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ATP9A

Probable phospholipid-transporting ATPase IIA · UniProt O75110

Length
1047 aa
Mass
118.6 kDa
Annotated
2026-06-09
13 papers in source corpus 9 papers cited in narrative 9 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

ATP9A is a CDC50-independent P4-ATPase phospholipid flippase that operates across the endosomal recycling system and trans-Golgi network to translocate phospholipids and thereby govern membrane trafficking (PMID:40876594, PMID:27733620). Cryo-EM of the outward-facing E2P state shows that, unlike canonical P-type ATPases, ATP9A achieves outward gating through movement of the TM6-10 helices to enlarge a phospholipid-binding cavity, and its ATPase activity is selectively stimulated by negatively charged phospholipids including phosphatidylserine and phosphorylated phosphatidylinositol species (PMID:40876594). ATP9A localizes to PS-positive early and recycling endosomes and supports recycling of transferrin and GLUT1 to the plasma membrane, while modulating RAB5 and RAB11 GTPase activation to drive endosomal recycling (PMID:27733620, PMID:36604604). It functions within an evolutionarily conserved MON2-DOPEY2-ATP9A complex that associates with the SNX3-retromer to direct endosome-to-Golgi cargo sorting, a role requiring its flippase activity (PMID:30213940). ATP9A also forms homomeric and heteromeric complexes with ATP9B that support exocytic VSVG transport from the Golgi and retain ATP9A in the Golgi (PMID:40234049), and it regulates exosome release and RAC1-dependent macropinocytosis (PMID:30947313, PMID:36715683). Loss of ATP9A produces neuronal phenotypes: Atp9a-null mice show movement, memory, and synaptic defects, and ATP9A is required for dendritic arborization and spine maturation, with pathogenic missense and nonsense variants causing mislocalization and aberrant endosomal recycling (PMID:36604604, PMID:40226306).

Mechanistic history

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

    Established ATP9A's cellular site of action by showing it resides on PS-positive early and recycling endosomes and is selectively required for recycling of specific cargoes rather than general endosomal transport.

    Evidence siRNA knockdown with transferrin recycling, GLUT1, EGF, and Shiga toxin readouts in HeLa cells

    PMID:27733620

    Open questions at the time
    • Did not demonstrate flippase activity biochemically
    • Mechanism linking flipping to cargo recycling not defined
  2. 2018 High

    Placed ATP9A in a defined molecular complex by identifying the conserved MON2-DOPEY2-ATP9A assembly that cooperates with SNX3-retromer, linking flippase activity to endosome-to-Golgi cargo sorting.

    Evidence Reciprocal Co-IP, C. elegans RNAi suppression, and ATPase-dead TAT-5(E246Q) mutant in Wnt signaling assays

    PMID:30213940

    Open questions at the time
    • Stoichiometry and architecture of the MON2-DOPEY2-ATP9A complex unresolved
    • How retromer recruits the complex not defined
  3. 2019 Medium

    Extended ATP9A function to vesicle secretion by showing its depletion increases extracellular vesicle and exosome release.

    Evidence siRNA knockdown with nanoparticle tracking analysis and pharmacological exosome inhibition in hepatoma cells

    PMID:30947313

    Open questions at the time
    • Single lab, single study
    • Molecular mechanism connecting flippase activity to exosome release not established
  4. 2023 High

    Connected the endosomal recycling defect to RAB GTPase regulation and to organismal phenotypes, defining ATP9A as required for neuronal morphology and viability.

    Evidence Atp9a knockout mouse with behavioral/synaptic phenotyping, primary neuron culture, and RAB5/RAB11 activation assays

    PMID:36604604

    Open questions at the time
    • How flippase activity modulates RAB GTPase activation mechanistically unclear
    • Direct disease causation in patients not established here
  5. 2023 Medium

    Revealed a role in nutrient-stress signaling by linking ATP9A to ATP6V1A trafficking, plasma membrane cholesterol, and RAC1-dependent macropinocytosis.

    Evidence Co-IP, subcellular fractionation, cholesterol staining, and macropinocytosis/RAC1 assays in hepatocellular carcinoma cells

    PMID:36715683

    Open questions at the time
    • Single lab, single study
    • Whether ATP6V1A is a direct flippase-dependent client unconfirmed
  6. 2025 High

    Resolved the structural basis of transport, showing CDC50-independence and a non-canonical TM6-10 gating mechanism activated by anionic phospholipid substrates.

    Evidence 2.2 Å cryo-EM of the E2P state, ATPase activity assays, and molecular dynamics simulations

    PMID:40876594

    Open questions at the time
    • Inward-facing states and full transport cycle not captured
    • Physiological substrate preference in cells not directly mapped to structure
  7. 2025 Medium

    Defined a Golgi exocytic function by showing ATP9A-ATP9B homo/heteromeric complexes and their flippase activities drive VSVG transport to the plasma membrane.

