Overview:

PRKAR1A or the cAMP-dependent protein kinase regulatory subunit type I alpha, is part of the type 1 PKA holoenzyme. PRKAR1A is found to be a tissue-specific extinguisher that down-regulates the expression of seven liver genes in hepatoma x fibroblast hybrids (1). Mutations in PRKAR1A gene cause Carney complex (CNC) and PRKAR1A can fuse to the RET protooncogene by gene rearrangement and form the thyroid tumor-specific chimeric oncogene known as PTC2 (2). A nonconventional nuclear localization sequence (NLS) has been found for this protein which suggests a role in DNA replication via the protein serving as a nuclear transport protein for the second subunit of the Replication Factor C (RFC40).

Gene Aliases:

CAR; CNC; CNC1; DKFZp779L0468; MGC17251; PKR1; PPNAD1; PRKAR1; TSE1

Genbank Number:

NM_002734

References:

1. Bossis I, et al: PRKAR1A: normal and abnormal functions. Endocrinology 145: 5452-5458, 2004.

2. Casey M, et al: Mutations in the protein kinase A R1-alpha regulatory subunit cause familial cardiac myxomas and Carney complex. J. Clin. Invest. 106: R31-R38, 2000.