PKAR1A: a regulatory subunit of cAMP-regulated protein kinase. The inactive form of the enzyme is composed of two regulatory chains and two catalytic chains. Activation by cAMP produces two active catalytic monomers and a regulatory dimer that binds four cAMP molecules. Four types of regulatory chains are found: I-alpha, I-beta, II-alpha, and II-beta. Their expression varies among tissues and is in some cases constitutive and in others inducible. Interacts with RFC2: the complex may be involved in cell survival. Defects in PRKAR1A are the cause of Carney complex type 1 (CNC1). CNC is a multiple neoplasia syndrome characterized by spotty skin pigmentation, cardiac and other myxomas, endocrine tumors, and psammomatous melanotic schwannomas.
Protein type: Protein kinase, regulatory subunit
Chromosomal Location of Human Ortholog: 17q24.2
Cellular Component: AMP-activated protein kinase complex; cAMP-dependent protein kinase complex; cytoplasm; cytosol; immunological synapse; membrane; neuromuscular junction; protein complex
Molecular Function: cAMP binding; cAMP-dependent protein kinase inhibitor activity; cAMP-dependent protein kinase regulator activity; protein binding; ubiquitin protein ligase binding
Biological Process: activation of protein kinase A; blood coagulation; cardiac muscle cell proliferation; energy reserve metabolic process; epidermal growth factor receptor signaling pathway; female meiosis; fibroblast growth factor receptor signaling pathway; innate immune response; mesoderm formation; negative regulation of activated T cell proliferation; negative regulation of meiosis; nerve growth factor receptor signaling pathway; phospholipase C activation; regulation of insulin secretion; regulation of transcription from RNA polymerase II promoter; renal water homeostasis; sarcomere organization; signal transduction; transmembrane transport; water transport
Disease: Acrodysostosis 1 With Or Without Hormone Resistance; Carney Complex, Type 1; Myxoma, Intracardiac; Pigmented Nodular Adrenocortical Disease, Primary, 1; Thyroid Carcinoma, Papillary
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