GTP cyclohydrolase 1, Mouse, Clone: 2C4, Abnova™

Catalog No.	Quantity
89-000-680	100 μg

Catalog No. 89-000-680 Supplier Abnova Corporation Supplier No. H00002643M02

Description

Mouse monoclonal antibody raised against a full-length recombinant GCH1.

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

Sequence: MEKGPVRAPAEKPRGARCSNGFPERDPPRPGPSRPAEKPPRPEAKSAQPADGWKGERPRSEEDNELNLPNLAAAYSSILSSLGENPQRQGLLKTPWRAASAMQFFTKGYQETISDVLNDAIFDEDHDEMVIVKDIDMFSMCEHHLVPFVGKVHIGYLPNKQVLGLSKLARIVEIYSRRLQVQERLTKQIAVAITEALRPAGVGVVVEATHMCMVMRGVQKMNSKTVTSTMLGVFREDPKTREEFLTLIRS

Specifications

• Antigen: GTP cyclohydrolase 1
• Applications: ELISA, Immunofluorescence
• Classification: Monoclonal
• Clone: 2C4
• Conjugate: Unconjugated
• Description: Mouse monoclonal antibody raised against a full length recombinant GCH1.
• Formulation: PBS with no preservative; pH 7.4
• Gene: GCH1
• Gene Accession No.: BC025415
• Gene Alias: DYT14/DYT5/GCH/GTP-CH-1/GTPCH1
• Gene Symbols: GCH1
• Host Species: Mouse
• Immunogen: GCH1 (AAH25415.1, 1 a.a. ∼ 250 a.a) full-length recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
• Purification Method: Affinity Purified
• Quantity: 100 μg
• Regulatory Status: RUO
• Whole Molecule: Yes
• Primary or Secondary: Primary
• Gene ID (Entrez): 2643
• Target Species: Human
• Content And Storage: Store at -20°C or lower. Aliquot to avoid repeated freezing and thawing.
• Product Type: Antibody
• Isotype: IgG1 κ