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/Abenign
rs7891910917:66,508,374T/Glikely benign
rs11181546317:66,508,452G/Tbenign
rs53400144517:66,508,527T/Auncertain significance
rs103726089717:66,508,608G/Alikely benign
rs250933845817:66,508,609G/Alikely benign
rs55975712017:66,508,621G/Tbenign
rs214308953417:66,508,627A/Gbenign
rs88605330417:66,508,643C/Tuncertain significance
rs102493213817:66,508,647G/Tlikely benign
rs54633809917:66,508,671C/Tuncertain significance
rs88605330517:66,508,680C/Auncertain significance
rs123107450817:66,508,681C/Auncertain significance
rs208529122617:66,508,682G/Tuncertain significance
rs208529130617:66,508,684C/Tuncertain significance
rs102151077017:66,508,685C/Tconflicting classifications of pathogenicity
rs250933906917:66,508,686C/Guncertain significance
rs144102517017:66,508,687C/Guncertain significance
rs208529153317:66,508,689G/Tuncertain significance
rs58777677317:66,508,690G/Apathogenic
rs214309100517:66,508,692G/Alikely benign
rs208529176517:66,508,694G/Auncertain significance
rs214309108517:66,508,695T/Glikely benign
rs214309111217:66,508,696G/Tuncertain significance
rs155581111717:66,508,703G/Clikely benign
rs991359517:66,510,824A/Gbenign
rs11467081017:66,511,223C/Tlikely benign
rs7284778517:66,511,299C/Tbenign
rs37364691117:66,511,528C/Tlikely benign
rs75053661117:66,511,530C/Tlikely benign
rs19080335917:66,511,531G/Tconflicting classifications of pathogenicity
rs214314818417:66,511,533A/Gconflicting classifications of pathogenicity
rs250935704517:66,511,534G/Auncertain significance
rs250935705317:66,511,535A/Guncertain significance
rs250935706217:66,511,536G/Auncertain significance
rs250935708417:66,511,539C/Auncertain significance
rs28186477917:66,511,541A/Gmissense variantpathogenic
rs250935713817:66,511,543G/Alikely pathogenic
rs160046176217:66,511,544G/Cuncertain significance
rs148844495817:66,511,547T/Cuncertain significance
rs75264340917:66,511,548C/Tuncertain significance
rs214314848717:66,511,550G/Tuncertain significance
rs214314854417:66,511,552C/Alikely benign
rs160046178917:66,511,553A/Cuncertain significance
rs214314863317:66,511,555T/Clikely benign
rs75597892617:66,511,558C/Tlikely benign
rs36921064617:66,511,559G/Aconflicting classifications of pathogenicity
rs75356509317:66,511,561C/Alikely benign
rs122429748617:66,511,562G/Auncertain significance
rs208540022617:66,511,563C/Auncertain significance
rs56115862917:66,511,564C/Tlikely benign
rs77846862617:66,511,565A/Guncertain significance
rs74539169217:66,511,567T/Guncertain significance
rs146788654417:66,511,568G/Auncertain significance
rs208540070017:66,511,573G/Cuncertain significance
rs129213370317:66,511,575C/Guncertain significance
rs117767463717:66,511,577C/Tuncertain significance
rs214314954417:66,511,578G/Auncertain significance
rs160046192117:66,511,579C/Tlikely benign
rs250935763017:66,511,580A/Guncertain significance
rs214314965817:66,511,582C/Tlikely benign
rs214314970717:66,511,583C/Auncertain significance
rs250935768517:66,511,584T/Cuncertain significance
rs88604122817:66,511,586C/Tstop gainedpathogenic
rs208540121817:66,511,587G/Auncertain significance
rs77151858117:66,511,591A/Tuncertain significance
rs155581169017:66,511,594T/Clikely benign
rs141808054617:66,511,597G/Alikely benign
rs250935785717:66,511,598C/Auncertain significance
rs160046198917:66,511,599T/Guncertain significance
rs37245186217:66,511,600C/Glikely benign
rs214315029517:66,511,602A/Guncertain significance
rs77711046417:66,511,603C/Tlikely benign
rs87885456117:66,511,604G/Tuncertain significance
rs208540215117:66,511,605T/Guncertain significance
rs76315837217:66,511,611A/Guncertain significance
rs138974057217:66,511,612G/Alikely benign
rs14079578717:66,511,613C/Tuncertain significance
rs214315073217:66,511,615T/Guncertain significance
rs77427742817:66,511,616A/Guncertain significance
rs214315078717:66,511,617A/Cuncertain significance
rs214315081417:66,511,618C/Tlikely benign
rs250935816417:66,511,619A/Guncertain significance
rs123531738617:66,511,621T/Guncertain significance
rs28186478017:66,511,622C/Gmissense variantuncertain significance
rs160046219117:66,511,623A/Cuncertain significance
rs214315100917:66,511,624A/Glikely benign
rs76072694117:66,511,625G/Tuncertain significance

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

Gene information from NCBI Gene. Variant classifications from ClinVar.