Human PAH ELISA Kit (V2LY-0626-LY5711)

Name: Human PAH ELISA Kit
SKU: V2LY-0626-LY5711
Rating: 5 (1 reviews)

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Tested Data

Datasheet

SDS

Request for COA

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

Basic Information

Sensitivity

0.0049 ng/mL

Detection Range

0.01-2 ng/mL

Sample Type

Serum, Plasma, cell culture supernates

Specificity

Human

Assay Type

Sandwich

Reactivity

Human

Assay Time

1.5 h

Molecule Mass

51.9 kDa

Components

Pre-coated ELISA plate: 12 wells * 8 detachable strips
Standard solution: 0.5ml x1
Standard diluent: 3ml x1
Streptavidin-HRP: 6ml x1
Stop solution: 6ml x1
Substrate solution A: 6ml x1
Substrate solution B: 6ml x1
Wash buffer concentrate (25x): 20ml x1
Biotinylated antibody: 1ml x1

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

Storage

Store at 2-8°C

More Infomation

Target

Full Name

Phenylalanine Hydroxylase

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

Alternative Versions

Cynomolgus Recombinant PAH protein, His Tag (CAT#: V2LY-0526-LY1618)

Human Recombinant PAH protein, His Tag (CAT#: V2LY-0526-LY5947)

Mouse Pah ELISA Kit (CAT#: V2LY-0626-LY2865)

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Human Recombinant PAH protein, His Tag (CAT#: V2LY-0526-LY5947)

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Mouse Pah ELISA Kit (CAT#: V2LY-0626-LY2865)

Cynomolgus Recombinant PAH protein, His Tag (CAT#: V2LY-0526-LY1618)

Rabbit Anti-PAH Recombinant Antibody (CBYC-P138) (CAT#: CBMAB-P0677-YC)

Mouse Anti-PAH Recombinant Antibody (717CT21.1.1) (CAT#: CBMAB-P0676-YC)

Isotype control

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

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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