Rabbit Anti-PHKA1 Recombinant Antibody (CBYC-P350) (CBMAB-P1681-YC)

Name: Rabbit Anti-PHKA1 Recombinant Antibody (CBYC-P350)
SKU: CBMAB-P1681-YC
Rating: 5 (1 reviews)

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Datasheet

SDS

Request for COA

Datasheet Target Q & As Review & reward Protocols Associated Products

Basic Information

Host Animal

Rabbit

Clone

CBYC-P350

Application

WB, IHC-P, FC

Specificity

Human, Mouse, Rat

Antibody Isotype

IgG

Clonality

Monoclonal

Application Notes

The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

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

Storage

Store at 4°C short term (1-2 weeks). Aliquot and store at-20°C long term. Avoid repeated freeze/thaw cycles.

More Infomation

Target

Full Name

Phosphorylase Kinase Regulatory Subunit Alpha 1

Introduction

Phosphorylase kinase is a polymer of 16 subunits, four each of alpha, beta, gamma and delta. The alpha subunit includes the skeletal muscle and hepatic isoforms, and the skeletal muscle isoform is encoded by this gene. The beta subunit is the same in both the muscle and hepatic isoforms, and encoded by one gene. The gamma subunit also includes the skeletal muscle and hepatic isoforms, which are encoded by two different genes. The delta subunit is a calmodulin and can be encoded by three different genes. The gamma subunits contain the active site of the enzyme, whereas the alpha and beta subunits have regulatory functions controlled by phosphorylation. The delta subunit mediates the dependence of the enzyme on calcium concentration. Mutations in this gene cause glycogen storage disease type 9D, also known as X-linked muscle glycogenosis. Alternatively spliced transcript variants encoding different isoforms have been identified in this gene. A pseudogene has been found on chromosome 1.

Entrez Gene ID

Human	5255
Mouse	18679
Rat	64561

UniProt ID

Human	P46020
Mouse	P18826
Rat	Q64649

Alternative Names

Phosphorylase Kinase Regulatory Subunit Alpha 1; Phosphorylase Kinase Alpha M Subunit; PHKA; Phosphorylase B Kinase Regulatory Subunit Alpha, Skeletal Muscle Isoform; Phosphorylase Kinase, Alpha 1 (Muscle), Muscle Glycogenosis; Phosphorylase Kinase, Alpha 1 (Muscle);

Function

Phosphorylase b kinase catalyzes the phosphorylation of serine in certain substrates, including troponin I. The alpha chain may bind calmodulin.

Biological Process

Generation of precursor metabolites and energyManual Assertion Based On ExperimentTAS:ProtInc
Glycogen metabolic processManual Assertion Based On ExperimentTAS:ProtInc

Cellular Location

Cell membrane

Involvement in disease

Glycogen storage disease 9D (GSD9D):
A metabolic disorder characterized by slowly progressive, predominantly distal muscle weakness and atrophy. Clinical features include exercise intolerance with early fatigability, pain, cramps and occasionally myoglobinuria.

PTM

Although the final Cys may be farnesylated, the terminal tripeptide is probably not removed, and the C-terminus is not methylated.

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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)
Proteogenomics
Other Protocols

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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