Affinage

TEPSIN

AP-4 complex accessory subunit Tepsin · UniProt Q96N21

Length
525 aa
Mass
55.1 kDa
Annotated
2026-06-10
15 papers in source corpus 8 papers cited in narrative 8 extracted findings
Cross-family judge vs UniProt: Affinage preferred faithfulness: 5/5 claims corpus-supported (100%)

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

Tepsin is the only known dedicated accessory protein of the AP-4 non-clathrin coat that operates at the trans-Golgi network, where it is recruited to membranes through the AP-4 complex rather than through direct lipid binding (PMID:22472443, PMID:25552650). Its recruitment is strictly AP-4-dependent: disruption of AP-4 assembly through loss-of-function mutation of the AP4S1 subunit abolishes tepsin membrane association (PMID:25552650), and two phylogenetically conserved C-terminal peptide motifs engage the β4 and ε appendage/ear domains of AP-4 in a bivalent interaction that increases avidity and can cross-link AP-4 heterotetramers during coat assembly (PMID:26542808, PMID:26756312). Structurally, tepsin's ENTH and VHS domains are degenerate—lacking the lipid-binding pocket of epsins and the cargo/ubiquitin-recognition surfaces of canonical VHS domains—accounting for its dependence on AP-4 for membrane localization (PMID:28691777). Beyond its scaffolding role, tepsin directly binds LC3B through a canonical LIR motif plus additional disordered-region motifs that engage the LC3B docking site with micromolar affinity in a multivalent manner (PMID:38381558, PMID:41198464), and this interaction is required for proper ATG9A export from the TGN and its peripheral distribution, linking tepsin to autophagosome biogenesis (PMID:38381558). Loss of tepsin in zebrafish causes abnormal head morphology and neural necrosis with altered atg9a and map1lc3b expression, situating this AP-4/autophagy axis in a developmental context (PMID:36642642).

Mechanistic history

Synthesis pass · year-by-year structured walk · 8 steps
  1. 2012 High

    Established that the AP-4 coat, unlike other adaptor complexes, possesses a dedicated accessory protein, identifying tepsin as the first such component and defining a starting point for AP-4 coat biology.

    Evidence SILAC quantitative mass spectrometry of AP-4-coated vesicle fractions with siRNA knockdown profiling in HeLa cells

    PMID:22472443

    Open questions at the time
    • Did not define how tepsin associates with AP-4 mechanistically
    • No cargo or downstream function established
  2. 2014 Medium

    Determined that tepsin's membrane localization is not autonomous but requires an intact AP-4 complex, establishing the hierarchy of coat assembly.

    Evidence Patient-derived fibroblasts carrying AP4S1 loss-of-function mutations, with immunofluorescence/Western blot of AP-4 subunits and tepsin

    PMID:25552650

    Open questions at the time
    • Single lab, patient-cell context
    • Did not map the molecular interface mediating recruitment
  3. 2015 High

    Defined the molecular basis of tepsin–AP-4 binding by identifying two conserved C-terminal motifs that engage the β4 and ε appendage domains, showing a bivalent interaction that could drive coat assembly.

    Evidence GST pulldown, yeast two-hybrid, phage display, structure-based mutagenesis and cellular localization assays

    PMID:26542808

    Open questions at the time
    • Structural detail of the binding surfaces not resolved
    • Functional consequence of cross-linking for vesicle formation untested
  4. 2016 High

    Mapped the tepsin LFxG[M/L]x[L/V] motif directly onto the β4 appendage surface, confirming specificity but revealing residual cellular binding that implied additional interaction sites.

    Evidence NMR chemical shift mapping, in vitro binding, point mutagenesis and co-immunoprecipitation in cells

    PMID:26756312

    Open questions at the time
    • Identity of the residual ε-mediated contribution not structurally resolved here
    • Did not address physiological cargo
  5. 2017 High

    Explained why tepsin depends on AP-4 by solving structures showing its ENTH and VHS domains are degenerate, lacking lipid-binding and cargo/ubiquitin-recognition features of canonical epsin/VHS proteins.

    Evidence X-ray crystallography of tepsin ENTH and VHS domains with biochemical binding assays and comparative genomics

    PMID:28691777

    Open questions at the time
    • Functional role of the degenerate domains, if any, remained undefined
    • No interactor identified for these domains
  6. 2022 Medium

    Placed tepsin function in a whole-organism developmental context, linking its loss to neural defects and dysregulated autophagy gene expression in vivo.

