PRKAR1A

protein kinase cAMP-dependent type I regulatory subunit alpha

Summary

cAMP is a signaling molecule important for a variety of cellular functions. cAMP exerts its effects by activating the cAMP-dependent protein kinase, which transduces the signal through phosphorylation of different target proteins. The inactive kinase holoenzyme is a tetramer composed of two regulatory and two catalytic subunits. cAMP causes the dissociation of the inactive holoenzyme into a dimer of regulatory subunits bound to four cAMP and two free monomeric catalytic subunits. Four different regulatory subunits and three catalytic subunits have been identified in humans. This gene encodes one of the regulatory subunits. This protein was found to be a tissue-specific extinguisher that down-regulates the expression of seven liver genes in hepatoma x fibroblast hybrids. Mutations in this gene cause Carney complex (CNC). This gene can fuse to the RET protooncogene by gene rearrangement and form the thyroid tumor-specific chimeric oncogene known as PTC2. 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). Several alternatively spliced transcript variants encoding two different isoforms have been observed. [provided by RefSeq, Jan 2013]

Known Variants815 total

rsidPosition (GRCh37)AllelesClassClinVar
rs37464447617:66,407,929C/T——
rs378561017:66,425,995T/Gdownstream gene variant—
rs807218017:66,433,181A/T——
rs55367460517:66,439,969G/A——
rs180833317:66,443,929C/G——
rs290920817:66,445,652G/Aintron variant—
rs290920917:66,446,136G/Aintron variant—
rs295229517:66,447,421A/C——
rs389665617:66,451,026A/G——
rs295229317:66,454,017G/Tregulatory region variant—
rs290921717:66,463,985C/Tintron variant—
rs5604645017:66,501,103C/Aintron variant—
rs808030617:66,508,243C/A—benign
rs7891910917:66,508,374T/G—likely benign
rs11181546317:66,508,452G/T—benign
rs53400144517:66,508,527T/A—uncertain significance
rs103726089717:66,508,608G/A—likely benign
rs250933845817:66,508,609G/A—likely benign
rs55975712017:66,508,621G/T—benign
rs214308953417:66,508,627A/G—benign
rs88605330417:66,508,643C/T—uncertain significance
rs102493213817:66,508,647G/T—likely benign
rs54633809917:66,508,671C/T—uncertain significance
rs88605330517:66,508,680C/A—uncertain significance
rs123107450817:66,508,681C/A—uncertain significance
rs208529122617:66,508,682G/T—uncertain significance
rs208529130617:66,508,684C/T—uncertain significance
rs102151077017:66,508,685C/T—conflicting classifications of pathogenicity
rs250933906917:66,508,686C/G—uncertain significance
rs144102517017:66,508,687C/G—uncertain significance
rs208529153317:66,508,689G/T—uncertain significance
rs58777677317:66,508,690G/A—pathogenic
rs214309100517:66,508,692G/A—likely benign
rs208529176517:66,508,694G/A—uncertain significance
rs214309108517:66,508,695T/G—likely benign
rs214309111217:66,508,696G/T—uncertain significance
rs155581111717:66,508,703G/C—likely benign
rs991359517:66,510,824A/G—benign
rs11467081017:66,511,223C/T—likely benign
rs7284778517:66,511,299C/T—benign
rs37364691117:66,511,528C/T—likely benign
rs75053661117:66,511,530C/T—likely benign
rs19080335917:66,511,531G/T—conflicting classifications of pathogenicity
rs214314818417:66,511,533A/G—conflicting classifications of pathogenicity
rs250935704517:66,511,534G/A—uncertain significance
rs250935705317:66,511,535A/G—uncertain significance
rs250935706217:66,511,536G/A—uncertain significance
rs250935708417:66,511,539C/A—uncertain significance
rs28186477917:66,511,541A/Gmissense variantpathogenic
rs250935713817:66,511,543G/A—likely pathogenic
rs160046176217:66,511,544G/C—uncertain significance
rs148844495817:66,511,547T/C—uncertain significance
rs75264340917:66,511,548C/T—uncertain significance
rs214314848717:66,511,550G/T—uncertain significance
rs214314854417:66,511,552C/A—likely benign
rs160046178917:66,511,553A/C—uncertain significance
rs214314863317:66,511,555T/C—likely benign
rs75597892617:66,511,558C/T—likely benign
rs36921064617:66,511,559G/A—conflicting classifications of pathogenicity
rs75356509317:66,511,561C/A—likely benign
rs122429748617:66,511,562G/A—uncertain significance
rs208540022617:66,511,563C/A—uncertain significance
rs56115862917:66,511,564C/T—likely benign
rs77846862617:66,511,565A/G—uncertain significance
rs74539169217:66,511,567T/G—uncertain significance
rs146788654417:66,511,568G/A—uncertain significance
rs208540070017:66,511,573G/C—uncertain significance
rs129213370317:66,511,575C/G—uncertain significance
rs117767463717:66,511,577C/T—uncertain significance
rs214314954417:66,511,578G/A—uncertain significance
rs160046192117:66,511,579C/T—likely benign
rs250935763017:66,511,580A/G—uncertain significance
rs214314965817:66,511,582C/T—likely benign
rs214314970717:66,511,583C/A—uncertain significance
rs250935768517:66,511,584T/C—uncertain significance
rs88604122817:66,511,586C/Tstop gainedpathogenic
rs208540121817:66,511,587G/A—uncertain significance
rs77151858117:66,511,591A/T—uncertain significance
rs155581169017:66,511,594T/C—likely benign
rs141808054617:66,511,597G/A—likely benign
rs250935785717:66,511,598C/A—uncertain significance
rs160046198917:66,511,599T/G—uncertain significance
rs37245186217:66,511,600C/G—likely benign
rs214315029517:66,511,602A/G—uncertain significance
rs77711046417:66,511,603C/T—likely benign
rs87885456117:66,511,604G/T—uncertain significance
rs208540215117:66,511,605T/G—uncertain significance
rs76315837217:66,511,611A/G—uncertain significance
rs138974057217:66,511,612G/A—likely benign
rs14079578717:66,511,613C/T—uncertain significance
rs214315073217:66,511,615T/G—uncertain significance
rs77427742817:66,511,616A/G—uncertain significance
rs214315078717:66,511,617A/C—uncertain significance
rs214315081417:66,511,618C/T—likely benign
rs250935816417:66,511,619A/G—uncertain significance
rs123531738617:66,511,621T/G—uncertain significance
rs28186478017:66,511,622C/Gmissense variantuncertain significance
rs160046219117:66,511,623A/C—uncertain significance
rs214315100917:66,511,624A/G—likely benign
rs76072694117:66,511,625G/T—uncertain significance

Showing 100 of 815 variants. Use the SNP search for the full list.

Gene information from NCBI Gene. Variant classifications from ClinVar.