    Evidence Reciprocal Co-IP, flippase-dead mutants, and VSVG transport assays with localization analysis

    PMID:40234049

    Open questions at the time
    • Functional division of labor between ATP9A and ATP9B not separated
    • Complex stoichiometry unresolved
  8. 2025 Medium

    Linked ATP9A variants directly to neuronal morphological deficits, establishing its requirement for dendritic arborization and spine maturation.

    Evidence Mutant overexpression and shRNA knockdown in HeLa cells and primary neurons with confocal spine/arborization analysis

    PMID:40226306

    Open questions at the time
    • Single lab
    • Causal link between specific recycling cargoes and spine phenotype not established
  9. 2025 Medium

    Implicated ATP9A in maintaining plasma membrane PI4P asymmetry, broadening its substrate scope beyond PS.

    Evidence siRNA knockdown with extracellular-leaflet PI4P asymmetry and neomycin sensitivity assays (preprint)

    PMID:bio_10.1101_2025.03.03.641220

    Open questions at the time
    • Preprint, not peer-reviewed
    • Whether ATP9A directly flips PI4P versus indirectly maintaining asymmetry not resolved

Open questions

Synthesis pass · forward-looking unresolved questions
  • How ATP9A's phospholipid translocation is mechanistically coupled to RAB GTPase regulation, retromer cargo selection, and the diverse downstream trafficking outputs (recycling, exosome release, macropinocytosis, exocytosis) remains unresolved.
  • No unifying model linking flippase substrate specificity to specific trafficking outcomes
  • Direct substrates in each pathway not individually validated

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0008289 lipid binding 2 GO:0016787 hydrolase activity 1 GO:0140657 ATP-dependent activity 1
Localization
GO:0005768 endosome 2 GO:0005794 Golgi apparatus 1 GO:0005886 plasma membrane 1
Pathway
R-HSA-5653656 Vesicle-mediated transport 3 R-HSA-162582 Signal Transduction 2 R-HSA-9609507 Protein localization 2
Complex memberships
ATP9A-ATP9B complexMON2-DOPEY2-ATP9A complex

Evidence

Reading pass · 9 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
2025 Cryo-EM structures of human monomeric ATP9A at 2.2 Å resolution in the outward-facing E2P state revealed a unique gating mechanism: unlike canonical P-type ATPases where gating is driven by TM1/TM2 helix movement linked to the A domain, ATP9A outward gating is achieved by movement of TM6-10 helices, likely initiated by TM6 unwinding, creating a larger phospholipid binding cavity. ATP9A does not require the auxiliary subunit CDC50 protein. ATPase activity is significantly increased by negatively charged phospholipids (phosphatidylserine, phosphatidylinositol, and phosphorylated PI species) but not electroneutral phospholipids. Molecular simulations showed spontaneous binding of phosphorylated PI species in the translocation pathway. Cryo-EM structure determination, ATPase activity assay, molecular dynamics simulation The Journal of biological chemistry High 40876594
2016 ATP9A localizes to phosphatidylserine (PS)-positive early and recycling endosomes (but not late endosomes) in HeLa cells. Depletion of ATP9A delayed recycling of transferrin from endosomes to the plasma membrane and caused accumulation of glucose transporter 1 (GLUT1) in endosomes. ATP9A depletion did not affect early/late endosomal transport and degradation of EGF, nor transport of Shiga toxin B from early/recycling endosomes to the Golgi. siRNA knockdown, fluorescence microscopy/subcellular fractionation, transferrin recycling assay, GLUT1 localization assay Molecular biology of the cell High 27733620
2018 ATP9A is part of an evolutionarily conserved endosome-associated membrane remodelling complex composed of MON2, DOPEY2, and ATP9A (putative aminophospholipid translocase). This complex associates with SNX3-retromer to mediate endosome-to-Golgi transport of Wntless. In vivo suppression of Ce-tat-5 (C. elegans ATP9A ortholog) phenocopies loss of SNX3-retromer function, leading to enhanced lysosomal degradation of Wntless and Wnt signaling defects. Overexpression of an ATPase-inhibited TAT-5(E246Q) mutant also perturbed Wnt signaling, implicating phospholipid flippase activity in SNX3-retromer-mediated Wntless sorting. Co-immunoprecipitation, in vivo RNAi (C. elegans), ATPase-dead mutant overexpression, Wnt phenotypic assay Nature communications High 30213940
2019 Knockdown of ATP9A expression in human hepatoma cells resulted in a significant increase in extracellular vesicle (EV) release. Pharmacological blocking of exosome release in ATP9A knockdown cells significantly reduced total EV numbers, supporting a role for ATP9A specifically in regulating exosome release. The increased EV release was independent of caspase-3 activation. siRNA knockdown, nanoparticle tracking analysis of EVs, pharmacological inhibition of exosome release PloS one Medium 30947313
2023 ATP9A localizes mainly to endosomes and modulates RAB5 and RAB11 GTPase activation to regulate the endosomal recycling pathway. Atp9a null mice show decreased muscle strength, memory deficits, hyperkinetic movement disorder, abnormal neurite morphology, and impaired synaptic transmission. ATP9A pathogenic mutants (nonsense variants) have aberrant subcellular localization and cause abnormal endosomal recycling. ATP9A is required for maintaining neuronal neurite morphology and viability of neural cells in vitro. Atp9a knockout mouse model, primary neuron culture, subcellular localization assay, RAB5/RAB11 activation assay Molecular psychiatry High 36604604
2023 ATP9A interacts with ATP6V1A (a vacuolar ATPase subunit) and facilitates its transport to the plasma membrane, promoting plasma membrane cholesterol accumulation and driving RAC1-dependent macropinocytosis in hepatocellular carcinoma cells. ATP9A is critical for regulating macropinocytosis under nutrient starvation. Co-immunoprecipitation, subcellular fractionation, cholesterol staining, RAC1 activation assay, macropinocytosis assay The Journal of pathology Medium 36715683
2025 ATP9A and ATP9B form homomeric and/or heteromeric complexes. Both are required for transport of VSVG from the Golgi to the plasma membrane in the exocytic pathway. Flippase activities of both ATP9A and ATP9B are crucial for this transport process. Heteromeric complex formation between ATP9A and ATP9B contributes to retention of ATP9A in the Golgi. Co-immunoprecipitation, VSVG transport assay, flippase-activity mutants, subcellular localization Life science alliance Medium 40234049
2025 Overexpression of four selected missense mutant forms of Atp9a in HeLa cells and primary neuronal cultures led to loss of mature dendritic spines. Three missense variants retained endosomal localization while one remained blocked in the endoplasmic reticulum. shRNA-mediated knockdown of ATP9A in neurons decreased the number of dendrites per neuron, demonstrating a role for ATP9A in neuronal arborization and dendritic spine maturation. Overexpression of mutant ATP9A in HeLa cells and primary neuronal cultures, shRNA knockdown, confocal microscopy of dendritic spines and arborization Human mutation Medium 40226306
2025 Knockdown of ATP9A in human cells causes exposure of phosphatidylinositol-4-phosphate (PI4P) in the extracellular leaflet of the plasma membrane, and ATP9A expression level correlates with neomycin sensitivity. This implicates ATP9A (ortholog of yeast Neo1) in maintaining PI4P membrane asymmetry at the plasma membrane. siRNA knockdown, PI4P membrane asymmetry assay (extracellular leaflet detection), neomycin sensitivity assay bioRxivpreprint Medium bio_10.1101_2025.03.03.641220

