PHKA1 KO cell lysate available now. KO validated by Western blot. Free of charge wild type control included. Knockout achieved by using CRISPR/Cas9, 2 bp deletion in exon14.
5330411D17, 9830108K24Rik, KPB1_HUMAN, MGC132604, PHKA, Pcyt1b, Phosphorylase b kinase regulatory subunit alpha, Phosphorylase b kinase regulatory subunit alpha, skeletal muscle isoform, Phosphorylase kinase alpha M subunit, RP23 210E20.1, kinase PHKA1, phosphorylase kinase, alpha 1 (muscle), skeletal muscle isoform
PHKA1 KO cell lysate available now. KO validated by Western blot. Free of charge wild type control included. Knockout achieved by using CRISPR/Cas9, 2 bp deletion in exon14.
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Lysate preparation: Our lysates are made using RIPA buffer to which we add a protease inhibitor cocktail and phosphatase inhibitor cocktail (ratio: 300:100:10). This means that the protein of interest is denatured. If you require a native form of the protein please use the live cell version. Please refer to our lysis protocol for further details on how our lysates are prepared.
User storage instructions: Lyophilizate may be stored at 4°C. After reconstitution, store at -20°C for short-term storage or -80°C for long-term storage.
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PHKA1 also known as phosphorylase kinase alpha 1 is a regulatory subunit of the phosphorylase kinase enzyme complex with a molecular mass around 138 kDa. This protein is mainly expressed in skeletal muscle and heart tissue. PHKA1 plays an important role in glycogen metabolism by regulating the conversion of phosphorylase b to phosphorylase a which ultimately leads to glycogen breakdown.
Glycogen metabolism involves PHKA1 as part of the phosphorylase kinase complex. This complex exists in the muscle and liver and includes alpha beta gamma and delta subunits. PHKA1 binds together with these subunits to form a large complex helping to regulate the activity state of the enzyme. The regulation of glycogen phosphorylase is important for maintaining energy supply in cells particularly during physical activity.
The activity of PHKA1 impacts glycogenolysis which is a significant metabolic pathway responsible for breaking down glycogen into glucose-1-phosphate. This pathway involves other proteins like glycogen phosphorylase and phosphoprotein phosphatase 1. PHKA1 has interactions within the cascade that ensures proper glycogen breakdown especially during conditions requiring increased energy output.
Mutations in PHKA1 are linked to glycogen storage disease type IXd (GSD IXd) which primarily affects muscle tissue. This condition features exercise intolerance and muscle weakness due to impaired glycogen breakdown. PHKB another homologous phosphorylase kinase subunit can connect to similar energy metabolism disruptions showing a clear association with the disorder pathway alongside PHKA1.
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Homozygous: 2 bp deletion in exon14
Lanes 1 - 2: Western blot - Anti-PHKA1 antibody [EPR12118] - BSA and Azide free (Anti-PHKA1 antibody [EPR12118] - BSA and Azide free ab249913) at 1/1000 dilution
Lanes 1 - 2: Western blot - Anti-PHKA1 antibody [EPR12118] (Anti-PHKA1 antibody [EPR12118] ab176338) at 1/1000 dilution
Lane 1: Wild-type HEK-293T cell lysate at 20 µg
Lane 2: PHKA1 knockout HEK-293T cell lysate at 20 µg
Lane 2: Western blot - Human PHKA1 knockout HEK-293T cell line (Human PHKA1 knockout HEK-293T cell line ab267337)
Lane 2: Western blot - Human PHKA1 knockout HEK-293T cell lysate (ab258111)
All lanes: Goat anti-Rabbit IgG H&L 800CW and Goat anti-Mouse IgG H&L 680RD at 1/20000 dilution
Performed under reducing conditions.
Observed band size: 130 kDa
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