Rabbit Anti-PAH Recombinant Antibody (
17D10) (V2LY-0725-LY991)

Name: Rabbit Anti-PAH Recombinant Antibody (17D10)
SKU: V2LY-0725-LY991
Rating: 5 (1 reviews)

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Tested Data

Rabbit Anti-PAH Recombinant Antibody (17D10) (V2LY-0725-LY991) in IHC

IHC image of V2LY-0725-LY991 diluted at 1:100 and staining in paraffin-embedded human liver cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a Goat anti-rabbit polymer IgG labeled by HRP and visualized using 0.05% DAB.

Rabbit Anti-PAH Recombinant Antibody (17D10) (V2LY-0725-LY991) in IF

Immunofluorescence staining of MCF-7 cell with V2LY-0725-LY991 at 1:50, counter-stained with DAPI. The cells were fixed in 4% formaldehyde and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 598-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).

Datasheet

SDS

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Datasheet Target References Q & As Review & reward Protocols Associated Products

Basic Information

Host Animal

Rabbit

Clone

17D10

Application

ELISA, IHC, IF

Immunogen

A synthesized peptide derived from human PAH.

Host Species

Rabbit

Specificity

Human

Antibody Isotype

IgG

Clonality

Monoclonal Antibody

Application Notes

Application	Note
IHC	1:50-1:200
IF	1:50-1:200

Formulations & Storage [For reference only, actual COA shall prevail!]

Format

Liquid

Buffer

PBS, glycerol

Preservative

Sodium azide

Concentration

Batch dependent

Purity

> 95% Purity determined by SDS-PAGE.

Storage

Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

More Infomation

Target

Full Name

Phenylalanine Hydroxylase

Entrez Gene ID

5053

UniProt ID

P00439

Function

Catalyzes the hydroxylation of L-phenylalanine to L-tyrosine.

Biological Process

Catecholamine biosynthetic process1 PublicationNAS:BHF-UCL
Cellular amino acid biosynthetic processManual Assertion Based On ExperimentTAS:ProtInc
L-phenylalanine catabolic processIEA:UniProtKB-UniPathway
Neurotransmitter biosynthetic process1 PublicationNAS:BHF-UCL
Tyrosine biosynthetic processManual Assertion Based On ExperimentIBA:GO_Central

Cellular Location

Cytosol

Involvement in disease

Phenylketonuria (PKU):
Autosomal recessive inborn error of phenylalanine metabolism, due to severe phenylalanine hydroxylase deficiency. It is characterized by blood concentrations of phenylalanine persistently above 1200 mumol (normal concentration 100 mumol) which usually causes mental retardation (unless low phenylalanine diet is introduced early in life). They tend to have light pigmentation, rashes similar to eczema, epilepsy, extreme hyperactivity, psychotic states and an unpleasant 'mousy' odor.
Non-phenylketonuria hyperphenylalaninemia (Non-PKU HPA):
Mild form of phenylalanine hydroxylase deficiency characterized by phenylalanine levels persistently below 600 mumol, which allows normal intellectual and behavioral development without treatment. Non-PKU HPA is usually caused by the combined effect of a mild hyperphenylalaninemia mutation and a severe one.
Hyperphenylalaninemia (HPA):
Mildest form of phenylalanine hydroxylase deficiency.

PTM

Phosphorylation at Ser-16 increases basal activity and facilitates activation by the substrate phenylalanine.

Yao, Y., Shi, L., Xiao, W., Guo, S., Liu, S., Li, H., & Zhang, S. (2022). Phenylalanine hydroxylase (PAH) plays a positive role during WSSV and Vibrio parahaemolyticus infection in Litopenaeus vannamei. Fish & Shellfish Immunology, 120, 515-525.

Foreman, P. K., Margulis, A. V., Alexander, K., Shediac, R., Calingaert, B., Harding, A., ... & Landis, S. (2021). Birth prevalence of phenylalanine hydroxylase deficiency: a systematic literature review and meta-analysis. Orphanet Journal of Rare Diseases, 16(1), 253.

Mordhorst, A., Dhandapani, P., Matthes, S., Mosienko, V., Rothe, M., Todiras, M., ... & Alenina, N. (2021). Phenylalanine hydroxylase contributes to serotonin synthesis in mice.

Aubi, O., Prestegård, K. S., Jung-Kc, K., Shi, T. J. S., Ying, M., Grindheim, A. K., ... & Martinez, A. (2021). The Pah-R261Q mouse reveals oxidative stress associated with amyloid-like hepatic aggregation of mutant phenylalanine hydroxylase. Nature Communications, 12(1), 2073.

Tao, Y., Han, D., Shen, H., & Li, X. (2021). Spectrum of PAH gene mutations and genotype-phenotype correlation in patients with phenylalanine hydroxylase deficiency from Shanxi province. Brain and Development, 43(2), 220-229.

Richards, D. Y., Winn, S. R., Dudley, S., Fedorov, L., Rimann, N., Thöny, B., & Harding, C. O. (2020). A novel Pah-exon1 deleted murine model of phenylalanine hydroxylase (PAH) deficiency. Molecular genetics and metabolism, 131(3), 306-315.

Arturo, E. C., Merkel, G. W., Borne, E., Hansen, M. R., Lisowski, S., Gupta, K., & Jaffe, E. K. (2020). Amino Acid Substitution at Phe80 of Mammalian Phenylalanine Hydroxylase Destabilizes Both Resting‐State and Activated Conformations Increasing the Population of Intermediates. The FASEB Journal, 34(S1), 1-1.

Flydal, M. I., Alcorlo-Pagés, M., Johannessen, F. G., Martínez-Caballero, S., Skjærven, L., Fernandez-Leiro, R., ... & Hermoso, J. A. (2019). Structure of full-length human phenylalanine hydroxylase in complex with tetrahydrobiopterin. Proceedings of the National Academy of Sciences, 116(23), 11229-11234.

Pecimonova, M., Kluckova, D., Csicsay, F., Reblova, K., Krahulec, J., Procházkova, D., ... & Soltysova, A. (2019). Structural and functional impact of seven missense variants of phenylalanine hydroxylase. Genes, 10(6), 459.

Rajabi, F., Rohr, F., Wessel, A., Martell, L., Dobrowolski, S. F., Guldberg, P., ... & Levy, H. L. (2019). Phenylalanine hydroxylase genotype-phenotype associations in the United States: A single center study. Molecular genetics and metabolism, 128(4), 415-421.

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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme Linked Immunospot (ELISpot)
Proteogenomic
Other Protocols

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Isotype control

For research use only. Not intended for any clinical use.

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