PRKAR1A Antibody

Datasheet
Code CSB-PA018694GA01HU
Size US$685
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Product Details

Uniprot No. P10644
Target Names PRKAR1A
Alternative Names ACRDYS1 antibody; ADOHR antibody; cAMP dependent protein kinase regulatory subunit alpha 1 antibody; cAMP dependent protein kinase regulatory subunit RIalpha antibody; cAMP dependent protein kinase type I alpha regulatory chain antibody; cAMP dependent protein kinase type I alpha regulatory subunit antibody; cAMP-dependent protein kinase type I-alpha regulatory subunit antibody; CAR antibody; Carney complex type 1 antibody; CNC 1 antibody; CNC antibody; CNC1 antibody; DKFZp779L0468 antibody; KAP0_HUMAN antibody; MGC17251 antibody; PKA RIA antibody; PKR 1 antibody; PKR1 antibody; PPNAD 1 antibody; PPNAD1 antibody; PRKAR 1 antibody; PRKAR1 antibody; PRKAR1A antibody; PRKAR1A/RARA FUSION GENE antibody; Protein kinase A type 1a regulatory subunit antibody; Protein kinase cAMP dependent regulatory type I alpha antibody; PTC2 CHIMERIC ONCOGENE; INCLUDED antibody; Tissue specific extinguisher 1 antibody; Tissue-specific extinguisher 1 antibody; TSE 1 antibody; TSE1 antibody
Raised in Rabbit
Species Reactivity Human,Mouse,Rat
Immunogen Human PRKAR1A
Immunogen Species Homo sapiens (Human)
Isotype IgG
Purification Method Antigen Affinity purified
Concentration It differs from different batches. Please contact us to confirm it.
Buffer PBS with 0.02% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
Tested Applications ELISA,WB
Protocols ELISA Protocol
Western Blotting(WB) Protocol
Troubleshooting and FAQs Antibody FAQs
Storage Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

