SAB1410516

Anti-GCH1 antibody produced in rabbit

purified immunoglobulin, buffered aqueous solution

• 동의어(들): DYT14, DYT5, GCH, GTP-CH-1, GTPCH1
• UNSPSC 코드: 12352203
• NACRES: NA.41

속성

• 생물학적 소스: rabbit
• Quality Level: 100
• 결합: unconjugated
• 항체 형태: purified immunoglobulin
• 항체 생산 유형: primary antibodies
• 클론: polyclonal
• 양식: buffered aqueous solution
• 분자량: antigen 27.9 kDa
• 종 반응성: human
• 기술: western blot: 1 μg/mL
• NCBI 수납 번호: NM_000161.2
• UniProt 수납 번호: P30793
• 배송 상태: dry ice
• 저장 온도: −20°C
• 타겟 번역 후 변형: unmodified
• 유전자 정보: human ... GCH1(2643)

설명

일반 설명

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)

면역원

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

Sequence
MEKGPVRAPAEKPRGARCSNGFPERDPPRPGPSRPAEKPPRPEAKSAQPADGWKGERPRSEEDNELNLPNLAAAYSSILSSLGENPQRQGLLKTPWRAASAMQFFTKGYQETISDVLNDAIFDEDHDEMVIVKDIDMFSMCEHHLVPFVGKVHIGYLPNKQVLGLSKLARIVEIYSRRLQVQERLTKQIAVAITEALRPAGVGVVVEATHMCMVMRGVQKMNSKTVTSTMLGVFREDPKTREEFLTLIRS

물리적 형태

Solution in phosphate buffered saline, pH 7.4

안전성 정보

• Storage Class Code: 10 - Combustible liquids
• WGK: WGK 3
• Flash Point (°F): Not applicable
• Flash Point (°C): Not applicable