GCH1 Antibody - C-terminal region

Product Specifications

CAS Number

9007-83-4

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

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

Type

Polyclonal Antibody

Sequence

LRPAGVGVVVEATHMCMVMRGVQKMNSKTVTSTMLGVFREDPKTREEFLT

Applications

Purification

Affinity Purified

Assay Protocol

Reconstitution & Storage Instructions Western Blotting/Immunoblotting (WB/IB) Protocol Immunohistochemistry (IHC) Protocol Immunocytochemistry (ICC) Protocol Enzyme-Linked ImmunoSorbent Assay (ELISA) Protocol Blocking Peptide Competition Protocol (BPCP) Immunoprecipitation (IP) Protocol Antibody Array (AA) Protocol Reconstitution & Storage Instructions Reconstitution & Storage Instructions Western Blotting/Immunoblotting (WB/IB) Protocol Western Blotting/Immunoblotting (WB/IB) Protocol Immunohistochemistry (IHC) Protocol Immunohistochemistry (IHC) Protocol Immunocytochemistry (ICC) Protocol Immunocytochemistry (ICC) Protocol Enzyme-Linked ImmunoSorbent Assay (ELISA) Protocol Enzyme-Linked ImmunoSorbent Assay (ELISA) Protocol Blocking Peptide Competition Protocol (BPCP) Blocking Peptide Competition Protocol (BPCP) Immunoprecipitation (IP) Protocol Immunoprecipitation (IP) Protocol Antibody Array (AA) Protocol Antibody Array (AA) Protocol

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 with 0.09% (w/v) sodium azide and 2% sucrose.

Reconstitution

For short term use, store at 2-8C up to 1 week. For long term storage, store at -20C in small aliquots to prevent freeze-thaw cycles.

Molecular Weight

28kDa

Protein Length

250

NCBI Gene Symbol

GCH1

Host or Source

Rabbit

Protein Name

GTP cyclohydrolase 1

Gene Name URL

GCH1

Nucleotide Accession Number

NM_000161

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