PRKAR1A

protein kinase cAMP-dependent type I regulatory subunit alpha

The PRKAR1A gene provides instructions for making one part (subunit) of an enzyme called protein kinase A. This enzyme promotes cell growth and division (proliferation). Protein kinase A is made up of four protein subunits, two of which are called regulatory subunits because they control whether this enzyme is turned on or off. The PRKAR1A gene provides instructions for making one of these regulatory subunits, called type 1 alpha. Protein kinase A remains turned off when the regulatory subunits are attached to the other two subunits of the enzyme. In order to turn on protein kinase A, the regulatory subunits must break away from the enzyme.

More than 117 mutations in the PRKAR1A gene have been found to cause Carney complex. Most of these mutations result in an abnormal type 1 alpha regulatory subunit that is quickly broken down (degraded) by the cell. The lack of this regulatory subunit causes protein kinase A to be turned on more often than normal, which leads to uncontrolled cell proliferation. The signs and symptoms of Carney complex are related to the unregulated growth of cells in many parts of the body.

Cytogenetic Location: 17q24.2, which is the long (q) arm of chromosome 17 at position 24.2

Molecular Location: base pairs 68,413,623 to 68,551,316 on chromosome 17 (Homo sapiens Annotation Release 108, GRCh38.p7) (NCBI)

Cytogenetic Location: 17q24.2, which is the long (q) arm of chromosome 17 at position 24.2
  • cAMP-dependent protein kinase regulatory subunit RIalpha
  • cAMP-dependent protein kinase type I-alpha regulatory chain
  • CAR
  • CNC1
  • DKFZp779L0468
  • KAP0_HUMAN
  • MGC17251
  • PKR1
  • PPNAD1
  • PRKAR1
  • protein kinase A type 1a regulatory subunit
  • protein kinase, cAMP-dependent, regulatory subunit type I alpha
  • protein kinase, cAMP-dependent, regulatory, type I, alpha
  • protein kinase, cAMP-dependent, regulatory, type I, alpha (tissue specific extinguisher 1)
  • tissue-specific extinguisher 1
  • TSE1