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    GENE:

    PRKAR1A (Protein Kinase CAMP-Dependent Type I Regulatory Subunit Alpha)

    i
    Other names: PRKAR1A, Protein Kinase CAMP-Dependent Type I Regulatory Subunit Alpha, PRKAR1, CNC1, TSE1, Protein Kinase, CAMP-Dependent, Regulatory Subunit Type I Alpha, CAMP-Dependent Protein Kinase Type I-Alpha Regulatory Subunit, Protein Kinase, CAMP-Dependent, Regulatory, Type I, Alpha, Tissue-Specific Extinguisher 1, Carney Complex Type 1, PKR1, CAMP-Dependent Protein Kinase Type I-Alpha Regulatory Chain, CAMP-Dependent Protein Kinase Regulatory Subunit RIalpha, Protein Kinase A Type 1a Regulatory Subunit, Epididymis Secretory Sperm Binding Protein, Tissue Specific Extinguisher 1, ACRDYS1, PPNAD1, ADOHR, CAR, CNC
    17d
    AI-derived prognostic model identifies high-risk gene signatures in pediatric gliomas. (PubMed, Front Immunol)
    Our findings highlight the potential of AI-driven transcriptomic analysis in improving pediatric glioma prognosis. The identified gene signatures may serve as biomarkers for risk stratification and personalized treatment strategies, advancing precision oncology in pediatric neuro-oncology.
    Journal • Gene Signature
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    PRKAR1A (Protein Kinase CAMP-Dependent Type I Regulatory Subunit Alpha) • CDC25B (Cell Division Cycle 25B) • GNRP (Ras-Specific Guanine Nucleotide-Releasing Factor 1) • NCAPD2 (Non-SMC Condensin I Complex Subunit D2)
    1m
    PRKAR1B as an oncogenic biomarker for diagnostic and prognostic stratification of tumor immunity, proliferation, and migration in head and neck squamous cell carcinoma. (PubMed, Front Immunol)
    Drug sensitivity analysis further suggested that Lapatinib and Erlotinib may be beneficial in HNSC patients with high PRKAR1B expression. Lastly, PRKAR1B protein expression was upregulated in clinical HNSC samples. Overall, this study thoroughly examined PRKAR1B expression and its prognostic significance in HNSC, investigated related molecular pathways and immune cell interactions, and validated its role via in vitro experiments.
    Journal • IO biomarker
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    PRKAR1A (Protein Kinase CAMP-Dependent Type I Regulatory Subunit Alpha)
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    erlotinib • lapatinib
    1m
    Immunohistochemistry of melanocytic tumours (PubMed, Pathologie (Heidelb))
    Cases of malignant peripheral nerve sheath tumours are, in contrast to desmoplastic melanoma S‑100 protein negative or express this marker only focally, and show a decreased nuclear expression of H3K27. Desmoplastic melanoma shows a focal nuclear expression of p53 in contrast to neurofibroma.
    Review • Journal
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    ALK (Anaplastic lymphoma kinase) • TP53 (Tumor protein P53) • ROS1 (Proto-Oncogene Tyrosine-Protein Kinase ROS) • NTRK1 (Neurotrophic tyrosine kinase, receptor, type 1) • NTRK3 (Neurotrophic tyrosine kinase, receptor, type 3) • BAP1 (BRCA1 Associated Protein 1) • CTNNB1 (Catenin (cadherin-associated protein), beta 1) • WT1 (WT1 Transcription Factor) • SOX10 (SRY-Box 10) • CD68 (CD68 Molecule) • PRAME (Preferentially Expressed Antigen In Melanoma) • CDKN1A (Cyclin-dependent kinase inhibitor 1A) • MITF (Melanocyte Inducing Transcription Factor) • NES (Nestin) • PRKAR1A (Protein Kinase CAMP-Dependent Type I Regulatory Subunit Alpha)
    1m
    A urologic manifestation of carney complex: Bilateral Sertoli tumors and scrotal myxoma in an adolescent male. (PubMed, Urol Case Rep)
    We present a case of CNC in an adolescent male with bilateral LCCSCTs and a very large scrotal myxoma, resulting in azoospermia and pain requiring surgical management. This report highlights the urologic manifestations of CNC and outlines appropriate evaluation, treatment, and surveillance strategies relevant to pediatric and adolescent urologic practice.
    Journal
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    PRKAR1A (Protein Kinase CAMP-Dependent Type I Regulatory Subunit Alpha)
    2ms
    HHEX-PRKAR2B axis-mediated PKA activation drives glucose metabolism-dependent progression of pancreatic ductal adenocarcinoma. (PubMed, iScience)
    In vivo, high glucose synergized with PKA activation to promote metastasis, whereas glycolysis inhibition blocked new metastases. Thus, HHEX-PRKAR2B-mediated PKA activation is a critical hub for PDAC progression, modulated by glucose and reinforcing glycolysis via HK2, revealing novel therapeutic targets for metabolic intervention.
    Journal
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    HK2 (Hexokinase 2) • PRKAR1A (Protein Kinase CAMP-Dependent Type I Regulatory Subunit Alpha) • PRKAR2B (Protein Kinase CAMP-Dependent Type II Regulatory Subunit Beta)
    3ms
    Harnessing the death switch: Empowering cancer therapy by modulating the apoptosis-pyroptosis transition. (PubMed, Biomaterials)
    Exploiting this 'death switch' offers a novel therapeutic framework through three principal strategies: (1) inducing pyroptosis to eliminate apoptosis-resistant cells, (2) utilizing pyroptosis-induced inflammation to enhance immune checkpoint inhibitor efficacy, and (3) developing targeted therapeutics that directly modulate these switch molecules. Although controlling pyroptosis-associated inflammation remains challenging, understanding and manipulating the apoptosis-to-pyroptosis transition provides an innovative approach to overcome drug resistance and develop more effective cancer treatments.