    Evidence CRISPR knockout in zebrafish embryos with morphological and gene-expression analysis at 24 hpf

    PMID:36642642

    Open questions at the time
    • Causal mechanism linking tepsin loss to neural necrosis not resolved
    • Single lab, indirect link to ATG9A trafficking
  7. 2024 High

    Connected tepsin to autophagosome biogenesis by demonstrating direct LIR-mediated LC3B binding required for ATG9A export from the TGN and peripheral distribution.

    Evidence Recombinant pulldown and calorimetry, structural modeling, siRNA knockdown with mRFP-GFP-LC3B reporter and LIR-mutant rescue experiments

    PMID:38381558

    Open questions at the time
    • Only partial rescue achieved by wild-type reintroduction
    • How ATG9A export couples to LC3B binding mechanistically unresolved
    • Effect on autophagic flux absent despite altered autophagosome number
  8. 2025 Medium

    Refined the tepsin–LC3B interaction as multivalent, identifying three additional LC3B-binding motifs and stoichiometry consistent with one tepsin engaging two LC3B molecules.

    Evidence AlphaFold Multimer modeling, bio-layer interferometry, biochemical binding and mutagenesis with thermodynamic/kinetic analysis

    PMID:41198464

    Open questions at the time
    • Functional relevance of multivalency in cells not directly tested
    • Modeling-derived motifs require structural confirmation
    • Single lab

Open questions

Synthesis pass · forward-looking unresolved questions
  • How tepsin mechanistically coordinates AP-4 coat assembly with LC3B-dependent ATG9A export, and whether its bivalent AP-4 binding and multivalent LC3B engagement are temporally coupled during vesicle formation, remains unresolved.
  • No integrated structural model of tepsin bridging AP-4 and LC3B
  • Cargo selectivity determinants for ATG9A export undefined
  • Physiological role of multivalent LC3B binding untested in cells

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0060090 molecular adaptor activity 4
Localization
GO:0005794 Golgi apparatus 3
Pathway
R-HSA-5653656 Vesicle-mediated transport 2 R-HSA-9612973 Autophagy 2
Complex memberships
AP-4 coat

Evidence

Reading pass · 8 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
2012 Tepsin was identified as the first accessory protein of the AP-4 coat complex, co-purifying with AP-4-coated vesicles from HeLa cells as determined by multivariate SILAC-based quantitative mass spectrometry and siRNA knockdown profiling. SILAC-based quantitative mass spectrometry, siRNA knockdown, principal component analysis of coated vesicle fractions The Journal of cell biology High 22472443
2014 Recruitment of tepsin to the membrane is abolished when AP-4 complex assembly is disrupted by loss-of-function mutations in AP4S1, demonstrating that tepsin membrane recruitment depends on intact AP-4 complex. Patient fibroblast cell line analysis; immunofluorescence/Western blot showing loss of AP-4 subunits and tepsin membrane recruitment upon AP4S1 frameshift/nonsense mutations Human molecular genetics Medium 25552650
2015 Tepsin contains two phylogenetically conserved peptide motifs in its unstructured C-terminus—[GS]LFXG[ML]X[LV] and S[AV]F[SA]FLN—that interact with the C-terminal ear/appendage domains of the β4 and ε subunits of AP-4, respectively. Both interactions are required for efficient association of tepsin with AP-4 and for tepsin recruitment to the TGN. Bivalent interaction increases avidity and may cross-link AP-4 heterotetramers to contribute to coat assembly. Protein interaction assays (GST pulldown, yeast two-hybrid, phage display), structure-based mutagenesis, cellular localization assays The Journal of biological chemistry High 26542808
2016 The tepsin C-terminal LFxG[M/L]x[L/V] motif binds directly and specifically to the AP-4 β4 appendage domain. NMR chemical shift mapping defined the binding site on the β4 appendage surface. Point mutations in either the tepsin motif or the cognate β4 surface abolish in vitro binding and greatly reduce (but do not completely abolish) tepsin–AP-4 interaction in cells, suggesting additional interaction sites exist. NMR chemical shift mapping, in vitro binding assays, point mutagenesis, co-immunoprecipitation in cells Traffic (Copenhagen, Denmark) High 26756312
2017 X-ray crystal structures of the tepsin ENTH and VHS/ENTH-like domains revealed that: (1) the tepsin ENTH domain lacks helix0, helix8, and a lipid-binding pocket present in epsin1/2/3, explaining why tepsin requires AP-4 for membrane recruitment rather than binding lipids directly; (2) the tepsin VHS domain lacks helix8 and does not mediate known VHS functions such as recognition of dileucine-based cargo motifs or ubiquitin binding. X-ray crystallography, biochemical/biophysical binding assays, phylogenetic and comparative genomic analysis Traffic (Copenhagen, Denmark) High 28691777
2024 Tepsin directly binds LC3B (preferentially over other mammalian ATG8 family members) via a canonical LC3-Interacting Region (LIR) motif, with micromolar affinity at the LC3B LIR docking site. Loss of tepsin in cultured cells dysregulates ATG9A export from the TGN and ATG9A distribution at the cell periphery. Tepsin depletion increases autophagosome volume and number without affecting autophagic flux. Reintroduction of wild-type tepsin partially rescues ATG9A trafficking defects, while tepsin with a mutated LIR motif or missing N-terminus fails to fully rescue ATG9A distribution. In silico LIR motif prediction, recombinant protein biochemistry (pulldown, calorimetry), structural modeling, siRNA knockdown, fluorescence microscopy (mRFP-GFP-LC3B reporter), rescue experiments with LIR-mutant tepsin Molecular biology of the cell High 38381558
2022 Loss of tepsin in CRISPR-edited zebrafish embryos causes abnormal head morphology and neural necrosis, and alters expression levels and patterns of autophagy genes atg9a and map1lc3b, linking tepsin function to AP-4-dependent ATG9A trafficking and autophagy in a developmental context. CRISPR-ExoCas9 knockout in zebrafish, morphological analysis at 24 hpf, gene expression analysis Advances in biological regulation Medium 36642642
2025 Computational modeling (AlphaFold Multimer) combined with bio-layer interferometry (BLI) and biochemical experiments identified three additional LC3B-binding motifs beyond the canonical LIR in tepsin's disordered regions, all engaging the LC3B LIR docking site. All four motifs must be mutated to abrogate LC3B binding in vitro. Stoichiometry data indicate one tepsin molecule likely binds two LC3B molecules simultaneously, suggesting multivalent LC3B engagement that could dynamically modulate binding strength in response to LC3B membrane concentrations. AlphaFold Multimer structural modeling, bio-layer interferometry (BLI), biochemical binding assays, mutagenesis, thermodynamic/kinetic analysis Advances in biological regulation Medium 41198464

