SAB1410516

Anti-GCH1 antibody produced in rabbit

purified immunoglobulin, buffered aqueous solution

• Sinonimo/i: DYT14, DYT5, GCH, GTP-CH-1, GTPCH1
• Codice UNSPSC: 12352203
• NACRES: NA.41

Proprietà

• Origine biologica: rabbit
• Coniugato: unconjugated
• Forma dell’anticorpo: purified immunoglobulin
• Tipo di anticorpo: primary antibodies
• Clone: polyclonal
• Forma fisica: buffered aqueous solution
• PM: antigen 27.9 kDa
• Reattività contro le specie: human
• tecniche: western blot: 1 μg/mL
• N° accesso NCBI: NM_000161.2
• N° accesso UniProt: P30793
• Condizioni di spedizione: dry ice
• Temperatura di conservazione: −20°C
• modifica post-traduzionali bersaglio: unmodified
• Informazioni sul gene: human ... GCH1(2643)

Descrizione

Descrizione generale

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)

Immunogeno

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

Sequence
MEKGPVRAPAEKPRGARCSNGFPERDPPRPGPSRPAEKPPRPEAKSAQPADGWKGERPRSEEDNELNLPNLAAAYSSILSSLGENPQRQGLLKTPWRAASAMQFFTKGYQETISDVLNDAIFDEDHDEMVIVKDIDMFSMCEHHLVPFVGKVHIGYLPNKQVLGLSKLARIVEIYSRRLQVQERLTKQIAVAITEALRPAGVGVVVEATHMCMVMRGVQKMNSKTVTSTMLGVFREDPKTREEFLTLIRS

Stato fisico

Solution in phosphate buffered saline, pH 7.4

Informazioni sulla sicurezza

• Codice della classe di stoccaggio: 10 - Combustible liquids
• Classe di pericolosità dell'acqua (WGK): WGK 3
• Punto d’infiammabilità (°F): Not applicable
• Punto d’infiammabilità (°C): Not applicable