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    GENE:

    PPFIBP1 (PPFIA Binding Protein 1)

    i
    Other names: PPFIBP1, PPFIA Binding Protein 1, PTPRF Interacting Protein, Binding Protein 1 (Liprin Beta 1), PTPRF-Interacting Protein-Binding Protein 1, Liprin-Beta-1, HSGT2, Protein-Tyrosine Phosphatase Receptor-Type F Polypeptide-Interacting Protein-Binding Protein 1, Protein Tyrosine Phosphatase Receptor Type F Polypeptide-Interacting Protein-Binding Protein 1, Liprin Related Protein, Liprin Beta 1, KIAA1230, HSgt2p, SGT2, L2
    4ms
    Molecular Heterogeneity and Clinicopathologic Correlations in Inflammatory Myofibroblastic Tumors of the Urinary Bladder: A Study of 20 Cases With Predominant FN1::ALK Fusions and Novel Kinase Rearrangements. (PubMed, Am J Surg Pathol)
    The predominance of FN1::ALK fusions, sharing identical breakpoints (ALK exons 18 to 19) with pseudosarcomatous myofibroblastic proliferations of the urinary bladder, alongside expanded molecular diversity (non-FN1/ROS1 fusions), supports their classification as a biological continuum of ALK-driven bladder mesenchymal neoplasms. These findings broaden the molecular genetic spectrum of bladder IMTs and advocate for histology-guided molecular testing to identify kinase fusions, reinforcing conservative management for these typically indolent tumors.
    Journal
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    ALK (Anaplastic lymphoma kinase) • ROS1 (Proto-Oncogene Tyrosine-Protein Kinase ROS) • DCTN1 (Dynactin Subunit 1) • PPFIBP1 (PPFIA Binding Protein 1)
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    ALK positive • ALK rearrangement • ALK fusion • ROS1 fusion
    7ms
    Epithelioid Fibrous Histiocytoma (EFH) With Rare PPFIBP1-ALK Fusion: A Predominantly Spindle Cell Variant Within the Emerging Spectrum of Myxoid Spindle Cell EFH. (PubMed, Cureus)
    Next-generation sequencing confirmed the presence of a rare PPFIBP1-ALK fusion. The presented case highlights a predominantly spindle cell variant of EFH and suggests inclusion within the recently described myxoid spindle cell EFH spectrum, which encompasses the superficial ALK-rearranged myxoid spindle cell neoplasms (SAMS).
    Journal
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    ALK (Anaplastic lymphoma kinase) • CD4 (CD4 Molecule) • PPFIBP1 (PPFIA Binding Protein 1) • CD68 (CD68 Molecule)
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    ALK rearrangement • ALK fusion
    1year
    NTRK3-rearranged spindle cell neoplasm of the skin: diagnostic pitfalls of an emerging entity, a case report. (PubMed, Acta Dermatovenerol Alp Pannonica Adriat)
    Recognition of NTRK-RSCNs is crucial for targeted therapy in selected cases, given the recent approval of kinase inhibitors. We describe the case of a 55-year-old male with an NTRK-RSCN located on the arm, harboring the novel fusion partner PPFIBP1::NTRK3, while providing additional clinical and morphological characteristics of this rare entity.
    Journal
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    NTRK3 (Neurotrophic tyrosine kinase, receptor, type 3) • PPFIBP1 (PPFIA Binding Protein 1) • NTRK (Neurotrophic receptor tyrosine kinase)
    over1year
    Identification and Validation of Prognostic Risk Model for Female-Specific Lung Adenocarcinoma. (PubMed, Altern Ther Health Med)
    Immunohistochemical staining showed lower CABLES1 expression was associated with higher pTNM stage in female LUAD but not in male LUAD (P < .05). Our study constructed and verified a prognostic signature based on 12 female-specific DEGs of LUAD, which could improve the understanding of sex-related risk factors involved in LUAD carcinogenesis and progression, and may provide personalized treatment strategies for female LUAD patients.
    Journal
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    EFNB2 (Ephrin B2) • PPFIBP1 (PPFIA Binding Protein 1) • SOX9 (SRY-Box Transcription Factor 9) • CXCL5 (Chemokine (C-X-C motif) ligand 5) • MEOX2 (Mesenchyme Homeobox 2)
    over2years
    PPFIBP1 activates NF-κB signaling to enhance chemoresistance of multiple myeloma. (PubMed, Transl Oncol)
    PPFPIBP1 enhances chemoresistance of MM cells to the treatment of bortezomib (BTZ), a proteasome inhibitor, and manipulation of PPFPIBP1 can alter chemosensitivity of MM cells to BTZ...Targeting PPFPIBP1 in a xenograft mouse model of MM prohibits tumor growth and prolongs overall survival of mice. Taken together, our findings suggest that PPFIBP1 is a crucial regulator of chemoresistance to PIs in MM cells, and shed light on developing therapeutic strategies to overcome chemoresistance by targeting PPFIBP1.
