protein, His (Fc)-Avi-tagged | HEK293 | Human | His&Fc&Avi |
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| GCH1-3024H-B | Recombinant Human GCH1 Protein Pre-coupled Magnetic Beads | HEK293 | Human |
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| GCH1-3504M | Recombinant Mouse GCH1 Protein, His (Fc)-Avi-tagged | HEK293 | Mouse | His&Fc&Avi |
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| GCH1-3504M-B | Recombinant Mouse GCH1 Protein Pre-coupled Magnetic Beads | HEK293 | Mouse |
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| GCH1-4794H | Recombinant Human GCH1 Protein, GST-tagged | Wheat Germ | Human | GST |
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| GCH1-5170HF | Recombinant Full Length Human GCH1 Protein, GST-tagged | In Vitro Cell Free System | Human | GST | Full L. 250 amino acids |
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Involved Pathway
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 |
|---|---|
| Metabolic pathways | NOS2A,ACSL5,LTC4S,MAPK1IP1L,SC5D,SMPD5,ATP5A1,ATP6V1B2,THTPA,UPB1 |
| Folate biosynthesis | ALPP,GCH2,SPRA,MOCS1,QDPRA,ALP3,ALPPL2,GGH,ALPI,ALPL |
Protein Function
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-dependent protein binding | CDC42,RASGRP4,RAPGEF6,RAB5B,PARD6A,RAB32,MRAS,ARFIP2,AP3B1,AP1G1 |
| coenzyme binding | TSTA3,HMGCRB,HMGCRA,QDPRB1,ALAS2,NDUFA9,ACAT1,TDH,TDH2,GCLC |
| zinc ion binding | ZYX,FHL2,TOPORS,RNF25,LIMD1,NR4A2,C6AST3,PCGF6,LIMCH1,ADAMTSL1 |
| GTP binding | REM2,ARF6B,RAB11A,RAB40C,MFN1,SEPHS1,ARF4A,MXE,NME1,SAR1B |
| protein binding | CTR9,BIK,MRE11A,TERF2,OAS1B,FLNA,SPOPL,ZFYVE16,TRIM10,CIC |
| GTP cyclohydrolase I activity | GCH2 |
| protein homodimerization activity | CCDC88A,PAFAH1B2,SMAD4,CORO1A,TP53I3,P2RX7,NR0B1,TRIM8,ABCG1,CHRNA7 |
| calcium ion binding | SYT4,CDH12A,CALML3,SLC25A25,NECAB3,LTBP3,HPCA,PCDH1G31,ANXA1C,GSNA |
Interacting Protein
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
Resources
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References
- 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).
