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GCH1 Antibody - C-terminal region : FITC (ARP59618_P050-FITC)

Selected Variant

ARP59618_P050-FITC

Size: 100 µL

Product Specifications

Gene Name

GTP cyclohydrolase 1

Gene Aliases

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

Gene ID

2643

Swiss Prot

P30793

Accession Number

NP_000152

Host

Rabbit

Reactivity

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

Immunogen

The immunogen is a synthetic peptide directed towards the C terminal region of human GCH1

Target

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.

Partner Proteins

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

Clonality

Polyclonal

Conjugation

FITC: Fluorescein Isothiocyanate

Type

Polyclonal Antibody

Applications

WB

Purification

Affinity Purified

Concentration

0.5 mg/ml

Homology

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

Format

Liquid. Purified antibody supplied in 1x PBS buffer.

Reconstitution

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.

Molecular Weight

28kDa

Shipping Conditions

Wet Ice

Protein Length

250

NCBI Gene Symbol

GCH1

NCBI GB Accession Number

GCH1

Host or Source

Rabbit

Protein Name

GTP cyclohydrolase 1

Nucleotide Accession Number

NM_000161

Peptide Sequence

LRPAGVGVVVEATHMCMVMRGVQKMNSKTVTSTMLGVFREDPKTREEFLT

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