Source papers

Stage 0 corpus · 15 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
2012 Multivariate proteomic profiling identifies novel accessory proteins of coated vesicles. The Journal of cell biology 140 22472443
2014 Recessive loss-of-function mutations in AP4S1 cause mild fever-sensitive seizures, developmental delay and spastic paraplegia through loss of AP-4 complex assembly. Human molecular genetics 49 25552650
2008 A promoter sequence variant of ZNF750 is linked with familial psoriasis. The Journal of investigative dermatology 31 18256691
2019 Frailty in middle age is associated with frailty status and race-specific changes to the transcriptome. Aging 24 31395793
2016 Molecular Basis for the Interaction Between AP4 β4 and its Accessory Protein, Tepsin. Traffic (Copenhagen, Denmark) 24 26756312
2015 Bivalent Motif-Ear Interactions Mediate the Association of the Accessory Protein Tepsin with the AP-4 Adaptor Complex. The Journal of biological chemistry 22 26542808
2020 The role of AP-4 in cargo export from the trans-Golgi network and hereditary spastic paraplegia. Biochemical Society transactions 16 33084855
2017 Structure and evolution of ENTH and VHS/ENTH-like domains in tepsin. Traffic (Copenhagen, Denmark) 14 28691777
2024 Tepsin binds LC3B to promote ATG9A trafficking and delivery. Molecular biology of the cell 5 38381558
2022 AP-4 loss in CRISPR-edited zebrafish affects early embryo development. Advances in biological regulation 4 36642642
2025 Characterization of the malaria parasite Plasmodium falciparum Tepsin homolog. Microbiology spectrum 3 40586818
2025 Vesicle adaptors in malaria parasites show conservation and flexibility of protein sorting machinery. The Journal of cell biology 2 41082687
2023 Tepsin binds LC3B to promote ATG9A export and delivery at the cell periphery. bioRxiv : the preprint server for biology 2 37502979
2025 Tepsin and AP4 mediate transport from the trans-Golgi to the plant-like vacuole in toxoplasma. The Journal of cell biology 1 41082686
2025 The AP-4 accessory protein tepsin exhibits multivalent binding to LC3B. Advances in biological regulation 1 41198464

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