    Journal
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    PPFIBP1 (PPFIA Binding Protein 1)
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    bortezomib
    over3years
    Novel cancer gene discovery using a forward genetic screen in RCAS-PDGFB-driven gliomas. (PubMed, Neuro Oncol)
    Altogether, the current study has demonstrated a novel approach to tagging glioma genes via forward genetics, validating previous results, and identifying PPFIBP1 as a putative oncogene in gliomagenesis.
    Journal
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    PPFIBP1 (PPFIA Binding Protein 1) • PDGFB (Platelet Derived Growth Factor Subunit B)
    over3years
    Whole exome sequencing reveals the genetic heterogeneity and evolutionary history of primary gliomas and matched recurrences. (PubMed, Comput Struct Biotechnol J)
    Intriguingly, the immunogenicity of recurrent gliomas did not increase significantly compared to the primary tumors. Genomic analysis of recurrent gliomas provided an opportunity to identify potentially clinically informative alterations not detected in clinically sampled primary tumors.
    Journal
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    KRAS (KRAS proto-oncogene GTPase) • TP53 (Tumor protein P53) • CDKN2A (Cyclin Dependent Kinase Inhibitor 2A) • ARID1A (AT-rich interaction domain 1A) • MSH6 (MutS homolog 6) • ATRX (ATRX Chromatin Remodeler) • PPFIBP1 (PPFIA Binding Protein 1) • TNFRSF14 (TNF Receptor Superfamily Member 14) • PDE4DIP (Phosphodiesterase 4D Interacting Protein) • TNFRSF18 (TNF Receptor Superfamily Member 18)
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    TP53 mutation • ARID1A mutation • CDKN2A deletion • CDKN2A mutation • ATRX mutation • MSH6 mutation • KRAS deletion
    almost4years
    Molecular investigation of ALK-rearranged epithelioid fibrous histiocytomas identifies CLTC as a novel fusion partner and evidence of fusion-independent transcription activation. (PubMed, Genes Chromosomes Cancer)
    These findings underscore the remarkable plasticity of ALK as an oncogenic driver and further expand the list of its potential fusion partners in EFH. Lastly this is also the first report of ALK-immunoreactive EFH with no underlying fusion suggesting a fusion independent transcription mechanism as seen in other tumors.
    Journal
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    ALK (Anaplastic lymphoma kinase) • DCTN1 (Dynactin Subunit 1) • PPFIBP1 (PPFIA Binding Protein 1) • CLTC (Clathrin Heavy Chain) • PRKAR1A (Protein Kinase CAMP-Dependent Type I Regulatory Subunit Alpha)
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    ALK positive • ALK rearrangement • ALK fusion
    over4years
    PPFIBP1 induces glioma cell migration and invasion through FAK/Src/JNK signaling pathway. (PubMed, Cell Death Dis)
    Finally, inhibition of phosphorylation of Src and FAK significantly reversed the augmentation of invasion and migration caused by PPFIBP1 overexpression in GBM cells. In conclusion, these findings uncover a novel mechanism of glioma invasion and identify PPFIBP1 as a potential therapeutic target of glioma.
    Journal
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    PPFIBP1 (PPFIA Binding Protein 1) • MAPK8 (Mitogen-activated protein kinase 8)
    over5years
    Bioinformatics analysis and verification of molecular targets in ovarian cancer stem-like cells. (PubMed, Heliyon)
    Growth inhibition assay was performed in SK-OV-3 cells after carboplatin treatment...The molecular signature and signaling pathways enriched in ovarian CSCs were identified by bioinformatics analysis. This analysis could provide further research ideas to find the new mechanism and novel potential therapeutic targets for ovarian CSCs.
    Journal
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    CXCR4 (Chemokine (C-X-C motif) receptor 4) • PPFIBP1 (PPFIA Binding Protein 1) • PTGS2 (Prostaglandin-Endoperoxide Synthase 2) • MMP1 (Matrix metallopeptidase 1)
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    carboplatin
    over5years
    [VIRTUAL] The landscape of targetable rearrangements in Chinese patients with gastrointestinal cancer (ESMO 2020)
    Legal entity responsible for the study: The authors. Funding: Has not received any funding.
    Clinical
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    EGFR (Epidermal growth factor receptor) • BRAF (B-raf proto-oncogene) • ALK (Anaplastic lymphoma kinase) • MSI (Microsatellite instability) • FGFR2 (Fibroblast growth factor receptor 2) • ROS1 (Proto-Oncogene Tyrosine-Protein Kinase ROS) • NTRK1 (Neurotrophic tyrosine kinase, receptor, type 1) • NTRK3 (Neurotrophic tyrosine kinase, receptor, type 3) • FGFR3 (Fibroblast growth factor receptor 3) • EML4 (EMAP Like 4) • TACC3 (Transforming acidic coiled-coil containing protein 3) • NCOA4 (Nuclear Receptor Coactivator 4) • PPFIBP1 (PPFIA Binding Protein 1)