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Merck
모든 사진(8)

주요 문서

HPA028407

Sigma-Aldrich

Anti-PAH antibody produced in rabbit

enhanced validation

Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution

동의어(들):

Anti-OTTHUMG00000007911, Anti-PH, Anti-phenylalanine hydroxylase

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About This Item

MDL number:
UNSPSC 코드:
12352203
인간 단백질 도해서 번호:
NACRES:
NA.41

생물학적 소스

rabbit

Quality Level

결합

unconjugated

항체 형태

affinity isolated antibody

항체 생산 유형

primary antibodies

클론

polyclonal

제품 라인

Prestige Antibodies® Powered by Atlas Antibodies

양식

buffered aqueous glycerol solution

종 반응성

human

향상된 검증

independent
orthogonal RNAseq
Learn more about Antibody Enhanced Validation

기술

immunohistochemistry: 1:500-1:1000
western blot: 0.04-0.4 μg/mL

면역원 서열

MSTAVLENPGLGRKLSDFGQETSYIEDNCNQNGAISLIFSLKEEVGALAKVLRLFEENDVNLTHIESRPSRLKKDEYEFFTHLDKRSLPALTNIIKILRHDIGATVHELSRDKKKDTVPWFPRTIQELDRFANQILSYGAELDADHPG

UniProt 수납 번호

배송 상태

wet ice

저장 온도

−20°C

타겟 번역 후 변형

unmodified

유전자 정보

human ... PAH(5053)

일반 설명

Phenylalanine hydroxylase (PAH) gene spanning 90100 kb, with 13 exons, is mapped to human chromosome 12q23.2. The gene codes for non-heme iron enzyme, phenylalanine hydroxylase that belongs to the pteridine-dependent aromatic amino acid monooxygenase family.

면역원

phenylalanine hydroxylase recombinant protein epitope signature tag (PrEST)

애플리케이션

All Prestige Antibodies Powered by Atlas Antibodies are developed and validated by the Human Protein Atlas (HPA) project and as a result, are supported by the most extensive characterization in the industry.

The Human Protein Atlas project can be subdivided into three efforts: Human Tissue Atlas, Cancer Atlas, and Human Cell Atlas. The antibodies that have been generated in support of the Tissue and Cancer Atlas projects have been tested by immunohistochemistry against hundreds of normal and disease tissues and through the recent efforts of the Human Cell Atlas project, many have been characterized by immunofluorescence to map the human proteome not only at the tissue level but now at the subcellular level. These images and the collection of this vast data set can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. We also provide Prestige Antibodies® protocols and other useful information.

생화학적/생리학적 작용

Phenylalanine hydroxylase (PAH) catalyzes the hydroxylation of phenylalanine to tyrosine in the presence of molecular oxygen and cofactor tetrahydrobiopterin (BH4). Mutation in the gene leads to the development of phenylketonuria (PKU) and hyperphenylalaninemia.

특징 및 장점

Prestige Antibodies® are highly characterized and extensively validated antibodies with the added benefit of all available characterization data for each target being accessible via the Human Protein Atlas portal linked just below the product name at the top of this page. The uniqueness and low cross-reactivity of the Prestige Antibodies® to other proteins are due to a thorough selection of antigen regions, affinity purification, and stringent selection. Prestige antigen controls are available for every corresponding Prestige Antibody and can be found in the linkage section.

Every Prestige Antibody is tested in the following ways:
  • IHC tissue array of 44 normal human tissues and 20 of the most common cancer type tissues.
  • Protein array of 364 human recombinant protein fragments.

결합

Corresponding Antigen APREST83043

물리적 형태

Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide.

법적 정보

Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany

면책조항

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

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Storage Class Code

10 - Combustible liquids

WGK

WGK 1

Flash Point (°F)

Not applicable

Flash Point (°C)

Not applicable


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시험 성적서(COA)

Lot/Batch Number

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문서 라이브러리에서 최근에 구매한 제품에 대한 문서를 찾아보세요.

문서 라이브러리 방문

Prenatal diagnosis of Chinese families with phenylketonuria.
Liu N, et al.
Genetics and molecular research : GMR, 14, 14615-14628 (2015)
Crystal structure of the ternary complex of the catalytic domain of human phenylalanine hydroxylase with tetrahydrobiopterin and 3-(2-thienyl)-L-alanine, and its implications for the mechanism of catalysis and substrate activation
Andersen OA, et al.
Journal of Molecular Biology, 320(5), 1095-1108 (2002)
Normal values and age-dependent changes in GTP cyclohydrolase I activity in stimulated mononuclear blood cells measured by high-performance liquid chromatography
Hibiya M, et al.
Journal of Chromatography. B, Biomedical Sciences and Applications, 740(1), 35-42 (2000)
Robin A Williams et al.
The Clinical biochemist. Reviews, 29(1), 31-41 (2008-06-21)
Phenylketonuria (PKU) is an autosomal recessive inborn error of phenylalanine (Phe) metabolism resulting from deficiency of phenylalanine hydroxylase (PAH). Most forms of PKU and hyperphenylalaninaemia (HPA) are caused by mutations in the PAH gene on chromosome 12q23.2. Untreated PKU is
The catecholamine system in health and disease
Nagatsu T
Proceedings of the Japan Academy. Series B, Physical and Biological Sciences, 82(10), 388-415 (2006)

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