SAB1410516

Anti-GCH1 antibody produced in rabbit

purified immunoglobulin, buffered aqueous solution

• 同義詞: DYT14, DYT5, GCH, GTP-CH-1, GTPCH1
• 分類程式碼代碼: 12352203
• NACRES: NA.41
• 共軛: unconjugated
• application: WB
• 無性繁殖: polyclonal
• 物種活性: human
• citations: 2
• 技術: western blot: 1 μg/mL

屬性

• 生物源: rabbit
• 品質等級: 100
• 共軛: unconjugated
• 抗體表格: purified immunoglobulin
• 抗體產品種類: primary antibodies
• 無性繁殖: polyclonal
• 形狀: buffered aqueous solution
• 分子量: antigen 27.9 kDa
• 物種活性: human
• 技術: western blot: 1 μg/mL
• NCBI登錄號: NM_000161.2
• UniProt登錄號: P30793
• 運輸包裝: dry ice
• 儲存溫度: −20°C
• 目標翻譯後修改: unmodified
• 基因資訊: human ... GCH1(2643)

描述

一般說明

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)

免疫原

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

Sequence
MEKGPVRAPAEKPRGARCSNGFPERDPPRPGPSRPAEKPPRPEAKSAQPADGWKGERPRSEEDNELNLPNLAAAYSSILSSLGENPQRQGLLKTPWRAASAMQFFTKGYQETISDVLNDAIFDEDHDEMVIVKDIDMFSMCEHHLVPFVGKVHIGYLPNKQVLGLSKLARIVEIYSRRLQVQERLTKQIAVAITEALRPAGVGVVVEATHMCMVMRGVQKMNSKTVTSTMLGVFREDPKTREEFLTLIRS

外觀

Solution in phosphate buffered saline, pH 7.4

安全資訊

• 儲存類別代碼: 10 - Combustible liquids
• 水污染物質分類（WGK）: WGK 3
• 閃點（°F）: Not applicable
• 閃點（°C）: Not applicable