PRKAR1A

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The cAMP-dependent protein kinase type I-alpha regulatory subunit (protein kinase A regulatory subunit-1-alpha, PKA regulatory subunit RIα, tissue-specific extinguisher 1, TSE1) is coded by the PRKAR1A gene at 17q23-q24 . Mutations cause:

The gene product is processed into cAMP-dependent protein kinase type I-alpha regulatory subunit, N-terminally processed.


The gene has interesting properties from the point of view of understanding extinction which refers in genetics to the lack of expression of a tissue-specific trait.

It is a component of one of four regulatory subunits of protein kinase A (PKA):

  • I-alpha
  • I-beta
  • II-alpha
  • II-beta

Its product is involved in signaling pathways. For example PKA regulates SMOH (Smo) cell surface accumulation and activity in response to sonic hedgehog and activation-induced cytidine deaminase (AID) in B cells .

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