GTP cyclohydrolase 1 (GCH1) Mouse Monoclonal Antibody [Kloon ID: LBI5A1]

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. [provided by RefSeq, Jul 2008]

Carrier-free (BSA/glycerol-free) GCH1 mouse monoclonal antibody, clone LBI5A1

DYT5; DYT5a; DYT14; GCH; GTP-CH-1; GTPCH1; HPABH4B

Homo sapiens GTP cyclohydrolase 1 (GCH1), transcript variant 2, mRNA.

2643

P30793

Unconjugated

Folate biosynthesis, Metabolic pathways

Folate biosynthesis, Metabolic pathways

WB 1:2000, IHC 1:500

Lyophilized powder (original buffer 1X PBS, pH 7.3, 8% trehalose)

Lyophilized powder (original buffer 1X PBS, pH 7.3, 8% trehalose)

Druggable Genome

Stable for 1 year at -20°C from date of shipment. For maximum recovery of product, centrifuge the original vial after thawing and opening the cap. Aliquot will be stable at 4°C short term. For long-term storage, aliquot and store at -20°C or below. Stable for 12 months at -20°C. Avoid repeated freeze-thaw cycles.

GCH1

https://www.ncbi.nlm.nih.gov/gene/?term=2643

https://www.uniprot.org/uniprot/P30793