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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