ID:KAP0_HUMAN DESCRIPTION: RecName: Full=cAMP-dependent protein kinase type I-alpha regulatory subunit; AltName: Full=Tissue-specific extinguisher 1; Short=TSE1; FUNCTION: Regulatory subunit of the cAMP-dependent protein kinases involved in cAMP signaling in cells. SUBUNIT: The inactive holoenzyme is composed of two regulatory chains and two catalytic chains. Activation by cAMP releases the two active catalytic monomers and the regulatory dimer. PRKAR1A also interacts with RFC2; the complex may be involved in cell survival. Interacts with AKAP4. Interacts with RARA; the interaction occurs in the presence of cAMP or FSH and regulates RARA transcriptional activity. Interacts with the phosphorylated form of PJA2. Interacts with CBFA2T3 (By similarity). Interacts with PRKX; regulates this cAMP-dependent protein kinase. INTERACTION: Q9H0R8:GABARAPL1; NbExp=2; IntAct=EBI-476431, EBI-746969; Q9H8W4:PLEKHF2; NbExp=3; IntAct=EBI-476431, EBI-742388; P51817:PRKX; NbExp=2; IntAct=EBI-476431, EBI-4302903; P35250:RFC2; NbExp=7; IntAct=EBI-476431, EBI-476409; Q01105:SET; NbExp=2; IntAct=EBI-476431, EBI-1053182; TISSUE SPECIFICITY: Four types of regulatory chains are found: I- alpha, I-beta, II-alpha, and II-beta. Their expression varies among tissues and is in some cases constitutive and in others inducible. PTM: The pseudophosphorylation site binds to the substrate-binding region of the catalytic chain, resulting in the inhibition of its activity. DISEASE: Defects in PRKAR1A are the cause of Carney complex type 1 (CNC1) [MIM:160980]. CNC is a multiple neoplasia syndrome characterized by spotty skin pigmentation, cardiac and other myxomas, endocrine tumors, and psammomatous melanotic schwannomas. DISEASE: Defects in PRKAR1A are the cause of intracardiac myxoma (INTMYX) [MIM:255960]. Inheritance is autosomal recessive. DISEASE: Defects in PRKAR1A are the cause of primary pigmented nodular adrenocortical disease type 1 (PPNAD1) [MIM:610489]. Primary pigmented nodular adrenocortical disease is a rare bilateral adrenal defect causing ACTH-independent Cushing syndrome. Macroscopic appearance of the adrenals is characteristic with small pigmented micronodules observed in the cortex. PPNAD1 is most often diagnosed in patients with Carney complex, but it can also be observed in patients without other manifestations or familial history. DISEASE: Defects in PRKAR1A are the cause of acrodysostosis type 1, with or without hormone resistance (ACRDYS1) [MIM:101800]. ACRDYS1 is a form of skeletal dysplasia characterized by short stature, severe brachydactyly, facial dysostosis, and nasal hypoplasia. Affected individuals often have advanced bone age and obesity. Laboratory studies show resistance to multiple hormones, including parathyroid, thyrotropin, calcitonin, growth hormone- releasing hormone, and gonadotropin. However, not all patients show endocrine abnormalities. SIMILARITY: Belongs to the cAMP-dependent kinase regulatory chain family. SIMILARITY: Contains 2 cyclic nucleotide-binding domains. WEB RESOURCE: Name=Atlas of Genetics and Cytogenetics in Oncology and Haematology; URL="http://atlasgeneticsoncology.org/Genes/PRKAR1AID387.html"; WEB RESOURCE: Name=GeneReviews; URL="http://www.ncbi.nlm.nih.gov/sites/GeneTests/lab/gene/PRKAR1A";
The RNAfold program from the Vienna RNA Package is used to perform the secondary structure predictions and folding calculations. The estimated folding energy is in kcal/mol. The more negative the energy, the more secondary structure the RNA is likely to have.
ModBase Predicted Comparative 3D Structure on P10644
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Orthologous Genes in Other Species
Orthologies between human, mouse, and rat are computed by taking the best BLASTP hit, and filtering out non-syntenic hits. For more distant species reciprocal-best BLASTP hits are used. Note that the absence of an ortholog in the table below may reflect incomplete annotations in the other species rather than a true absence of the orthologous gene.
