G9005-98B
Clone Type
PolyclonalHost
GoatSource
HumanIsotype
IgGGrade
Affinity PurifiedApplications
E WBCrossreactivity
Hu Mo RtAccession #
NP_000152.1, NP_001019195.1Gene ID
2643, 14528, 29244Shipping Temp
Blue IceStorage Temp
-20°CGoat Anti-GCH1 (GTP Cyclohydrolase 1, GTP Cyclohydrolase I, GTP-CH-I, DYT5, GCH)
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.
Applications
Suitable for use in ELISA and Western Blot. Other applications not tested.
Recommended Dilution
ELISA: 1:16,000 Western Blot: 0.5-1.5ug/ml, observed in human tonsil, lymph node and thymus lysates. on ~37kD bands Optimal dilutions to be determined by the researcher.
Storage and Stability
May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Immunogen
Synthetic peptide corresponding to C-GKVHIGYLPNKQ, from human GCH1, at the internal region of the protein (NP_000152.1, NP_001019195.1).
Form
Supplied as a liquid in Tris saline, 0.02% sodium azide, pH 7.3, 0.5% BSA.
Purity
Purified by immunoaffinity chromatography.
Specificity
Recognizes human GCH1. Species sequence homology: Mouse and rat.
