Affinage

TUFT1

Tuftelin · UniProt Q9NNX1

Length
390 aa
Mass
44.3 kDa
Annotated
2026-06-10
34 papers in source corpus 15 papers cited in narrative 15 extracted findings
Cross-family judge vs UniProt: Affinage preferred faithfulness: 7/8 claims corpus-supported (88%)

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

TUFT1 (tuftelin 1) is a multifunctional scaffolding protein that operates in two broad arenas: maintenance of epidermal desmosomal integrity and promotion of growth-signaling, vesicular-trafficking, and centrosomal homeostasis in proliferating and malignant cells (PMID:29423269, PMID:36689522, PMID:41022752). In skin, TUFT1 localizes to peripheral keratinocyte cell membranes within desmosomes in a manner dependent on the desmoplakin carboxy-terminal tail, and biallelic loss-of-function variants cause woolly hair and superficial skin fragility characterized by desmosomal detachment, acantholysis, and perinuclear retraction of intermediate filaments, a phenotype recapitulated in Tuft1-knockout mice (PMID:36689522, PMID:37716648). In its trafficking/signaling role, TUFT1 physically interacts with the Rab GTPase-activating protein RABGAP1 to control lysosomal positioning and vesicle trafficking and to promote mTORC1 activation (PMID:29423269). Across multiple cancers it activates PI3K/AKT-mTOR signaling and drives proliferation, invasion, and epithelial-mesenchymal transition, acting through a HIF1-Snail axis, a p85α/Rab5/Tiam1/Rac1 and Rac1/β-catenin cascade, and Akt-mTOR/Akt-GSK3β pathways (PMID:30250300, PMID:27566398, PMID:31572059, PMID:31338333, PMID:30662679). Its oncogenic activity is gated by post-translational SUMOylation at K79 by TRIM27, which is required for AKT/mTOR activation and for forming a perinuclear mTORC1-promoting complex with RABGAP1 (PMID:36380570). TUFT1 also stabilizes TGF-β receptor II by binding it through its N-terminus and competing with caveolin-1, redirecting the receptor away from lysosomal degradation toward endosomal signaling to drive myofibroblastic activation of hepatic stellate cells (PMID:41593321). At the centrosome, TUFT1 localizes to proximal ends of parent centrioles and prevents centrosome amplification and spindle multipolarity by suppressing premature PLK1 activation, with its function regulated by NEK2-mediated phosphorylation (PMID:41022752). Its expression is controlled epigenetically by BRD9/P300-driven H3K27 acetylation and post-transcriptionally by miRNAs targeting its 3'-UTR (PMID:30250300, PMID:32908135).

Mechanistic history

Synthesis pass · year-by-year structured walk · 10 steps
  1. 2016 Medium

    Established TUFT1 as a driver of epithelial-mesenchymal transition in cancer, linking it to a transcriptional EMT program for the first time.

    Evidence shRNA knockdown/overexpression with migration, invasion, and metastasis readouts plus EMT marker and HIF1 activity analysis in pancreatic cancer

    PMID:27566398

    Open questions at the time
    • HIF1-Snail link is associative, not reconstituted
    • no direct binding partner identified
    • molecular mechanism by which TUFT1 alters Snail unknown
  2. 2018 High

    Identified the first direct physical partner of TUFT1 (RABGAP1) and connected it to lysosomal positioning, vesicle trafficking, and mTORC1 activation, defining a core trafficking/signaling function.

    Evidence Co-IP, cellular fractionation/lysosomal positioning assays, and mTORC1 activity assays with TUFT1 perturbation

    PMID:29423269

    Open questions at the time
    • structural basis of TUFT1-RABGAP1 interaction unresolved
    • how TUFT1 modulates RABGAP1 GAP activity not defined
    • link between trafficking role and skin/centrosome functions unexplored
  3. 2018 Medium

    Defined an upstream regulatory and downstream signaling axis showing hypoxia drives TUFT1 via HIF-1α/miR-671-5p and TUFT1 activates Ca2+/PI3K/AKT to promote HCC growth and metastasis.

