MABN754
Anti-PAH Antibody, clone 6H10.1
clone 6H10.1, from mouse
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All Photos(3)
Synonym(s):
Phenylalanine-4-hydroxylase, PAH, Phe-4-monooxygenase
UNSPSC Code:
12352203
eCl@ss:
32160702
NACRES:
NA.41
Recommended Products
biological source
mouse
Quality Level
antibody form
purified immunoglobulin
antibody product type
primary antibodies
clone
6H10.1, monoclonal
species reactivity
human
technique(s)
immunohistochemistry: suitable
western blot: suitable
isotype
IgG1κ
NCBI accession no.
UniProt accession no.
shipped in
wet ice
target post-translational modification
unmodified
Gene Information
human ... PAH(5053)
General description
PAH, 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.
Immunogen
GST-tagged recombinant protein corresponding to human PAH.
Application
This Anti-PAH antibody is validated for use in WB, IH for the detection of PAH.
Western 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.
Immunohistochemistry Analysis: A 1:50-250 dilution from a representative lot detected PAH in human cerebral cortex and human liver tissue.
Quality
Evaluated 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.
Western Blotting Analysis: 1.0 µg/mL of this antibody detected PAH in 10 µg of HepG2 cell lysate.
Target description
~52 kDa observed
Physical form
Format: Purified
Other Notes
Concentration: Please refer to lot specific datasheet.
WGK
WGK 1
Flash Point(F)
Not applicable
Flash Point(C)
Not applicable
Certificates of Analysis (COA)
Search for Certificates of Analysis (COA) by entering the products Lot/Batch Number. Lot and Batch Numbers can be found on a product’s label following the words ‘Lot’ or ‘Batch’.
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