Target Data

Function Regulatory subunit of the cAMP-dependent protein kinases involved in cAMP signaling in cells.
Gene References into Functions
  1. In Pseudohypoparathyroidism, mutations were found in PRKAR1A, PDE4D, TRPS1, and PTHLH. PMID: 29499646
  2. A report of the occurrence of an aberrant serotonergic regulatory loop in primary pigmented nodular adrenocortical disease tissues from patients with PRKAR1A mutations. PMID: 27699247
  3. Splice site mutation of the PRKAR1A gene is associated with Carney complex. PMID: 29318463
  4. PRKAR1A is a potent tumor suppressor that inhibits the ERK/Snail/E-cadherin pathway in lung adenocarcinoma PMID: 27995993
  5. t-Darpp phosphorylation at T39 seems to be crucial for t-Darpp-mediated PKA activation and this activation appears to occur through an association with RI and sequestering of RI away from PKAc. The t-Darpp-RI interaction could be a druggable target to reduce PKA activity in drug-resistant cancer. PMID: 28867659
  6. the T allele of SNP rs60684937 located at 67,419,130 bp on chromosome 17 was associated with increased plasma EPO and a relatively increased expression of a non-coding transcript of PRKAR1A in sickle cell disease patients PMID: 28173069
  7. In this study we demonstrate a new role of MRAP2 in the regulation of the orexin receptor 1 (OX1R) and identify the specific regions of MRAP2 required for the regulation of OX1R and PKR1. Importantly, like MC4R and PKRs, OX1R is predominately expressed in the brain where it regulates food intake PMID: 28939058
  8. Screening of PRKAR1A and PDE4D in a Large Italian Series of Patients Clinically Diagnosed With Albright Hereditary Osteodystrophy and/or Pseudohypoparathyroidism PMID: 26763073
  9. we used next-generation sequencing to assess 480 cancer-related genes and performed immunohistochemistry on 13 cases morphologically consistent with pigmented epithelioid melanocytoma. These results further validate the concept of pigmented epithelioid melanocytoma as a distinctive intermediate/borderline melanocytic tumor, and also illustrate its molecular heterogeneity PMID: 28796000
  10. Found evidence for kidney and liver cystic phenotypes in the Carney complex, a tumoral syndrome caused by mutations in PRKAR1A. PMID: 28615245
  11. Data suggest that introduction of cGMP-specific residues using site-directed mutagenesis reduces selectivity of cyclic nucleotide-binding domain (CNBD) of PRKAR1A; combination of two mutations (G316R/A336T) results in a cGMP-selective binding site in the C-terminal CNBD; introduction of corresponding mutations (T192R/A212T) into the N-terminal CNBD results in a highly cGMP-selective binding site. PMID: 28583991
  12. Data show that ELOVL7, SOCS3, ACSL4 and CLU were upregulated while PRKAR1A and ABCG1 were downregulated in the phlegm-dampness group. PMID: 27928700
  13. Electrostatic interactions are mediators in the allosteric activation of protein kinase A RIalpha. PMID: 28221775
  14. The obtained data on the mechanism of cAMP-dependent activation of PKA I alpha may contribute to new approaches to designing pharmaceuticals based on cAMP analogs. PMID: 28367443
  15. the present study reported for the first time an intronic splice site mutation in the PRKAR1A gene of a Chinese family with Carney complex, which probably caused skin pigmentation observed in affected family members. PMID: 26788925
  16. This study reports a novel point mutation of the PRKAR1A gene in a patient with Carney complex who presented with significant osteoporosis and fractures. PMID: 27377598
  17. Letter/Case Report: novel PRKAR1A mutation resulting in a splicing variant in a case of Carney complex. PMID: 26354069
  18. P-Rex1 contributes to the spatiotemporal localization of type I PKA, which tightly regulates this guanine exchange factor by a multistep mechanism. PMID: 26797121
  19. In the absence of a PRKAR1A gene mutation, our Cushing's syndrome patients do not fit the criteria for Carney's complex PMID: 26619967
  20. Case Report: although there was no family history of any of the Carney complex features and no mutations in the PRKAR1A gene were found, findings lead to the diagnosis of sporadic Carney complex. PMID: 25576349
  21. this study evaluated the functional characteristics of PRKAR1A regulatory subunits carrying eight different mutations identified in patients with acrodysostosis and compared the results with those obtained for the two alternative mutations involved in the Carney complex PMID: 26405036
