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Anti-PHKA1 antibody produced in rabbit

Name: Anti-PHKA1 antibody produced in rabbit
Brand: SIGMA

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

Synonym(s):

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

UNSPSC Code:

12352203

Human Protein Atlas Number:

HPA001081

NACRES:

NA.41

biological source

rabbit

Quality Level

100

conjugate

unconjugated

antibody form

affinity isolated antibody

antibody product type

primary antibodies

clone

polyclonal

product line

Prestige Antibodies^® Powered by Atlas Antibodies

form

buffered aqueous glycerol solution

species reactivity

human

enhanced validation

orthogonal RNAseq
Learn more about Antibody Enhanced Validation

technique(s)

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

immunogen sequence

AHSLRCSAEEATEGLMNLSPSAMKNLLHHILSGKEFGVERSVRPTDSNVSPAISIHEIGAVGATKTERTGIMQLKSEIKQSPGTSMTPSSGSFPSAYDQQSSKDSRQGQWQ

shipped in

wet ice

storage temp.

−20°C

target post-translational modification

unmodified

Gene Information

human ... PHKA1P1(646780)

General description

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)

Application

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.

Biochem/physiol Actions

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.

Features and Benefits

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.

Linkage

Corresponding Antigen APREST74010

Physical form

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

Legal Information

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

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Storage Class Code

10 - Combustible liquids

WGK

WGK 1

Flash Point(F)

Not applicable

Flash Point(C)

Not applicable

Personal Protective Equipment

dust mask type N95 (US), Eyeshields, Gloves

Certificates of Analysis (COA)

Search for Certificates of Analysis (COA) by entering the products Lot/Batch Number. Lot and Batch Numbers can be found on a product’s label following the words ‘Lot’ or ‘Batch’.

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Phosphorylase b kinase inheritance in mice.

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

Phosphorylase kinase deficiency in I-strain mice is associated with a frameshift mutation in the alpha subunit muscle isoform.

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.

Muscle glycogenosis with low phosphorylase kinase activity: mutations in PHKA1, PHKG1 or six other candidate genes explain only a minority of cases.

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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