Gene Ontology (GO) Annotations with Structured Vocabulary
Molecular Function: GO:0000166 nucleotide binding GO:0004862 cAMP-dependent protein kinase inhibitor activity GO:0005515 protein binding GO:0008603 cAMP-dependent protein kinase regulator activity GO:0019904 protein domain specific binding GO:0030552 cAMP binding GO:0031625 ubiquitin protein ligase binding GO:0034236 protein kinase A catalytic subunit binding
Biological Process: GO:0001707 mesoderm formation GO:0001932 regulation of protein phosphorylation GO:0003091 renal water homeostasis GO:0006357 regulation of transcription from RNA polymerase II promoter GO:0006469 negative regulation of protein kinase activity GO:0007143 female meiotic division GO:0007507 heart development GO:0007596 blood coagulation GO:0034199 activation of protein kinase A activity GO:0035556 intracellular signal transduction GO:0045214 sarcomere organization GO:0045835 negative regulation of meiotic nuclear division GO:0045859 regulation of protein kinase activity GO:0046007 negative regulation of activated T cell proliferation GO:0060038 cardiac muscle cell proliferation GO:0071377 cellular response to glucagon stimulus GO:2000480 negative regulation of cAMP-dependent protein kinase activity
LP895348 - Sequence 212 from Patent EP3253886. Y07642 - H.sapiens mRNA for cAMP-dependent protein kinase regulatory subunit RIalpha. BC093042 - Homo sapiens protein kinase, cAMP-dependent, regulatory, type I, alpha (tissue specific extinguisher 1), mRNA (cDNA clone MGC:111057 IMAGE:6514677), complete cds. BC036285 - Homo sapiens protein kinase, cAMP-dependent, regulatory, type I, alpha (tissue specific extinguisher 1), mRNA (cDNA clone MGC:17251 IMAGE:4340015), complete cds. GQ901048 - Homo sapiens clone HEL-T-160 epididymis secretory sperm binding protein mRNA, complete cds. AH003751 - Homo sapiens TSE1=protein kinase A regulatory subunit gene [human, mRNA Partial, 1033 nt 4 segments]. AK312307 - Homo sapiens cDNA, FLJ92612, Homo sapiens protein kinase, cAMP-dependent, regulatory, type I, alpha (tissue specific extinguisher 1) (PRKAR1A), mRNA. CR749311 - Homo sapiens mRNA; cDNA DKFZp779L0468 (from clone DKFZp779L0468). AK124586 - Homo sapiens cDNA FLJ42595 fis, clone BRACE3010221, highly similar to cAMP-dependent protein kinase type I-alpha regulatory chain. A12295 - R1 alpha cDNA sequence. M18468 - Human cAMP-dependent protein kinase regulatory subunit type I (PRKAR1A) mRNA, complete cds. M33336 - Human cAMP-dependent protein kinase type I-alpha subunit (PRKAR1A) mRNA, complete cds. JD424991 - Sequence 406015 from Patent EP1572962. JD409391 - Sequence 390415 from Patent EP1572962. JD458566 - Sequence 439590 from Patent EP1572962. JD193140 - Sequence 174164 from Patent EP1572962. JD139859 - Sequence 120883 from Patent EP1572962. JD405843 - Sequence 386867 from Patent EP1572962. AK097580 - Homo sapiens cDNA FLJ40261 fis, clone TESTI2025609, highly similar to cAMP-dependent protein kinase type I-alpha regulatory subunit. EF428111 - Homo sapiens protein kinase A regulatory subunit type I/alpha-retinoic acid receptor alpha fusion protein isoform b (PRKAR1A/RARA fusion) mRNA, complete cds. EF428110 - Homo sapiens protein kinase A regulatory subunit type I/alpha-retinoic acid receptor alpha fusion protein isoform a (PRKAR1A/RARA fusion) mRNA, partial cds. DQ891775 - Synthetic construct clone IMAGE:100004405; FLH179817.01X; RZPDo839C01132D protein kinase, cAMP-dependent, regulatory, type I, alpha (tissue specific extinguisher 1) (PRKAR1A) gene, encodes complete protein. DQ894959 - Synthetic construct Homo sapiens clone IMAGE:100009419; FLH179813.01L; RZPDo839C01131D protein kinase, cAMP-dependent, regulatory, type I, alpha (tissue specific extinguisher 1) (PRKAR1A) gene, encodes complete protein. AB384780 - Synthetic construct DNA, clone: pF1KB3304, Homo sapiens PRKAR1A gene for cAMP-dependent protein kinase type I-alpha regulatory subunit, complete cds, without stop codon, in Flexi system. KJ891886 - Synthetic construct Homo sapiens clone ccsbBroadEn_01280 PRKAR1A gene, encodes complete protein. KJ905284 - Synthetic construct Homo sapiens clone ccsbBroadEn_14784 PRKAR1A gene, encodes complete protein. KR709580 - Synthetic construct Homo sapiens clone CCSBHm_00003651 PRKAR1A (PRKAR1A) mRNA, encodes complete protein. KR709581 - Synthetic construct Homo sapiens clone CCSBHm_00003656 PRKAR1A (PRKAR1A) mRNA, encodes complete protein. KR709582 - Synthetic construct Homo sapiens clone CCSBHm_00003662 PRKAR1A (PRKAR1A) mRNA, encodes complete protein. KR709583 - Synthetic construct Homo sapiens clone CCSBHm_00003668 PRKAR1A (PRKAR1A) mRNA, encodes complete protein. JD301172 - Sequence 282196 from Patent EP1572962. JD460282 - Sequence 441306 from Patent EP1572962. JD127393 - Sequence 108417 from Patent EP1572962. JD060499 - Sequence 41523 from Patent EP1572962. JD313592 - Sequence 294616 from Patent EP1572962. JD080993 - Sequence 62017 from Patent EP1572962. JD186542 - Sequence 167566 from Patent EP1572962. JD447807 - Sequence 428831 from Patent EP1572962. JD243012 - Sequence 224036 from Patent EP1572962. JD552394 - Sequence 533418 from Patent EP1572962. JD562772 - Sequence 543796 from Patent EP1572962. JD268296 - Sequence 249320 from Patent EP1572962. JD455438 - Sequence 436462 from Patent EP1572962. JD083040 - Sequence 64064 from Patent EP1572962. AL050038 - Homo sapiens mRNA; cDNA DKFZp566J0124 (from clone DKFZp566J0124). JD350567 - Sequence 331591 from Patent EP1572962. JD308526 - Sequence 289550 from Patent EP1572962. AY007115 - Homo sapiens clone TCCCTA00211 mRNA sequence. JD297201 - Sequence 278225 from Patent EP1572962.
Biochemical and Signaling Pathways
BioCarta from NCI Cancer Genome Anatomy Project h_igf1rPathway - Multiple antiapoptotic pathways from IGF-1R signaling lead to BAD phosphorylation h_nfatPathway - NFAT and Hypertrophy of the heart (Transcription in the broken heart) h_shhPathway - Sonic Hedgehog (Shh) Pathway h_vipPathway - Neuropeptides VIP and PACAP inhibit the apoptosis of activated T cells h_CSKPathway - Activation of Csk by cAMP-dependent Protein Kinase Inhibits Signaling through the T Cell Receptor h_GATA3pathway - GATA3 participate in activating the Th2 cytokine genes expression h_chrebpPathway - ChREBP regulation by carbohydrates and cAMP h_crebPathway - Transcription factor CREB and its extracellular signals h_agpcrPathway - Attenuation of GPCR Signaling h_badPathway - Regulation of BAD phosphorylation h_dreampathway - Repression of Pain Sensation by the Transcriptional Regulator DREAM h_gpcrPathway - Signaling Pathway from G-Protein Families h_mPRPathway - How Progesterone Initiates the Oocyte Maturation h_nos1Pathway - Nitric Oxide Signaling Pathway h_pparaPathway - Mechanism of Gene Regulation by Peroxisome Proliferators via PPARa(alpha) h_gsPathway - Activation of cAMP-dependent protein kinase, PKA h_mCalpainPathway - mCalpain and friends in Cell motility h_no1Pathway - Actions of Nitric Oxide in the Heart h_cftrPathway - Cystic fibrosis transmembrane conductance regulator (CFTR) and beta 2 adrenergic receptor (b2AR) pathway h_ck1Pathway - Regulation of ck1/cdk5 by type 1 glutamate receptors h_plcePathway - Phospholipase C-epsilon pathway h_stathminPathway - Stathmin and breast cancer resistance to antimicrotubule agents h_carm1Pathway - Transcription Regulation by Methyltransferase of CARM1
Reactome (by CSHL, EBI, and GO)
Protein P10644 (Reactome details) participates in the following event(s):
US Server
Sequence and Links to Tools and Databases 
Common Gene Haplotype Alleles 
