PRKAR1A

protein kinase cAMP-dependent type I regulatory subunit alpha

Tier: Gold Standard Ciliary Genes

Ensembl:
ENSG00000108946
UniProt:
P10644
OMIM:
188830
Synonyms:
CNC1, PKR1, PRKAR1, PRKAR1ALPHA, TSE1

Ciliogenesis screen results (4 screens)

  • Kim2016: Not Reported
  • Wheway et al. 2015 (siRNA) [siRNA]: No effect PMID:26167766
  • Breslow et al. 2018 (CRISPR) [CRISPR]: Positive Regulator (Hh signaling, casTLE effect=-2.9) PMID:29459680
  • Roosing et al. 2015 (siRNA) [siRNA]: No effect PMID:26595381

Subcellular localization

cilia, cytosol

References: PMID:26585297, PMID:21266715, PMID:28615245, PMID:39743588

Functional category

  • Signaling (Hedgehog, GPCRs, ion channels)

Function

PRKAR1A encodes the PKA regulatory subunit RIα, which localizes to primary cilia and regulates cilia-dependent Hedgehog and cAMP signaling (PMID: 26585297, PMID: 21266715). Kidney-specific loss of Prkar1a induces cystic kidney disease and markedly aggravates Pkd1-associated cystogenesis, establishing PRKAR1A as a ciliary signaling regulator relevant to ciliopathies(PMID: 28615245). Identified by cryo-EM as a component of radial spoke 3 in sperm flagella and other motile cilia (Leung et al., 2025, Nature, PMID 39743588).