SAB1410516

Anti-GCH1 antibody produced in rabbit

purified immunoglobulin, buffered aqueous solution

• Sinônimo(s): DYT14, DYT5, GCH, GTP-CH-1, GTPCH1
• Código UNSPSC: 12352203
• NACRES: NA.41

Propriedades

• fonte biológica: rabbit
• Nível de qualidade: 100
• conjugado: unconjugated
• forma do anticorpo: purified immunoglobulin
• tipo de produto de anticorpo: primary antibodies
• clone: polyclonal
• forma: buffered aqueous solution
• peso molecular: antigen 27.9 kDa
• reatividade de espécies: human
• técnica(s): western blot: 1 μg/mL
• nº de adesão NCBI: NM_000161.2
• nº de adesão UniProt: P30793
• Condições de expedição: dry ice
• temperatura de armazenamento: −20°C
• modificação pós-traducional do alvo: unmodified
• Informações sobre genes: human ... GCH1(2643)

Descrição

Descrição geral

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)

Imunogênio

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

Sequence
MEKGPVRAPAEKPRGARCSNGFPERDPPRPGPSRPAEKPPRPEAKSAQPADGWKGERPRSEEDNELNLPNLAAAYSSILSSLGENPQRQGLLKTPWRAASAMQFFTKGYQETISDVLNDAIFDEDHDEMVIVKDIDMFSMCEHHLVPFVGKVHIGYLPNKQVLGLSKLARIVEIYSRRLQVQERLTKQIAVAITEALRPAGVGVVVEATHMCMVMRGVQKMNSKTVTSTMLGVFREDPKTREEFLTLIRS

forma física

Solution in phosphate buffered saline, pH 7.4

Informações de segurança

• Código de classe de armazenamento: 10 - Combustible liquids
• Classe de risco de água (WGK): WGK 3
• Ponto de fulgor (°F): Not applicable
• Ponto de fulgor (°C): Not applicable