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Proteintech
ERCC6/CSB Polyclonal Antibody
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Product Details
24291-1-AP
Applications
Tested Dilution
Publications
Product Specifications
Species Reactivity
Human
Host/Isotype
Rabbit
/ IgG
Class
Polyclonal
Type
Antibody
Immunogen
ERCC6/CSB Fusion Protein Ag18009 (1144-1493 aa encoded by BC127104)
Conjugate
Form
Liquid
Concentration
0.59 mg/mL
Purification
Antigen affinity chromatography
Storage buffer
PBS, pH 7.3, with 50% glycerol
Contains
0.02% sodium azide
Storage conditions
-20°C
Shipping conditions
Wet ice (domestic); Dry ice (international)
Product Specific Information
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)
Target Information
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.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
Bioinformatics
Protein Aliases: 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
Gene Aliases: 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
Performance Guarantee
If an Invitrogen™ antibody doesn't perform as described on our website or datasheet,we'll replace the product at no cost to you, or provide you with a credit for a future purchase.*
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