  22. PRKAR1A gene and its locus are altered in mixed odontogenic tumors. Expression is decreased in a subset of tumors, and Prkar1a(+) (/) (-) mice do not show abnormalities, which indicates that additional genes play a role in this tumor's pathogenesis. PMID: 25870248
  23. The truncated enzyme lacks the functional cyclic adenosine monophosphate (cAMP) binding domain at the C-terminus, causing PRKAR1A haploinsufficiency. PMID: 26416542
  24. PRKAR1A gene mutation of c.491_492delTG is associated with multiple and extensive cardiac myxomas and skin pigmentation. PMID: 25890363
  25. our data shows that an increased PRKAR1A expression is associated with aggressive and undifferentiated thyroid tumors PMID: 25393625
  26. Protein Kinase A Opposes the Phosphorylation-dependent Recruitment of Glycogen Synthase Kinase 3beta to A-kinase Anchoring Protein 220. PMID: 26088133
  27. Although the depletion of PRKAR1A and PRKAR2B in adrenocortical cells has similar effects on cell proliferation and apoptosis; loss of these PKA subunits differentially affects cyclin expression. PMID: 25268545
  28. Results show that mouse Prkar1a and human PRKAR2A exhibited a dynamic spatio-temporal expression in tooth development, whereas neither human PRKAR1A nor mouse Prkar2a showed their expression in odontogenesis. PMID: 24755349
  29. A Carney complex-related pituitary adenoma with a somatic mutation and a large inherited deletion of the PRKAR1A gene was identified. PMID: 25336503
  30. we conclude that PRKAR1A mutations may play no major role in the pathogenesis of BDE. PMID: 23425300
  31. These results show that RIalpha inactivation leads to multiple, compartment-specific alterations of the cAMP/PKA pathway revealing new aspects of signaling dysregulation in tumorigenesis. PMID: 24122441
  32. PRKAR1A appears to play a role in the development of both syndromic and nonsyndromic cardiac myxomas PMID: 24618615
  33. Mutagenesis of residues on Protein Kinase A RIalpha interface not only leads to structural and biochemical changes, but is also linked to Carney complex disease. PMID: 24316401
  34. Increased PKA signaling in perirenal adipose tissue was associated with lower BMI in Cushing syndrome. Differences in fat distribution may contribute to phenotypic differences between patients with Cushing syndrome with and without PRKAR1A mutations. PMID: 24248186
  35. A significant number (21.6%) of patients with Carney complex that are negative in currently available testing may have PRKAR1A haploinsufficiency due to genomic defects that are not detected by Sanger sequencing. PMID: 24170103
  36. Data suggest that gene rearrangement in PRKAR1A can result in Carney complex; a large in-frame deletion of exons 3-6 has been identified in three members of a Portuguese family; the truncated PRKAR1A produced in these subjects is unstable. PMID: 24144965
  37. Data suggest that protein kinase A enzymatic activity in parathyroid adenoma is less than in normal glands; however, 5 out of 8 parathyroid adenoma tissues expressed higher content of PKA-RIalpha protein as compared with normal parathyroid tissues. PMID: 23197043
  38. Evidence for a new mechanism by which hypercapnia via soluble adenylyl cyclase, cAMP, PKA Type Ialpha, and alpha-adducin regulates Na,K-ATPase endocytosis in alveolar epithelial cells. PMID: 23349050
  39. REVIEW: Prkar1a in the regulation of insulin secretion PMID: 22951902
  40. RIaalpha and RIIaalpha were identified as cCMP-binding proteins. PMID: 22808067
  41. Phenotypic differences, including the presence of resistance to GPCR-cAMP signaling hormones in PRKAR1A but not PDE4D patients, indicate phenotype-genotype correlations and highlight the specific contributions of PRKAR1A and PDE4D in cAMP signaling. PMID: 23043190
  42. Epithelioid and fusiform blue nevus of chronically sun-damaged skin is a unique subtype of blue nevus without loss of PRKAR1A expression. PMID: 22892599
  43. PRKAR1A overexpression is associated with increased ECPKA autoantibody in liver fluke-associated cholangiocarcinoma. PMID: 22922884
  44. PRKAR1A mutational analysis and PKA enzymatic activity in endometrial tumors. PMID: 22461635
  45. PRKAR1A mutation is associated with Carney complex. PMID: 22297707
  46. We treated a 40-year-old man who presented with an embolic stroke. He had a family history of cardiac myxoma and underwent resection of a right atrial myxoma 7 years ago. Genetic evaluation revealed a heterozygous mutation in the PRKAR1A gene. PMID: 22632512
  47. Protein kinase A 1 selectively regulates Kv1.3 channels in human T lymphocytes. PMID: 22378744