    Evidence ChIP, microRNA array, luciferase 3'-UTR reporter, functional rescue, and xenograft models

    PMID:30250300

    Open questions at the time
    • mechanism linking TUFT1 to Ca2+/PI3K/AKT not biochemically reconstituted
    • single tumor type
  4. 2018 Medium

    Showed TUFT1 acts as a secreted/recombinant-active factor signaling through Akt-mTOR and Akt-GSK3β, with pharmacological epistasis dissecting distinct contributions to invasion versus proliferation.

    Evidence Recombinant protein treatment plus mTOR and GSK3β inhibitor epistasis and xenograft in thyroid carcinoma

    PMID:30662679

    Open questions at the time
    • receptor mediating recombinant TUFT1 effects unidentified
    • single lab
  5. 2019 Medium

    Mapped a TUFT1-driven small-GTPase cascade in TNBC, identifying p85α binding leading to Rab5, Tiam1, and Rac1 activation alongside Rac1/β-catenin-mediated stemness and chemoresistance.

    Evidence Co-IP for p85α binding, Rab5-GTP/Rac1-GTP activity assays, sphere formation, and xenograft metastasis models

    PMID:31338333 PMID:31572059

    Open questions at the time
    • direct versus indirect nature of p85α binding not fully resolved
    • how one scaffold coordinates multiple GTPases unclear
  6. 2020 Medium

    Revealed the epigenetic control of TUFT1, showing BRD9 recruits P300 to deposit H3K27Ac at the TUFT1 promoter to activate AKT signaling.

    Evidence ChIP-qPCR for P300/H3K27Ac, BRD9 knockdown/inhibition, and TUFT1 rescue in HCC

    PMID:32908135

    Open questions at the time
    • whether BRD9 binds the promoter directly not established
    • single tumor context
  7. 2022 Medium

    Identified SUMOylation at K79 by TRIM27 as a post-translational switch required for TUFT1 oncogenic AKT/mTOR activation and RABGAP1-dependent perinuclear mTORC1 accumulation.

    Evidence Site-directed mutagenesis (K79), Co-IP, TRIM27 knockdown epistasis, and functional assays in gastric cancer

    PMID:36380570

    Open questions at the time
    • how SUMOylation alters TUFT1 conformation/partner binding unknown
    • deSUMOylation enzymes not identified
  8. 2023 High

    Established TUFT1 as a desmosome-associated protein essential for epidermal integrity, with biallelic loss causing woolly hair and skin fragility, defining its first Mendelian disease role.

    Evidence Exome/genome sequencing across families, immunofluorescence, TEM, scRNA-seq, desmoplakin tail-dependent transfection, and Tuft1-knockout mouse

    PMID:36689522 PMID:37716648

    Open questions at the time
    • molecular interaction of TUFT1 with desmosomal components beyond desmoplakin-tail dependence undefined
    • how the same protein serves desmosomal and cytoplasmic signaling roles unresolved
  9. 2025 Medium

    Placed TUFT1 at the centrosome as a regulator of centriole number and spindle bipolarity by restraining premature PLK1 activation, under control of NEK2 phosphorylation.

    Evidence Immunofluorescence localization, loss-of-function centrosome/spindle readouts, PLK1 activity epistasis, and NEK2 phosphorylation assays

    PMID:41022752

    Open questions at the time
    • mechanism by which TUFT1 suppresses PLK1 activation undefined
    • functional phospho-sites on TUFT1 not mapped
    • relationship to its trafficking/signaling roles unclear
  10. 2026 High

    Demonstrated a receptor-stabilization mechanism in which TUFT1 binds TβRII via its N-terminus and competes with caveolin-1 to divert TβRII from lysosomal degradation into endosomal signaling, driving hepatic stellate cell activation.

