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Invitrogen™ CSB Polyclonal Antibody
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Catalog No. PIPA5120625
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100 μL
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PIPA5120625 100 μL
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Catalog No. PIPA5120625 Supplier Invitrogen™ Supplier No. PA5120625
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Description

Rabbit Polyclonal Antibody

Positive test controls include: 293T, A-549, Mouse thymus, Rat testis. Immunogen sequence: LPVFMEQFSV PITMGGYSNA SPVQVKTAYK CACVLRDTIN PYLLRRMKSD VKMSLSLPDK NEQVLFCRLT DEQHKVYQNF VDSKEVYRIL NGEMQIFSGL I.

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.
TRUSTED_SUSTAINABILITY

Specifications

Antigen CSB
Applications ELISA, Immunohistochemistry (Paraffin), Western Blot
Classification Polyclonal
Concentration 0.7 mg/mL
Conjugate Unconjugated
Formulation PBS with 50% glycerol and 0.05% ProClin 300; pH 7.3
Gene ERCC6
Gene Accession No. Q03468
Gene Alias 4732403I04; ARMD5; ATP-dependent helicase ERCC6; C130058G22Rik; Chimeric CSB-PGBD3 protein; Chimeric ERCC6-PGBD3 protein; CKN2; Cockayne syndrome group B protein; cockayne syndrome protein CSB; COFS; COFS1; CS group B correcting; CSB; DNA excision repair protein ERCC-6; DNA excision repair protein ERCC-6; ERCC6-PGBD3 fusion protein; ERCC excision repair 6, chromatin remodeling factor; ERCC6; ERCC6-PGBD3 fusion protein (isoform 1); excision repair cross-complementation group 6; excision repair cross-complementing rodent repair deficiency, complementation group 6; POF11; RAD26; UVSS1
Gene Symbols ERCC6
Host Species Rabbit
Immunogen A synthetic peptide corresponding to a sequence within amino acids 700-800 of human ERCC6 (NP_0001151).
Purification Method Affinity chromatography
Quantity 100 μL
Regulatory Status RUO
Primary or Secondary Primary
Gene ID (Entrez) 2074, 306274, 319955
Target Species Human, Mouse, Rat
Content And Storage -20°C, Avoid Freeze/Thaw Cycles
Product Type Antibody
Form Liquid
Isotype IgG
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