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Merck

HPA001081

Sigma-Aldrich

Anti-PHKA1 antibody produced in rabbit

enhanced validation

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

Synonim(y):

Anti-Phosphorylase b kinase regulatory subunit-α, skeletal muscle isoform antibody produced in rabbit, Anti-Phosphorylase kinase-α M subunit antibody produced in rabbit

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About This Item

Kod UNSPSC:
12352203
Numer w atlasie ludzkich białek:
NACRES:
NA.41

pochodzenie biologiczne

rabbit

Poziom jakości

białko sprzężone

unconjugated

forma przeciwciała

affinity isolated antibody

rodzaj przeciwciała

primary antibodies

klon

polyclonal

linia produktu

Prestige Antibodies® Powered by Atlas Antibodies

Postać

buffered aqueous glycerol solution

reaktywność gatunkowa

human

rozszerzona walidacja

orthogonal RNAseq
Learn more about Antibody Enhanced Validation

metody

immunofluorescence: 0.25-2 μg/mL
immunohistochemistry: 1:50-1:200
western blot: 0.04-0.4 μg/mL

sekwencja immunogenna

AHSLRCSAEEATEGLMNLSPSAMKNLLHHILSGKEFGVERSVRPTDSNVSPAISIHEIGAVGATKTERTGIMQLKSEIKQSPGTSMTPSSGSFPSAYDQQSSKDSRQGQWQ

Warunki transportu

wet ice

temp. przechowywania

−20°C

docelowa modyfikacja potranslacyjna

unmodified

informacje o genach

Opis ogólny

The gene PHKA1 (phosphorylase kinase, α 1) encodes the skeletal muscle isoform of the α subunit of phosphorylase kinase. This enzyme is a hexadekameric protein with 16 subunits containing four each of α, β, γ and δ. The gene is mapped to chromosome X in humans.

Immunogen

Phosphorylase b kinase regulatory subunit-α, skeletal muscle isoform recombinant protein epitope signature tag (PrEST)

Zastosowanie

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.

Działania biochem./fizjol.

The hexadekameric complex of phosphorylase kinase (Phk) is involved in the regulation of glycogen breakdown by phosphorylation of glycogen phosphorylase. Mutation in the PHKA1 (phosphorylase kinase, α 1) gene can cause a deficiency of Phk leading to glycogen storage diseases or muscle-specific glycogenosis. This is characterized by exercise intolerance with early fatigue, pain, cramps and rarely myoglobinuria.

Cechy i korzyści

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.

Powiązanie

Corresponding Antigen APREST74010

Postać fizyczna

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

Informacje prawne

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

10 - Combustible liquids

Klasa zagrożenia wodnego (WGK)

WGK 1

Temperatura zapłonu (°F)

Not applicable

Temperatura zapłonu (°C)

Not applicable

Środki ochrony indywidualnej

Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)


Certyfikaty analizy (CoA)

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Masz już ten produkt?

Dokumenty związane z niedawno zakupionymi produktami zostały zamieszczone w Bibliotece dokumentów.

Odwiedź Bibliotekę dokumentów

J B Lyon et al.
Science (New York, N.Y.), 155(3769), 1550-1551 (1967-03-24)
The gene for phosphoryl ase b kinase, a skeletal muscle enzyme, has been located on the X chromo some of mice. The inheritance of the enzyme through two generations from original matings between one inbred strain of mice, the I
A Schneider et al.
Nature genetics, 5(4), 381-385 (1993-12-01)
Heritable phosphorylase kinase (Phk) deficiency underlies a group of glycogenoses in humans, mice and rats that differ in mode of inheritance and tissue-specificity. It is assumed that this heterogeneity is caused by mutations affecting different subunits and isoforms of Phk.
Barbara Burwinkel et al.
European journal of human genetics : EJHG, 11(7), 516-526 (2003-06-26)
Muscle-specific deficiency of phosphorylase kinase (Phk) causes glycogen storage disease, clinically manifesting in exercise intolerance with early fatiguability, pain, cramps and occasionally myoglobinuria. In two patients and in a mouse mutant with muscle Phk deficiency, mutations were previously found in

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