SAB1410516

Anti-GCH1 antibody produced in rabbit

purified immunoglobulin, buffered aqueous solution

• Synonym(e): DYT14, DYT5, GCH, GTP-CH-1, GTPCH1
• UNSPSC-Code: 12352203
• NACRES: NA.41

Eigenschaften

• Biologische Quelle: rabbit
• Qualitätsniveau: 100
• Konjugat: unconjugated
• Antikörperform: purified immunoglobulin
• Antikörper-Produkttyp: primary antibodies
• Klon: polyclonal
• Form: buffered aqueous solution
• Mol-Gew.: antigen 27.9 kDa
• Speziesreaktivität: human
• Methode(n): western blot: 1 μg/mL
• NCBI-Hinterlegungsnummer: NM_000161.2
• UniProt-Hinterlegungsnummer: P30793
• Versandbedingung: dry ice
• Lagertemp.: −20°C
• Posttranslationale Modifikation Target: unmodified
• Angaben zum Gen: human ... GCH1(2643)

Beschreibung

Allgemeine Beschreibung

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. (provided by RefSeq)

Immunogen

GCH1 (NP_000152.1, 1 a.a. ~ 250 a.a) full-length human protein.

Sequence
MEKGPVRAPAEKPRGARCSNGFPERDPPRPGPSRPAEKPPRPEAKSAQPADGWKGERPRSEEDNELNLPNLAAAYSSILSSLGENPQRQGLLKTPWRAASAMQFFTKGYQETISDVLNDAIFDEDHDEMVIVKDIDMFSMCEHHLVPFVGKVHIGYLPNKQVLGLSKLARIVEIYSRRLQVQERLTKQIAVAITEALRPAGVGVVVEATHMCMVMRGVQKMNSKTVTSTMLGVFREDPKTREEFLTLIRS

Physikalische Form

Solution in phosphate buffered saline, pH 7.4

Sicherheitshinweise

• Lagerklassenschlüssel: 10 - Combustible liquids
• WGK: WGK 3
• Flammpunkt (°F): Not applicable
• Flammpunkt (°C): Not applicable