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.\n\nApplications:\nSuitable for use in ELISA, Western Blot, Immunoprecipitation and Immunohistochemistry. Other applications not tested.\n\nRecommended Dilutions:\nImmunohistochemistry (FFPE): 3ug/ml\nOptimal dilutions to be determined by the researcher.\n\nAmino Acid Sequence:\nENPQRQGLLKTPWRAASAMQFFTKGYQETISDVLNDAIFDEDHDEMVIVKDIDMFSMCEHHLVPFVGKVHIGYLPNKQVLGLSKLARIV\n\nStorage and Stability:\nMay be stored at 4 degrees C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degrees C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

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SPECIFICATIONS

Catalog Number

127227

Size

100ug

Applications

ELISA, IHC, IP, WB

Hosts

Mouse

Reactivities

Hum

Form

Supplied as a liquid in PBS, pH 7.2.

P Type

Mab

Purity

Purified

Isotype

IgG2a,k

References

1. Vascular Dysfunction in Streptozotocin-Induced Experimental Diabetes Strictly Depends on Insulin Deficiency. Oelze M, Knorr M, Schuhmacher S, Heeren T, Otto C, Schulz E, Reifenberg K, Wenzel P, Munzel T, Daiber A.J Vasc Res. 2011 Jan 27;48(4):275-284.

Additional Info

Recognizes human GCH1.