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GTPCH1 / GCH1

GTP cyclohydrolase 1

GTPCH1 / GCH1 is 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.

Gene Name: GTP cyclohydrolase 1
Synonyms: GCH1, DYT14, DYT5a, Dystonia 14, GTP cyclohydrolase 1, GTP cyclohydrolase I, GTPCH1, HPABH4B, GTP-CH-1, DYT5, GCH, GTP-CH-I
Target Sequences: NM_000161 NP_000152.1 P30793

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Proteins (6)
Over-Expression Lysate (4)
Recombinant (2)
GTPCH1 / GCH1 (6)
No (6)
GST (1)
GST, N-terminus (1)
Myc-DDK (Flag) (4)
Human (4)
293T Cells (2)
HEK 293 Cells (2)
Wheat Germ Extract (2)
Purified (1)
GTPCH1 / GCH1 Protein - 12.5% SDS-PAGE of human GCH1 stained with Coomassie Blue
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Wheat Germ Extract
GST
53.24 kDa
10 µg/$479; 25 µg/$670
GTPCH1 / GCH1 Protein - 12.5% SDS-PAGE Stained with Coomassie Blue.
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Wheat Germ Extract
GST, N-terminus
10 µg/$479; 25 µg/$670
GTPCH1 / GCH1 Protein - Western validation with an anti-DDK antibody * L: Control HEK293 lysate R: Over-expression lysate
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HEK 293 Cells
Myc-DDK (Flag)
27.9 kDa
100 µg/$494
GTPCH1 / GCH1 Protein - Western validation with an anti-DDK antibody * L: Control HEK293 lysate R: Over-expression lysate
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293T Cells
Myc-DDK (Flag)
27.9 kDa
20 µg/$150
GTPCH1 / GCH1 Protein - Western validation with an anti-DDK antibody * L: Control HEK293 lysate R: Over-expression lysate
Select
293T Cells
Myc-DDK (Flag)
27.9 kDa
20 µg/$150
GTPCH1 / GCH1 Protein - Western validation with an anti-DDK antibody * L: Control HEK293 lysate R: Over-expression lysate
Select
HEK 293 Cells
Myc-DDK (Flag)
27.9 kDa
100 µg/$494
Viewing 1-6 of 6 product results


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PLEASE NOTE

For RESEARCH USE ONLY. Intended for use by laboratory professionals. Not intended for human diagnostic or therapeutic purposes.

The data on this page has been compiled from LifeSpan internal sources, the National Center for Biotechnology Information (NCBI), and The Universal Protein Resource (UniProt).