GCH1 Antilichaam - C-terminal regio : HRP (ARP59618 P050-HRP)

GTP cyclohydrolase 1

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

2643

P30793

NP_000152

Human, Mouse, Rat, Cow, Dog, Guinea Pig, Horse, Rabbit, Yeast, Zebrafish

The immunogen is a synthetic peptide directed towards the C terminal region of human 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.

GCH1; CALML6; CAMKK1; CSNK1G1; CALML5; PACSIN3; RGS19; MAP2K4; MAPK12; MAP2K5; MAPK10; PRKAR2B; PRKAR2A; PPM1B; CSNK2B; CSNK2A1; GIT2; YWHAZ; CNRIP1; RAB26; AHSA1; SP3; RLF; HSPA8; GCHFR; ATP1B1; CDC37; SH3GL2; OSBPL9; RPH3A

HRP: Horseradish Peroxidase

Polyclonal Antibody

WB

Affinity Purified

0.5 mg/ml

Cow: 100%; Dog: 100%; Guinea Pig: 100%; Horse: 100%; Human: 100%; Mouse: 100%; Rabbit: 100%; Rat: 100%; Yeast: 85%; Zebrafish: 93%

Liquid. Purified antibody is supplied in high phosphate PBS, 100 mm phosphate, 150 mM NaCl, pH 7.6.

All conjugated antibodies should be stored in light-protected vials or covered with a light protecting material (i.e. aluminum foil) . Conjugated antibodies are stable for at least 12 months at 4C. If longer storage is desired (24 months), conjugates may be diluted with up to 50% glycerol and stored at -20C to -80C. Freezing and thawing conjugated antibodies will compromise enzyme activity as well as antibody binding.

28kDa

Wet Ice

250

GCH1

GCH1

Rabbit

GTP cyclohydrolase 1

NM_000161

LRPAGVGVVVEATHMCMVMRGVQKMNSKTVTSTMLGVFREDPKTREEFLT