Source papers

Stage 0 corpus · 13 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
2018 SNX3-retromer requires an evolutionary conserved MON2:DOPEY2:ATP9A complex to mediate Wntless sorting and Wnt secretion. Nature communications 71 30213940
2016 The phospholipid flippase ATP9A is required for the recycling pathway from the endosomes to the plasma membrane. Molecular biology of the cell 56 27733620
2019 The P4-ATPase ATP9A is a novel determinant of exosome release. PloS one 31 30947313
2023 ATP9A deficiency causes ADHD and aberrant endosomal recycling via modulating RAB5 and RAB11 activity. Molecular psychiatry 22 36604604
2021 Biallelic truncating variants in ATP9A cause a novel neurodevelopmental disorder involving postnatal microcephaly and failure to thrive. Journal of medical genetics 20 34379057
2023 The phospholipid flippase ATP9A enhances macropinocytosis to promote nutrient starvation tolerance in hepatocellular carcinoma. The Journal of pathology 12 36715683
2025 Lipid flippases ATP9A and ATP9B form a complex and contribute to the exocytic pathway from the Golgi. Life science alliance 5 40234049
2025 Circular RNA ATP9A Stimulates Non-small Cell Lung Cancer Progression via MicroRNA-582-3p/Ribosomal Protein Large P0 Axis and Activating Phosphatidylinositol 3-Kinase/Protein Kinase B Signaling Pathway. Applied biochemistry and biotechnology 3 39832103
2025 Heterozygous Missense Variants in the ATPase Phospholipid Transporting 9A Gene, ATP9A, Alter Dendritic Spine Maturation and Cause Dominantly Inherited Nonsyndromic Intellectual Disability. Human mutation 2 40226306
2026 The role of ATP9A (c.1091G > C; p.(Arg364Thr)) variant in cognitive impairment: diagnostic insight from whole exome sequencing. Molecular biology reports 1 41604004
2025 Integration of Mendelian Randomization to explore the genetic influences of pediatric sepsis: a focus on RGL4, ATP9A, MAP3K7CL, and DDX11L2. BMC pediatrics 1 39871218
2025 A unique gating mechanism revealed by the cryo-EM structure of monomeric ATP9A flippase. The Journal of biological chemistry 1 40876594
2026 Identification of ATP9A-NFATC2 gene fusion transcript in Behcet's disease, a subtype of uveitis. Indian journal of ophthalmology 0 41581045

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