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Rabbit Anti-PAH Polyclonal Antibody#abs135975

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Description

Catalog-specification	Delivery time	USD price
abs135975-100ug	1-2 Weeks	301.0
abs135975-50ug	1-2 Weeks	201.0

Overview
catalog	abs135975
Description	Phenylalanine hydroxylase is an enzyme encoded by PAH and plays a crucial role as the rate-limiting step in the breakdown of phenylalanine. If this enzyme's activity is deficient due to genetic causes, it can lead to a rare inherited disorder known as phenylketonuria, which is inherited in an autosomal recessive pattern.
Other names	Phenylalanine hydroxylase, also known as PH or PAH, is an enzyme that plays a critical role in the catabolism of phenylalanine. This enzyme is responsible for the conversion of phenylalanine into tyrosine, an essential amino acid. Deficiencies in this enzyme are associated with phenylketonuria (PKU), a genetic disorder that affects the body's ability to metabolize phenylalanine. PKU can lead to neurological damage, intellectual disability, and other health problems if left untreated. Phe-4-monooxygenase and Phenylalanine-4-hydroxylase are alternative names for the same enzyme. The PKU1 gene encodes for the synthesis of phenylalanine hydroxylase, and mutations in this gene can give rise to PKU.
Source	Rabbit
Specificity	The PAH Antibody is capable of recognizing the natural amounts of total PAH present within the system. This implies that it can specifically target and detect endogenous levels of PAH.
Species Reactivity	Human;Mouse;Rat
Predictive reaction species	Rabbit;Pig;Dog;Horse;
Antigen	PAH
Application	The dilution ratios for Western blot (WB) range from 1:500 to 1:2000, for immunohistochemistry (IHC) range from 1:50 to 1:200, and for ELISA (peptide) range from 1:20000 to 1:40000.
Immunogen	A synthesized peptide derived from human PAH.
MW	52kDa
Properties
Concentration	1mg/ml
purification	Utilizing SulfoLink™ Coupling Resin, the antiserum was purified through peptide affinity chromatography. To generate a highly similar content, the original text information was rearranged while maintaining its meaning.
Clonality	Polyclonal Antibody
Stability & Storage	To ensure optimal preservation, it is recommended to store the substance at a temperature of -20 °C for a duration of one year. It is important to avoid subjecting the substance to repeated cycles of freezing and thawing in order to maintain its efficacy.
Storage buffer	Rabbit IgG in phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.Store at -20 °C.Stable for 12 months from date of receipt.
Target
Background	L-phenylalanine + tetrahydrobiopterin + O2 = L-tyrosine + 4a-hydroxytetrahydrobiopterin.
Posttranslational modification	Phosphorylation at Ser-16 increases basal activity and facilitates activation by the substrate phenylalanine.
Celluar localization	Cytosol;Extracellular region or secreted;
UniPort	P00439