  48. This was the first demonstration of proteasomal degradation of RIalpha protein variants leading to PRKAR1A haploinsufficiency and Carney complex, adding protein surveillance to nonsense mRNA decay in the cellular mechanisms overseeing RIalpha synthesis. PMID: 22205709
  49. Data suggest that not all cAMP activation is the same; adrenal lesions harboring PRKAR1A or GNAS mutations share downstream activation of certain oncogenic signals (such as MAPK and cell cycle genes) but differ substantially in effects on others. PMID: 22259056
  50. A novel PRKAR1A mutation in Korean Carney complex family. PMID: 22020668
  51. In a large family with Carney complex, one amino acid substitution caused a spectrum of adrenal disease that ranged from lack of manifestations to cancer. PMID: 22112814
  52. Variations in expression of Prkar1a gene transcripts suggest their regulation from multiple promoters that respond to a variety of signals arising in or out of the cell in tissue and developmental stage-specific manner. PMID: 21638026
  53. PRKAR1A could function as a tumor suppressor gene in pancreatic tissue, at least in the context of Carney complex. PMID: 21900385
  54. abrogation of gene expression caused significant CCA cell growth inhibition, oncogenic signaling and coupled apoptosis induction, suggesting PRKAR1A's potential as a drug target for CCA therapy PMID: 20824711
  55. Thermal denaturation of RIalpha leads to partial loss of native packing with exposure of aggregation-prone motifs. PMID: 21394209
  56. We report a germ-line mutation in the gene encoding PRKAR1A, the cyclic AMP (cAMP)-dependent regulatory subunit of protein kinase A, in three unrelated patients with acrodysostosis and resistance to multiple hormones. PMID: 21651393
  57. c.349 G>T, a novel splice site germline PRKAR1A defect, has low penetrance resulting in incomplete clinical expression of Primary pigmented nodular adrenocortical disease PMID: 20190548
  58. A comprehensive analysis of all presently known PRKAR1A sequence variations is presented and their molecular context and clinical phenotype, is discussed. PMID: 20358582
  59. A novel mutation in the PRKAR1A gene in a family with Carney complex and multiple members with primary pigmented nodular adrenocortical disease. PMID: 19833579
  60. Includes the study of a disease-related upstream ORF in this gene, and shows that it functions to reduce protein levels by ~95%. PMID: 19372376
  61. Data show that RSK1 regulates PKAc activity in a cAMP-independent manner, and PKARIalpha by associating with RSK1 regulates its activation and its biological functions. PMID: 20048145
  62. Leukemic transformation by acute promyelocytic leuckemia fusion protein PRKAR1A-RARalpha is critically dependent on RXRalpha. PMID: 19965660
  63. these results showed a potentially novel mechanism of cAMP signaling mediated through the interaction of RIalpha with PATZ1. PMID: 20026299
  64. Investigated the presence of activating missense mutations in the GNAS1 gene as well as of inactivating mutations in PRKAR1A in 29 sporadically occurring cardiac myxomas. PMID: 19494712
  65. Association of the M1V PRKAR1A mutation with primary pigmented nodular adrenocortical disease in two large families. PMID: 19915019
  66. Formation of inactive cAMP-saturated holoenzyme of cAMP-dependent protein kinase under physiological conditions PMID: 11834733
  67. Protein kinase A type I: a target for cancer therapy PMID: 11839645
  68. mutations in the gene coding for PRKAR1A in Carney complex - review PMID: 12119264
  69. The regulatory subunit type I-alpha of protein kinase A functions as a thyroid cancer tumor-suppressor gene. PMID: 12203783
  70. germline and somatic mutations of the PRKAR1A gene in Cushing's syndrome caused by sporadic primary pigmented nodular adrenocortical disease PMID: 12213893
  71. Patients with Carney complex and primary pigmented nodular adrenocortical disease (PPNAD) reveals novel mutations and clues for pathophysiology: augmented PKA signaling is associated with adrenal tumorigenesis in PPNAD PMID: 12424709
  72. PRKAR1A, one of the Carney complex genes, and its locus (17q22-24) are rarely altered in pituitary tumours outside the Carney complex PMID: 12471216
  73. RIalpha and RIbeta homodimers as well as an RIalpha:RIbeta heterodimer and several of the mutants were able to bind to the R-binding domain of AKAP149/D-AKAP1 PMID: 12634056
  74. (PRKAR1 alpha) has been discovered and found to be associated with a high risk of developing cardiac myxomas. PMID: 12752185
  75. Reduced IL-10 secretion in common variable immunodeficiency patients seems to involve the cAMP-dependent protein kinase A type I system. PMID: 12759461
