Affinage

PABIR1

PPP2R1A-PPP2R2A-interacting phosphatase regulator 1 · UniProt Q96E09

Length
287 aa
Mass
30.5 kDa
Annotated
2026-06-10
14 papers in source corpus 11 papers cited in narrative 11 extracted findings
Cross-family judge vs UniProt: Affinage preferred faithfulness: 6/6 claims corpus-supported (100%)

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

PABIR1 (FAM122A) is an intrinsically disordered, endogenous substrate-competitive inhibitor of the PP2A:B55α holoenzyme that couples phosphatase regulation to cell-cycle progression, the DNA replication-stress response, and developmental signalling (PMID:27588481, PMID:38123684, PMID:38378890). It binds the PP2A-Aα and B55α subunits (but not B56α) and inhibits holoenzyme activity, and additionally promotes poly-ubiquitination and proteasomal degradation of the catalytic PP2A-Cα subunit (PMID:27588481). Structurally, FAM122A engages B55α through a conserved N-terminal SLiM ([RK]-V-x-x-[VI]-R) that folds into a short helix docking in the B55α top groove, while an adjacent helix occludes the catalytic subunit to block CDK-substrate dephosphorylation; efficient inhibition also requires a C-terminal region (residues 150–170) containing a cell-cycle-regulated phospho-Ser158, defining a bipartite binding mode (PMID:38123684, PMID:38982062, PMID:41928937). Functionally, FAM122A-dependent inhibition of PP2A-B55 is the initiating event of mitotic entry, permitting cyclin A/Cdk substrate phosphorylation and full cyclin B/Cdk1 and Greatwall activation before Greatwall-phosphorylated ARPP19/ENSA displaces it to sustain phosphatase inhibition through mitosis (PMID:38378890). CHK1 directly phosphorylates FAM122A, and its loss activates PP2A-B55α to dephosphorylate and stabilize WEE1, abrogating G1/S and intra-S checkpoints and conferring resistance to CHK1 inhibitors (PMID:33108758, PMID:38982062). Beyond cell-cycle control, FAM122A is SUMOylated at K89 (reversed by SENP1) to further reduce PP2A-Cα (PMID:29678583), interacts with the GATA1 zinc-finger to suppress erythroid differentiation (PMID:32763160), and is required for mesendodermal/cardiac differentiation via PP2A-dependent regulation of histone modifications and Wnt/Hippo signalling (PMID:36715298).

Mechanistic history

Synthesis pass · year-by-year structured walk · 9 steps
  1. 2016 Medium

    Established FAM122A as a direct binding partner and inhibitor of a specific PP2A holoenzyme, defining its core molecular activity.

    Evidence Reciprocal Co-IP, in-cell phosphatase and ubiquitination assays with overexpression/knockdown in cell lines

    PMID:27588481

    Open questions at the time
    • Did not resolve binding interface or distinguish inhibition from catalytic-subunit degradation as the primary mechanism
    • B-subunit specificity tested only against B56α
  2. 2018 Medium

    Showed FAM122A activity is itself post-translationally tuned, linking SUMOylation to PP2A-Cα abundance and proliferation control.

    Evidence K89 site-directed mutagenesis, SUMO conjugation/SENP1 de-conjugation assays and phosphatase activity readout in cells

    PMID:29678583

    Open questions at the time
    • SUMO E3 ligase and physiological trigger for K89 SUMOylation not identified
    • Mechanistic link between FAM122A SUMOylation and reduced PP2A-Cα not resolved
  3. 2020 High

    Placed FAM122A in the replication-stress checkpoint by showing CHK1 phosphorylates it and its loss stabilizes WEE1 via PP2A-B55α, explaining CHK1-inhibitor resistance.

    Evidence In vitro CHK1 kinase assay, CRISPR KO, WEE1 ubiquitination/immunoblot, PP2A activity assay and genetic rescue

    PMID:33108758

    Open questions at the time
    • CHK1 phosphosite(s) on FAM122A and their functional consequence not mapped here
    • Whether WEE1 is a direct PP2A-B55α substrate not fully resolved
  4. 2020 Medium

    Extended FAM122A function beyond PP2A by identifying transcriptional (GATA1) and genome-stability (TOP2α) partners.

