PHKA1 : phosphorylase kinase regulatory subunit alpha 1


Description

The PHKA1 (phosphorylase kinase regulatory subunit alpha 1) is a protein-coding gene located on chromosome X.

The PHKA1 gene instructs the production of the alpha subunit of the phosphorylase b kinase enzyme. This enzyme is a multi-component complex, containing four copies each of alpha, beta, gamma, and delta subunits. The alpha subunit (produced from the PHKA1 gene) plays a key role in regulating the enzyme's activity. This enzyme is found in various tissues, with a particularly high concentration in the liver and muscles. A specific version of the enzyme is present in muscle cells, utilizing the alpha-1 subunit encoded by the PHKA1 gene. Phosphorylase b kinase is vital for providing cells with energy. Glucose, the primary energy source, is stored as glycogen in muscle and liver cells. When energy is required, for example, during physical activity, glycogen is rapidly broken down. Phosphorylase b kinase activates another enzyme called glycogen phosphorylase b by converting it to its active form, glycogen phosphorylase a. This active form then breaks down glycogen.

Phosphorylase b kinase, the enzyme containing the PHKA1 protein, is responsible for adding a phosphate group to serine residues on specific target proteins, including troponin I. The alpha chain of the enzyme can potentially bind to calmodulin, a calcium-binding protein.

PHKA1 is also known as PHKA.

Associated Diseases


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