    Evidence IP-MS partner discovery, domain-mapping Co-IP, caveolin-1 competition and lysosomal degradation assays, and in vivo HSC/CRC mouse models

    PMID:41593321

    Open questions at the time
    • whether this TGF-β mechanism operates in epithelial/cancer contexts not tested
    • structural detail of TUFT1/caveolin-1 competition unknown

Open questions

Synthesis pass · forward-looking unresolved questions
  • How a single protein integrates its desmosomal-structural, centrosomal, vesicular-trafficking, and growth-signaling functions — and which post-translational modifications and partners govern the switch between them — remains unresolved.
  • no unifying structural model
  • domain architecture mapped only piecemeal
  • context-determining factors between roles unidentified

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0060090 molecular adaptor activity 3 GO:0098772 molecular function regulator activity 3 GO:0005198 structural molecule activity 2
Localization
GO:0005886 plasma membrane 2 GO:0031410 cytoplasmic vesicle 2 GO:0005815 microtubule organizing center 1
Pathway
R-HSA-162582 Signal Transduction 4 R-HSA-1643685 Disease 2 R-HSA-5653656 Vesicle-mediated transport 2 R-HSA-1640170 Cell Cycle 1
Complex memberships
desmosome

Evidence

Reading pass · 15 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
2018 TUFT1 physically interacts with RABGAP1 (a Rab GTPase activating protein), thereby modulating intracellular lysosomal positioning and vesicular trafficking, and promotes mTORC1 signaling activation. Co-immunoprecipitation, cellular fractionation/lysosomal positioning assays, mTORC1 activity assays with TUFT1 knockdown/overexpression Cell discovery High 29423269
2018 TUFT1 promotes HCC cell growth, metastasis and epithelial-mesenchymal transition via activation of the Ca2+/PI3K/AKT pathway. Hypoxia upregulates TUFT1 through HIF-1α-dependent downregulation of miR-671-5p, which targets the 3'-UTR of TUFT1 mRNA. MTT, BrdU, Transwell assays, subcutaneous/tail vein xenograft models, chromatin immunoprecipitation (HIF-1α binding to TUFT1 promoter), microRNA array, luciferase reporter (miR-671-5p/TUFT1 3'-UTR), western blot for pathway activation Oncogene Medium 30250300
2016 TUFT1 promotes pancreatic cancer EMT by altering expression of Snail and is associated with HIF1 expression and activity, suggesting TUFT1 regulates EMT via a HIF1-Snail signaling axis. shRNA knockdown and overexpression, migration/invasion assays, in vivo metastasis models, western blot for EMT markers (E-cadherin, vimentin, Snail), HIF1 activity assays Cancer letters Medium 27566398
2022 TUFT1 is SUMOylated at lysine 79 by the E3 SUMO ligase TRIM27. TUFT1 binds TRIM27 through its N-terminus. SUMOylation-deficient TUFT1 (K79 mutant) fails to activate AKT/mTOR signaling and impairs gastric cancer cell proliferation, migration, and invasion. SUMOylated TUFT1 also forms a complex with RABGAP1 to promote perinuclear accumulation of mTORC1. SUMOylation site mutagenesis, Co-IP, functional assays (proliferation, migration, invasion), western blot for AKT/mTOR phosphorylation, TRIM27 knockdown epistasis Cancer science Medium 36380570
2019 TUFT1 promotes metastasis and chemoresistance in TNBC by activating Rab5 through binding to p85α, which leads to Tiam1 recruitment and subsequent Rac1 activation, with concurrent downregulation of NF-κB pathway and proapoptotic factors. Co-immunoprecipitation (TUFT1-p85α interaction), GTP activity assays (Rab5-GTP, Rac1-GTP), shRNA knockdown, nude mouse xenograft metastasis model, Transwell assays Cancer cell international Medium 31572059
2019 TUFT1 promotes TNBC tumor cell metastasis and stemness by upregulating the Rac1/β-catenin pathway, and lack of TUFT1 sensitizes cells to chemotherapy via downregulation of this pathway. shRNA knockdown, GTP activity assays (Rac1-GTP), migration/invasion assays, sphere formation, apoptosis assay, nude mouse xenograft spontaneous lung metastasis model, western blot Frontiers in oncology Medium 31338333
2020 BRD9 epigenetically upregulates TUFT1 expression by promoting binding of P300 acetyltransferase to the TUFT1 promoter and increasing H3K27Ac at the promoter, thereby activating AKT signaling in HCC cells. ChIP-qPCR (P300 and H3K27Ac at TUFT1 promoter), BRD9 knockdown/inhibition, TUFT1 rescue experiments, western blot for AKT phosphorylation Cell death & disease Medium 32908135