    Review • Journal
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    STING (stimulator of interferon response cGAMP interactor 1) • CASP3 (Caspase 3) • CASP8 (Caspase 8) • PRKAR1A (Protein Kinase CAMP-Dependent Type I Regulatory Subunit Alpha) • FOSL2 (FOS Like 2) • GSDME (Gasdermin E) • PPM1A (Protein Phosphatase Mg2+/Mn2+ Dependent 1A) • PPP2CA (Protein Phosphatase 2 Catalytic Subunit Alpha 2)
    3ms
    Case Reports: Exploring the Varied Presentations and Clinical Features of Carney Complex, A Detailed Report on Three Distinct Cases. (PubMed, J Clin Res Pediatr Endocrinol)
    All cases highlight the absence of a consistent genotype-phenotype correlation in CNC, emphasizing the need for individualized management strategies. The findings underscore the complexity of diagnosing and treating CNC, particularly in pediatric populations, and call for further research into the underlying molecular mechanisms to develop more targeted therapies.
    Journal
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    PRKAR1A (Protein Kinase CAMP-Dependent Type I Regulatory Subunit Alpha)
    4ms
    From misclassified AIP variant to carney complex: a case report and retrospective evaluation of PRKAR1A in pituitary tumor predisposition. (PubMed, Pituitary)
    This case underscores the importance of periodically reassessing genetic variants, as reclassification can significantly impact patient management. It also highlights the clinical variability of CNC and the need to screen for CNC features in young patients with acromegaly. Further research is warranted to determine the value ofPRKAR1A testing in isolated GH- and GH/PRL-secreting PA.
    Retrospective data • Journal
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    PRKAR1A (Protein Kinase CAMP-Dependent Type I Regulatory Subunit Alpha)
    4ms
    Glioma angiogenesis phosphoproteome landscape and biomarker sets identified with phenome-centered multiomics toward 3P medical approaches. (PubMed, EPMA J)
    These findings provide concrete molecular targets for antiangiogenic therapy and establish clinically actionable biomarkers for glioma patient stratification in the 3PM framework. The online version contains supplementary material available at 10.1007/s13167-025-00428-1.
    Journal
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    HIF1A (Hypoxia inducible factor 1, alpha subunit) • CAMK2D (Calcium/Calmodulin Dependent Protein Kinase II Delta) • HSPB1 (Heat shock 27kDa protein 1) • L1CAM (L1 cell adhesion molecule) • PRKAR1A (Protein Kinase CAMP-Dependent Type I Regulatory Subunit Alpha) • MYLK (Myosin Light Chain Kinase) • PDHA1 (Pyruvate Dehydrogenase E1 Subunit Alpha 1) • PRKAR2B (Protein Kinase CAMP-Dependent Type II Regulatory Subunit Beta)
    5ms
    Epigenomic characterization and therapeutic challenges of melanoma arising in giant nevi in pediatric patients. (PubMed, Discov Oncol)
    MM arising within CGMN poses diagnostic and therapeutic challenges. While molecular and epigenomic profiling supports accurate classification and understanding of disease biology, the role of immunotherapy remains uncertain-marked by reduced efficacy and significant immune-related toxicity. A multidisciplinary approach is essential to guide management and improve outcomes in this rare pediatric malignancy.
    Journal • PD(L)-1 Biomarker • IO biomarker
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    PD-L1 (Programmed death ligand 1) • BRAF (B-raf proto-oncogene) • NRAS (Neuroblastoma RAS viral oncogene homolog) • PRKAR1A (Protein Kinase CAMP-Dependent Type I Regulatory Subunit Alpha)
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    PD-L1 expression • BRAF mutation • NRAS mutation • NRAS Q61 • BRAF fusion
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    Opdivo (nivolumab) • Yervoy (ipilimumab)
    5ms
    Malignant melanotic nerve sheath tumor mimicking a benign nerve sheath lesion: illustrative case. (PubMed, J Neurosurg Case Lessons)
    MMNST should be considered when encountering suspicious peripheral nerve sheath lesions. Promptly performing appropriate biopsies and molecular testing is critical due to this condition's poor prognosis. https://thejns.org/doi/abs/10.3171/CASE25314.
    Journal
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    PRKAR1A (Protein Kinase CAMP-Dependent Type I Regulatory Subunit Alpha)
    5ms
    Sinonasal Tract Osteochondromyxoma: Future Directions Beyond Morphologic Description. (PubMed, Head Neck Pathol)
    To transition from pathologic description to clinical impact, future efforts must focus on validating diagnostic criteria in large, multi-institutional cohorts, integrating comprehensive molecular profiling to uncover therapeutic vulnerabilities, and establishing evidence-based management protocols. The pathologic recognition of STOX must be seamlessly connected to systematic genetic evaluation and long-term monitoring, fulfilling the translational imperative of this diagnosis.
    Review • Journal
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    PRKAR1A (Protein Kinase CAMP-Dependent Type I Regulatory Subunit Alpha)