cAMP-dependent protein kinase type I-alpha regulatory subunit is an enzyme

Enzyme

Enzymes are proteins that catalyze chemical reactions. In enzymatic reactions, the molecules at the beginning of the process, called substrates, are converted into different molecules, called products. Almost all chemical reactions in a biological cell need enzymes in order to occur at rates...

 that in humans is encoded by the PRKAR1A gene

Gene

A gene is a molecular unit of heredity of a living organism. It is a name given to some stretches of DNA and RNA that code for a type of protein or for an RNA chain that has a function in the organism. Living beings depend on genes, as they specify all proteins and functional RNA chains...

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Function

cAMP

Cyclic adenosine monophosphate

Cyclic adenosine monophosphate is a second messenger important in many biological processes...

 is a signaling molecule important for a variety of cellular functions. cAMP exerts its effects by activating the cAMP-dependent protein kinase A (PKA), which transduces the signal through phosphorylation

Phosphorylation

Phosphorylation is the addition of a phosphate group to a protein or other organic molecule. Phosphorylation activates or deactivates many protein enzymes....

 of different target proteins. The inactive holoenzyme of PKA 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 of PKA have been identified in humans. The protein encoded by this gene is 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 Three alternatively spliced transcript variants encoding the same protein have been observed.

Clinical significance

Functional null mutations in this gene cause Carney complex

Carney complex

Carney complex, also known as LAMB syndrome and NAME syndrome is an autosomal dominant condition comprising myxomas of the heart and skin, hyperpigmentation of the skin , and endocrine overactivity It is distinct from Carney's triad...

 (CNC), an autosomal dominant multiple neoplasia syndrome. This gene can fuse to the RET protooncogene by gene rearrangement and form the thyroid tumor-specific chimeric oncogene known as PTC2.

Mutation of PRKAR1A leads to the Carney complex, associating multiple endocrine tumors.

Interactions

PRKAR1A has been shown to interact

Protein-protein interaction

Protein–protein interactions occur when two or more proteins bind together, often to carry out their biological function. Many of the most important molecular processes in the cell such as DNA replication are carried out by large molecular machines that are built from a large number of protein...

 with AKAP1

AKAP1

A kinase anchor protein 1, mitochondrial is an enzyme that in humans is encoded by the AKAP1 gene.-Interactions:AKAP1 has been shown to interact with MYCBP, C3orf15, PRKAR1A, PRKAR2A and PRKAR1B.-Further reading:...

, AKAP10

AKAP10

A kinase anchor protein 10, mitochondrial is an enzyme that in humans is encoded by the AKAP10 gene.-Interactions:AKAP10 has been shown to interact with PDZK1 and PRKAR1A.-Further reading:...

, ARFGEF1

ARFGEF1

Brefeldin A-inhibited guanine nucleotide-exchange protein 1 is a protein that in humans is encoded by the ARFGEF1 gene.-Interactions:ARFGEF1 has been shown to interact with FKBP2, ARFGEF2 and PRKAR1A.-Further reading:...

, ARFGEF2

ARFGEF2

Brefeldin A-inhibited guanine nucleotide-exchange protein 2 is a protein that in humans is encoded by the ARFGEF2 gene.-Interactions:ARFGEF2 has been shown to interact with ARFGEF1, PRKAR1A and PRKAR2A.-Further reading:...

, UBE2M

UBE2M

NEDD8-conjugating enzyme Ubc12 is a protein that in humans is encoded by the UBE2M gene.-Interactions:UBE2M has been shown to interact with NEDD8, PRKAR1A and UBA3.-Further reading:...

, AKAP4

AKAP4

A-kinase anchor protein 4 is an enzyme that in humans is encoded by the AKAP4 gene.-Interactions:AKAP4 has been shown to interact with PRKAR1A and AKAP3.-Further reading:...

, Grb2

Grb2

Growth factor receptor-bound protein 2 also known as Grb2 is an adaptor protein involved in signal transduction/cell communication. In humans, the GRB2 protein is encoded by the GRB2 gene....

, PRKAR1B

PRKAR1B

cAMP-dependent protein kinase type I-beta regulatory subunit is an enzyme that in humans is encoded by the PRKAR1B gene.-Interactions:PRKAR1B has been shown to interact with AKAP1 and PRKAR1A.-Further reading:...

 and MYO7A

MYO7A

Myosin VIIA is protein that in humans is encoded by the MYO7A gene. Myosin VIIA is a member of the unconventional myosin superfamily of proteins...

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Further reading