HPA001081

Anti-PHKA1 antibody produced in rabbit

Name: Anti-PHKA1 antibody produced in rabbit
Brand: SIGMA
Price: 881 CAD

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

Synonyme(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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100 μL

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A propos de cet article

UNSPSC Code:

12352203

NACRES:

NA.41

Human Protein Atlas Number:

HPA001081

Conjugate:

unconjugated

Clone:

polyclonal

Application:

immunofluorescence
immunohistochemistry
western blot

Species reactivity:

human

Citations:

Technique(s):

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

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Nom du produit

Anti-PHKA1 antibody produced in rabbit, Prestige Antibodies^® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution

biological source

rabbit

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

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Cet article	HPA000869	HPA000839	HPA006456
Sigma-Aldrich HPA001081 Anti-PHKA1 antibody produced in rabbit	Sigma-Aldrich HPA000869 Anti-GTF2A1 antibody produced in rabbit	Sigma-Aldrich HPA000839 Anti-GABRA3 antibody produced in rabbit	Sigma-Aldrich HPA006456 Anti-AK1 antibody produced in rabbit
biological source rabbit	biological source rabbit	biological source rabbit	biological source rabbit
Quality Level 100	Quality Level 100	Quality Level 100	Quality Level 100
conjugate unconjugated	conjugate unconjugated	conjugate unconjugated	conjugate unconjugated
antibody form affinity isolated antibody	antibody form affinity isolated antibody	antibody form affinity isolated antibody	antibody form affinity isolated antibody
product line Prestige Antibodies^® Powered by Atlas Antibodies	product line Prestige Antibodies^® Powered by Atlas Antibodies	product line -	product line Prestige Antibodies^® Powered by Atlas Antibodies
species reactivity human	species reactivity human	species reactivity human	species reactivity mouse, rat, human

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.

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)

Other Notes

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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Classe de stockage

10 - Combustible liquids

wgk

WGK 1

flash_point_f

Not applicable

flash_point_c

Not applicable

ppe

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

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Certificats d'analyse (COA)

Lot/Batch Number

R00216

R000216

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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

Contenu apparenté

Procedure for Fixation Immunostaining and Imaging in 384-well Plates

Prestige Antibodies Immunofluorescence Procedure

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