    Evidence Co-IP and ChIP in hematopoietic progenitors/erythroleukemia cells (GATA1); Co-IP, CRISPR KO, γH2AX and comet assays (TOP2α)

    PMID:32763160 PMID:32866497

    Open questions at the time
    • Whether GATA1 and TOP2α effects are PP2A-dependent or independent not established
    • Direct vs indirect nature of TOP2α protein-level regulation unclear
  5. 2023 High

    Resolved the structural basis of inhibition, revealing FAM122A as a disordered protein engaging B55α at multiple sites distinct from ARPP19.

    Evidence Single-particle cryo-EM of PP2A:B55–FAM122A complexes, NMR and biochemical binding assays

    PMID:38123684

    Open questions at the time
    • Functional dissection of individual contact sites limited within structural study
    • C-terminal phospho-dependent contacts not yet defined
  6. 2024 High

    Defined the inhibitory mechanism at residue resolution: a docking SLiM helix plus a catalytic-subunit-occluding helix make FAM122A substrate-competitive and checkpoint-essential.

    Evidence Helical-motif mutagenesis, in vitro competition and CDK-substrate dephosphorylation assays, CRISPR KO, flow cytometry, checkpoint-kinase immunoblot

    PMID:36945596 PMID:38982062

    Open questions at the time
    • How FAM122A inhibition is relieved at mitotic exit not addressed
    • Contribution of C-terminal region not yet incorporated
  7. 2024 High

    Established FAM122A as the initiating PP2A-B55 inhibitor at mitotic entry, ordering its action ahead of the ARPP19/ENSA hand-off and showing conservation in vivo.

    Evidence Xenopus egg extract add-back/depletion biochemistry and C. elegans germline RNAi with Cdk-substrate and Greatwall/ENSA phosphorylation readouts

    PMID:38378890

    Open questions at the time
    • Trigger that switches inhibition from FAM122A to ARPP19/ENSA not fully defined
    • Regulation of FAM122A levels/activity across the cell cycle not resolved here
  8. 2023 Medium

    Connected FAM122A to embryonic development, showing it is required for mesendoderm/cardiac differentiation through PP2A-dependent chromatin and Wnt/Hippo control.

    Evidence Conditional KO mice, mouse ESC differentiation, PP2A activity assays, histone-modification immunoblot and Wnt/Hippo reporters

    PMID:36715298

    Open questions at the time
    • Direct PP2A substrates mediating histone and Wnt/Hippo changes not identified
    • Cell-type specificity of the requirement not resolved
  9. 2026 Medium

    Revised the binding model to bipartite, adding a C-terminal region and a cell-cycle-regulated phospho-Ser158 as determinants of efficient PP2A-B55 inhibition.

    Evidence Alanine scanning of residues 150–170, Ser158 phospho-mutagenesis, Co-IP in human cells, Xenopus mitotic-entry assay and phosphoproteomic staging (preprint)

    PMID:41928937

    Open questions at the time
    • Preprint; kinase phosphorylating Ser158 not identified
    • Structural basis of the C-terminal contact not yet resolved

Open questions

Synthesis pass · forward-looking unresolved questions
  • How FAM122A integrates its PP2A-inhibitory role with its PP2A-independent partners (GATA1, TOP2α) and how its activity is switched on and off across the cell cycle remain open.
  • Upstream signals regulating FAM122A abundance/modification not unified
  • Whether transcriptional and genome-stability functions are PP2A-dependent unresolved

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0098772 molecular function regulator activity 4 GO:0140096 catalytic activity, acting on a protein 1
Pathway
R-HSA-1640170 Cell Cycle 2 R-HSA-73894 DNA Repair 2 R-HSA-1266738 Developmental Biology 1

