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MABN754 Anti-PAH Antibody, clone 6H10.1

This Anti-PAH antibody is validated for use in WB, IH for the detection of PAH.

Anti-PAH Antibody, clone 6H10.1 MSDS (material safety data sheet) or SDS, CoA and CoQ, dossiers, brochures and other available documents.
Research Category: Neuroscience Research Sub-Category: Developmental Signaling Gene Symbol: PAH UniProt Number: P00439
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MABN754
100 µg  
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      Key Spec Table

      Species ReactivityKey ApplicationsHostFormatAntibody Type
      HWB, IHCMPurifiedMonoclonal Antibody
      Description
      Catalogue NumberMABN754
      DescriptionAnti-PAH Antibody, clone 6H10.1
      Alternate Names
      • Phenylalanine-4-hydroxylase
      • PAH
      • Phe-4-monooxygenase
      Background InformationPAH, also known as Phenylalanine-4-hydroxylase , Phe-4-monooxygenase, and encoded by the gene name PAH, belongs to the biopterin-dependent aromatic amino acid hydroxylase family. Phenylalanine hydroxylase is the rate-limiting enzyme of the metabolic pathway that degrades excess phenylalanine. Phenylalanine hydroxylase (PheOH, alternatively PheH or PAH) is an enzyme that catalyzes the hydroxylation of the aromatic side-chain of phenylalanine to generate tyrosine. PheOH is one of three members of the pterin-dependent amino acid hydroxylases, a class of monooxygenase that uses tetrahydrobiopterin (BH4, a pteridine cofactor) and a non-heme iron for catalysis. During the reaction, molecular oxygen is heterolytically cleaved with sequential incorporation of one oxygen atom into BH4 and phenylalanine substrate. PAH has been associated with Phenylketonuria PKU, an autosomal recessive inborn error of phenylalanine metabolism, due to severe phenylalanine hydroxylase deficiency. Additioanlly, PAH has been associated with Non-phenylketonuria hyperphenylalaninemia (Non-PKU HPA), a mild form of phenylalanine hydroxylase deficiency characterized by phenylalanine levels persistently below 600 mumol, which allows normal intellectual and behavioral development without treatment. Finally, PAH may play a role in the Hyperphenylalaninemia (HPA), a mildest form of phenylalanine hydroxylase deficiency. PAH is broadly expressed, with greatest levels in skeletal muscle followed by heart, brain, pancreas and testis.
      References
      Product Information
      FormatPurified
      PresentationPurified mouse monoclonal IgG1κ in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.
      Quality LevelMQ100
      Applications
      ApplicationThis Anti-PAH antibody is validated for use in WB, IH for the detection of PAH.
      Key Applications
      • Western Blotting
      • Immunohistochemistry
      Application NotesWestern Blotting Analysis: 1.0 µg/mL from a representative lot detected PAH in 10 µg of human liver tissue lysate.
      Immunohistochemistry Analysis: A 1:50-250 dilution from a representative lot detected PAH in human cerebral cortex and human liver tissue.
      Biological Information
      ImmunogenGST-tagged recombinant protein corresponding to human PAH.
      Clone6H10.1
      ConcentrationPlease refer to lot specific datasheet.
      HostMouse
      IsotypeIgG1κ
      Species Reactivity
      • Human
      Antibody TypeMonoclonal Antibody
      Entrez Gene Number
      Gene Symbol
      • PAH
      Purification MethodProtein G Purified
      UniProt Number
      Molecular Weight~52 kDa observed
      Physicochemical Information
      Dimensions
      Materials Information
      Toxicological Information
      Safety Information according to GHS
      Safety Information
      Product Usage Statements
      Quality AssuranceEvaluated by Western Blotting in HepG2 cell lysate.

      Western Blotting Analysis: 1.0 µg/mL of this antibody detected PAH in 10 µg of HepG2 cell lysate.
      Usage Statement
      • Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
      Storage and Shipping Information
      Storage ConditionsStable for 1 year at 2-8°C from date of receipt.
      Packaging Information
      Material Size100 µg
      Transport Information
      Supplemental Information
      Specifications
      Global Trade Item Number
      Catalogue Number GTIN
      MABN754 04055977122374