  76. PRKAR1A activity in Carney complex cells is increased and it has a role in phosphorylation, cell metabolism and proliferation PMID: 12812976
  77. PKA-RIbeta binds to merlin and may be involved in signaling with merlin in the cytoskeleton PMID: 12896975
  78. PKA regulates ethanol-induced cAMP response element-mediated gene expression via activation of CREB-binding protein and inhibition of MAPK PMID: 15299023
  79. PRKAR1A might function as a tumor suppressor gene PMID: 15331577
  80. concluded that although PRKAR1A haploinsufficiency does predispose to tumorigenesis, distinct secondary genetic events are required for tumor formation PMID: 15371594
  81. Low expression of R1A protein may favor cAMP-dependent proliferation of transformed somatotrophs. PMID: 15604292
  82. evidence is presented for a novel interaction between RFC40 and the RIalpha subunit of PKA; findings indicate that modulation in the formation of the RFC40-RIa complex is associated with decreased cell survival PMID: 15655353
  83. Mutation of the PRKAR1A gene may be associated with familial cardiac myxoma in Carney complex but may not be associated with sporadic cardiac myxoma. PMID: 15982496
  84. Molecular genetic analysis demonstrated involvement of a mutation in the PRKAR1alpha gene. PMID: 16020889
  85. switching of PKA isozyme can cause tumor cells to undergo phenotypic reversion of the malignancy [revoew] PMID: 16394127
  86. Described simulations shed light on the mechanism by which PKA regulatory subunit RI alpha undergoes its dramatic conformational change upon binding to the C subunit. PMID: 16407073
  87. cAMP signaling control is achieved by A-kinase anchoring proteins including type I PKA holoenzyme PMID: 16728392
  88. Epac1 and cAMP-dependent protein kinase holoenzyme have similar cAMP affinity, but different cAMP domains PMID: 16728394
  89. Haploinsufficiency at the protein kinase A RI alpha gene locus leads to fertility defects in male mice and men. PMID: 16728532
  90. Phosphorylated-mTOR levels and mTOR activity were dramatically increased in HEK 293 cells with RIalpha levels reduced by siRNA. PMID: 16963469
  91. Role of mutations in in primary pigmented nodular adrenocortical disease (Review) PMID: 17036196
  92. Reviews genetic aspects of hereditary GH-secreting tumors and data from animal models resulting from the inactivation of the PRKAR1A tumor suppressor gene. PMID: 17047380
  93. human cells with partially inactivated RIalpha levels have increased proliferation and survival. PMID: 17079485
  94. A high R1/R2 ratio favors the proliferation of well differentiated and hormone producing adrenocortical cells, while unbalanced expression of these subunits is not required for malignant transformation. PMID: 17904549
  95. High expression of PKA regulatory subunit 1A protein is related to proliferation of melanoma PMID: 17906691
  96. first immortalized cell line with naturally occurring PRKAR1A-inactivating mutation associated with tumor formation; data support hypothesis that balance between PKA-I & -II is critical for tumor induction &/or promotion in tissues with PRKAR1A mutations PMID: 18056771
  97. Loss of expression of PRKAR1 offers a useful diagnostic test that helps to distinguish pigmented epithelioid melanocytoma from lesions that mimic it histologically. PMID: 18059235
  98. Large PRKAR1A deletions may be responsible for Carney complex in patients that do not have PRKAR1A gene defects identifiable by sequencing. PMID: 18223213
  99. data further corroborate the previous finding that altered PRKAR1A function, not only haploinsufficiency, is enough to elevate PKA activity which is apparently associated with tumorigenesis in tissues affected by Carney complex PMID: 18241045
  100. BLM deficiency is associated with a relative excess of PRKAR1A in fibroblasts compared to other PKA subunits; PRKAR1A deficiency is associated with increased BLM protein in adrenal hyperplasias. PMID: 18401830

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Involvement in disease Carney complex 1 (CNC1); Intracardiac myxoma (INTMYX); Primary pigmented nodular adrenocortical disease 1 (PPNAD1); Acrodysostosis 1, with or without hormone resistance (ACRDYS1)
Subcellular Location Cell membrane
Protein Families CAMP-dependent kinase regulatory chain family
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.
Database Links

HGNC: 9388

OMIM: 101800

KEGG: hsa:5573

STRING: 9606.ENSP00000351410

UniGene: Hs.280342

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