Evidence

Reading pass · 11 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
2016 FAM122A directly interacts with PP2A-Aα and B55α subunits (but not B56α) and inhibits the phosphatase activity of the PP2A-Aα/B55α/Cα holoenzyme; additionally, FAM122A promotes poly-ubiquitination and proteasomal degradation of the catalytic subunit PP2A-Cα. Co-immunoprecipitation, phosphatase activity assay, ubiquitination assay, overexpression and knockdown in cell lines Oncotarget Medium 27588481
2018 FAM122A is SUMOylated at lysine 89, a modification that can be removed by the SUMO protease SENP1; SUMOylation of FAM122A reduces PP2A-Cα protein levels and PP2A phosphatase activity, thereby suppressing cell proliferation. Site-directed mutagenesis (K89 mutation), Co-IP/SUMO conjugation assay, SENP1 de-conjugation assay, phosphatase activity assay Biochemical and biophysical research communications Medium 29678583
2020 CHK1 directly phosphorylates FAM122A; loss of FAM122A (CRISPR KO) activates PP2A-B55α, which dephosphorylates WEE1 and rescues it from ubiquitin-mediated degradation, elevating WEE1 protein and thereby reducing replication stress and conferring resistance to CHK1 inhibitors. CRISPR knockout, in vitro kinase assay (CHK1 phosphorylation of FAM122A), immunoblot for WEE1 ubiquitination and protein level, PP2A activity assay, genetic rescue experiments Molecular cell High 33108758
2020 FAM122A directly interacts with the C-terminal zinc finger domain of GATA1, reducing GATA1 chromatin occupancy on target gene promoters and suppressing GATA1 transcriptional activity, thereby inhibiting erythroid differentiation. Co-immunoprecipitation, chromatin immunoprecipitation (ChIP), overexpression in primary human hematopoietic progenitor cells and erythroleukemia cells Stem cell reports Medium 32763160
2020 FAM122A interacts with TOP2α (but not TOP2β); FAM122A deletion increases TOP2α protein level and enhances TOP2 poison-induced DNA damage in cancer cells without affecting ROS levels or short-term DNA repair, suggesting FAM122A maintains DNA stability by modulating TOP2α. Co-immunoprecipitation, CRISPR KO, γH2AX immunofluorescence/foci, comet assay, ROS measurement Experimental cell research Medium 32866497
2023 Cryo-EM structures of PP2A:B55 bound to FAM122A (and separately to phosphorylated ARPP19) reveal that FAM122A, an intrinsically disordered protein, binds PP2A:B55 through multiple distinct sites on the B55 subunit in a manner different from ARPP19; NMR and biochemical data show how substrates and inhibitors are recruited to PP2A:B55. Single-particle cryo-EM, NMR spectroscopy, biochemical binding assays Nature High 38123684
2023 FAM122A contains a conserved short linear motif (SLiM) [RK]-V-x-x-[VI]-R that forms a short α-helix required for binding to the top groove of B55α; a second adjacent helix occludes the catalytic subunit, making FAM122A a substrate-competitive inhibitor of B55α/PP2A that prevents CDK substrate dephosphorylation; FAM122A deficiency abrogates G1/S and intra-S phase checkpoints and attenuates CHK1/CHK2 activation under replication stress. Mutagenesis of SLiM, AlphaFold2 structural prediction, in vitro competition/binding assays, dephosphorylation assays in cell lysates, CRISPR KO in HEK293, flow cytometry cell-cycle analysis bioRxivpreprint Medium 36945596
2024 FAM122A uses helical motifs (a substrate-docking SHeM [RK]-V-x-x-[VI]-R helix and an adjacent catalytic-subunit-occluding helix) to act as a substrate-competitive inhibitor of B55α/PP2A; FAM122A deficiency impairs G1/S and intra-S phase checkpoint responses and attenuates CHK1 and CHK2 activation after replication stress. Mutagenesis of helical motifs, in vitro competition assay, CDK-substrate dephosphorylation assay in cell lysates, CRISPR KO, flow cytometry, immunoblot for checkpoint kinases Nature communications High 38982062
2024 FAM122A-dependent inhibition of PP2A-B55 is the initial mitotic-entry event: FAM122A permits phosphorylation of early mitotic substrates by cyclin A/Cdk, enabling full cyclin B/Cdk1 and Greatwall kinase activation; subsequently, Greatwall-phosphorylated Arpp19/ENSA competes with FAM122A and displaces it from PP2A-B55, taking over phosphatase inhibition for the remainder of mitosis. Loss of the FAM122A orthologue in C. elegans prevents germline stem cells from entering mitosis. Xenopus egg extract biochemistry (add-back/depletion), C. elegans RNAi (germline mitotic entry assay), immunoblot for Cdk substrate phosphorylation, Greatwall/ENSA phosphorylation assays The EMBO journal High 38378890
2023 FAM122A is required for mesendodermal specification and cardiac differentiation from mouse embryonic stem cells; Fam122a conditional cardiac KO causes embryonic lethality with cardiovascular defects; the differentiation defect is mechanistically linked to dysregulation of histone modifications and Wnt and Hippo signalling pathways through modulation of PP2A activity. Conditional KO mice, mouse ESC differentiation assays, PP2A activity assay, histone modification immunoblot, Wnt/Hippo pathway reporter assays Stem cells (Dayton, Ohio) Medium 36715298
2026 FAM122A inhibits PP2A-B55 through a bipartite binding mechanism: the N-terminal helices and a novel C-terminal region (residues 150–170) are both required for efficient binding; conserved Ser158 within this C-terminal region is important for PP2A-B55 inhibition in human cells and for stimulation of mitotic entry in Xenopus egg extracts; Ser158 phosphorylation occupancy increases at cell cycle stages requiring PP2A-B55 inhibition. Systematic alanine scanning of residues 150–170, phospho-site mutagenesis (Ser158), Co-IP in human cells, Xenopus egg extract mitotic-entry assay, phosphoproteomics/cell-cycle staging bioRxivpreprint Medium 41928937

