Invitrogen™ CSB Polyclonal Antibody

Rabbit Polyclonal Antibody

Supplier:  Invitrogen™ PA5120625

Catalog No. PIPA5120625

Description

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.

Specifications

• Antigen: CSB
• Classification: Polyclonal
• Conjugate: Unconjugated
• Gene: ERCC6
• 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
• Host Species: Rabbit
• Purification Method: Affinity chromatography
• Regulatory Status: RUO
• Gene ID (Entrez): 2074, 306274, 319955
• Content And Storage: -20°C, Avoid Freeze/Thaw Cycles
• Form: Liquid

• Applications: ELISA, Immunohistochemistry (Paraffin), Western Blot
• Concentration: 0.7 mg/mL
• Formulation: PBS with 50% glycerol and 0.05% ProClin 300; pH 7.3
• Gene Accession No.: Q03468
• Gene Symbols: ERCC6
• Immunogen: A synthetic peptide corresponding to a sequence within amino acids 700-800 of human ERCC6 (NP_0001151).
• Quantity: 100 μL
• Primary or Secondary: Primary
• Target Species: Human, Mouse, Rat
• Product Type: Antibody
• Isotype: IgG