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Proteintech
ERCC6/CSB Polyclonal Antibody
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产品信息
24291-1-AP
应用
建议稀释比
已发表文章
产品规格
种属反应
Human
宿主/亚型
Rabbit
/ IgG
分类
Polyclonal
类型
Antibody
抗原
ERCC6/CSB Fusion Protein Ag18009 (1144-1493 aa encoded by BC127104)
偶联物
形式
Liquid
浓度
0.59 mg/mL
纯化类型
Antigen affinity chromatography
保存液
PBS, pH 7.3, with 50% glycerol
内含物
0.02% sodium azide
保存条件
-20°C
运输条件
Wet ice (domestic); Dry ice (international)
产品详细信息
Immunogen sequence: DESIDEKLG LSYKRERPSQ AQTEAFWENK QMENNFYKHK SKTKHHSVAE EETLEKHLRP KQKPKNSKHC RDAKFEGTRI PHLVKKRRYQ KQDSENKSEA KEQSNDDYVL EKLFKKSVGV HSVMKHDAIM DGASPDYVLV EAEANRVAQD APKALRLSRQ RCLGAVSGVP TWTGHRGISG APAGKKSRFG KKRNSNFSVQ HPSSTSPTEK CQDGIMKKEG KDNVPEHFSG RAEDADSSSG PLASSSLLAK MRARNHLILP ERLESESGHL QEASALLPTT EHDDLLVEMR NFIAFQAHTD GQASTREILQ EFESKLSASQ SCVFRELLRN LCTFHRTSGG EGIWKLKPEY C (1144-1493 aa encoded by BC127104)
靶标信息
This gene encodes a member of the GTP cyclohydrolase family. The encoded protein is the first and rate-limiting enzyme in tetrahydrobiopterin 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.
仅用于科研。不用于诊断过程。未经明确授权不得转售。
生物信息学
蛋白别名: ATP-dependent helicase ERCC6; Chimeric CSB-PGBD3 protein; Chimeric ERCC6-PGBD3 protein; Cockayne syndrome group B protein; Cockayne syndrome protein CSB; DNA excision repair protein ERCC-6; ERCC6-PGBD3 fusion protein (isoform 1); excision repair cross-complementation group 6; excision repair cross-complementing rodent repair deficiency, complementation group 6
基因别名: ARMD5; CKN2; COFS; COFS1; CSB; ERCC6; POF11; RAD26; UVSS1
UniProt ID: (Human) Q03468
Entrez Gene ID: (Human) 2074
response to superoxide
transcription-coupled nucleotide-excision repair
base-excision repair
pyrimidine dimer repair
transcription elongation from RNA polymerase I promoter
transcription from RNA polymerase II promoter
response to oxidative stress
activation of JNKK activity
activation of JUN kinase activity
intrinsic apoptotic signaling pathway in response to DNA damage
response to UV
response to toxic substance
response to X-ray
response to UV-B
response to gamma radiation
regulation of DNA-templated transcription, elongation
positive regulation of DNA-templated transcription, elongation
multicellular organism growth
photoreceptor cell maintenance
positive regulation of gene expression, epigenetic
positive regulation of protein tyrosine kinase activity
