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

Provided herein is a Rabbit monoclonal antibody against Human Phosphorylase Kinase Regulatory Subunit Alpha 1. The antibody can be used for immunoassay techniques, such as WB, IHC-P, FC.
See all PHKA1 antibodies

Summary

Host Animal
Rabbit
Specificity
Human, Mouse, Rat
Clone
CBYC-P350
Antibody Isotype
IgG
Application
WB, IHC-P, FC

Basic Information

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.

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
Human5255
Mouse18679
Rat64561
UniProt ID
HumanP46020
MouseP18826
RatQ64649
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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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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