Source papers

Stage 0 corpus · 14 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
2020 CHK1 Inhibitor Blocks Phosphorylation of FAM122A and Promotes Replication Stress. Molecular cell 50 33108758
2016 FAM122A, a new endogenous inhibitor of protein phosphatase 2A. Oncotarget 34 27588481
2023 Cryo-EM structures of PP2A:B55-FAM122A and PP2A:B55-ARPP19. Nature 32 38123684
2019 FAM122A supports the growth of hepatocellular carcinoma cells and its deletion enhances Doxorubicin-induced cytotoxicity. Experimental cell research 13 31711919
2024 FAM122A ensures cell cycle interphase progression and checkpoint control by inhibiting B55α/PP2A through helical motifs. Nature communications 8 38982062
2020 FAM122A Inhibits Erythroid Differentiation through GATA1. Stem cell reports 6 32763160
2024 Increases in cyclin A/Cdk activity and in PP2A-B55 inhibition by FAM122A are key mitosis-inducing events. The EMBO journal 5 38378890
2018 Identifying the SUMO1 modification of FAM122A leading to the degradation of PP2A-Cα by ubiquitin-proteasome system. Biochemical and biophysical research communications 4 29678583
2023 FAM122A ensures cell cycle interphase progression and checkpoint control as a SLiM-dependent substrate-competitive inhibitor to the B55⍺/PP2A phosphatase. bioRxiv : the preprint server for biology 3 36945596
2020 FAM122A maintains DNA stability possibly through the regulation of topoisomerase IIα expression. Experimental cell research 3 32866497
2024 FAM122A functions as a tumor suppressor in oral squamous cell carcinoma. Experimental cell research 2 39009214
2023 FAM122A Is Required for Mesendodermal and Cardiac Differentiation of Embryonic Stem Cells. Stem cells (Dayton, Ohio) 2 36715298
2026 FAM122A inhibition of PP2A-B55 through a bipartite binding mechanism. bioRxiv : the preprint server for biology 0 41928937
2023 Cryo-EM structures of PP2A:B55-FAM122A and PP2A:B55-ARPP19. bioRxiv : the preprint server for biology 0 37693408

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