PAH Recombinant Rabbit Monoclonal Antibody [JE64-47]

About this product

Phenylalanine hydroxylase (PAH) (EC 1.14.16.1) is an enzyme that catalyzes the hydroxylation of the aromatic side-chain of phenylalanine to generate tyrosine. PAH is one of three members of the biopterin-dependent aromatic 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. In humans, mutations in its encoding gene, PAH, can lead to the metabolic disorder phenylketonuria. Deficiency in PAH activity due to mutations in PAH causes hyperphenylalaninemia (HPA). Since phenylketonuria can cause irreversible damage, it is imperative that deficiencies in the phenylalanine hydroxylase are determined early on in development. Originally, this was done using a bacterial inhibition assay known as the Guthrie Test. Now, PKU is part of newborn screening in many countries, and elevated phenylalanine levels are identified shortly after birth by measurement with tandem mass spectrometry. Placing the individual on a low phenylalanine, high tyrosine diet can help prevent any long-term damage to their development.

Specifications

Catalog number

HA721122

Size / count

100 µL

Dry ice

No

Hazardous

No

Discontinued

No

CAS Number

9000-83-3

Product Name Alternative

PAH antibody PH antibody PH4H_HUMAN antibody Phe 4 monooxygenase antibody Phe-4-monooxygenase antibody Phenylalanine 4 hydroxylase antibody Phenylalanine hydroxylase antibody Phenylalanine-4-hydroxylase antibody PKU antibody PKU1 antibody

Abbreviation

PAH

Swiss Prot

P00439 Human, P16331 Mouse, P04176 Rat

Cellular Locus

Cytosol.

Host

Rabbit

Species Reactivity

Human, Mouse, Rat

Immunogen

Synthetic peptide within human PAH aa 50-100/452.

Isotype

IgG

Conjugation

Non-conjugated

Type

Recombinant Rabbit monoclonal Antibody

Applications

WB, IHC-P, FC

Positive Control

Rat liver tissue lysates, rat kidney tissue, mouse kidney tissue, HepG2.

Concentration

1 mg/mL

Dilution

WB: 1:500 ;IHC-P: 1:100-1:400 ;FC: 1:500-1:1,000

Purity

Protein A affinity purified.

Form

Liquid

Buffer

1*TBS (pH7.4), 0.05% BSA, 40% Glycerol. Preservative: 0.05% Sodium Azide.

Molecular Weight

Predicted band size: 52 kDa

Storage Conditions

Store at +4°C after thawing. Aliquot store at -20°C. Avoid repeated freeze/thaw cycles.

Recombinant Antibody

Yes