2023 Biallelic loss-of-function TUFT1 variants cause woolly hair and superficial skin fragility. Tuftelin-1 is localized to peripheral cell membranes of keratinocytes in normal skin, and loss of TUFT1 leads to desmosomal detachment, acantholysis, widening of intercellular spaces throughout the epidermis, and perinuclear retraction of intermediate filaments. Whole-genome/exome sequencing, immunofluorescence microscopy (localization in normal vs. patient skin), transmission electron microscopy (desmosomal ultrastructure), haplotype analysis, single-cell RNA sequencing co-expression analysis The British journal of dermatology High 36689522
2023 TUFT1 is a desmosome-associated protein whose location within the desmosome depends on the presence of the desmoplakin carboxy-terminal tail. Loss of TUFT1 causes acantholysis, perinuclear retraction of intermediate filaments, and reduced mechanical stress resistance in keratinocytes. A Tuft1-knockout mouse model recapitulates skin fragility, woolly hair, and palmoplantar keratoderma. Immunolabeling (desmosomal localization), transfection studies (desmoplakin carboxy-terminal tail dependence), Tuft1-knockout mouse model, keratinocyte mechanical stress assays, patient skin histology The Journal of investigative dermatology High 37716648
2017 Overexpression of wild-type or mutant TUFT1 in ATDC5 chondrogenic cells inhibits chondrogenic differentiation (decreased marker gene expression, reduced cartilage nodule formation) in calcifying conditions. The T175M variant increases extracellular matrix calcium content and reduces proteoglycan content compared to wild-type TUFT1 overexpression. HEK293 cell transfection (mRNA/protein expression), ATDC5 overexpression in calcifying conditions, chondrogenic marker gene expression, cartilage nodule formation assay, calcium and proteoglycan content measurement PloS one Medium 28410428
2018 Tuft1 promotes thyroid carcinoma cell invasion and proliferation and suppresses apoptosis through the Akt-mTOR and Akt-GSK3β signaling pathways. Addition of recombinant Tuft1 protein to TC cells increases phosphorylation of Akt, mTOR, and GSK3β; mTOR inhibitor abrogates Tuft1-induced invasion and proliferation, while GSK3β inhibitor abrogates Tuft1-induced proliferation only. shRNA knockdown, recombinant protein treatment, mTOR inhibitor (Dactolisib) and GSK3β inhibitor (CHIR-98014) pharmacological epistasis, western blot for pathway phosphorylation, in vivo xenograft American journal of translational research Medium 30662679
2025 TUFT1 is a centrosomal protein that localizes to the proximal ends of parent centrioles. TUFT1 prevents centrosome amplification and mitotic spindle multipolarity by suppressing premature polo-like kinase 1 (PLK1) activation. TUFT1 is phosphorylated by NEK2 kinase, and the phosphorylation status of TUFT1 is essential for coordinating centrosome number and cell proliferation. Immunofluorescence (centrosomal localization), TUFT1 loss-of-function (centrosome number, spindle multipolarity readouts), PLK1 activity assays, NEK2-mediated phosphorylation assay, western blot, clinical breast cancer sample analysis Cell death & disease Medium 41022752
2026 TUFT1 is a novel TGF-β receptor II (TβRII) binding protein in hepatic stellate cells (HSCs). TUFT1 interacts with TβRII via its N-terminal fragments (amino acids 1-86 and 87-157), protecting TβRII from lysosomal degradation by competing with caveolin-1 for TβRII binding, diverting TβRII from the lipid rafts/caveolae-mediated degradation pathway into the endosome-mediated trafficking and signaling pathway, thereby facilitating TGF-β signaling and myofibroblastic activation of HSCs. Immunoprecipitation coupled with mass spectrometry (TβRII-TUFT1 interaction discovery), Co-IP (domain mapping, caveolin-1 competition), lysosomal degradation assays, TUFT1 knockdown with TGF-β signaling readouts, in vivo HSC/CRC co-implantation and portal vein injection mouse models, bulk RNA sequencing Cell death and differentiation High 41593321
2025 TUFT1 binds to ATF1 (activating transcription factor 1) in cervical cancer cells and positively regulates ATF1 expression. ATF1 overexpression counteracts the effects of TUFT1 silencing on proliferation, migration, invasion, stemness, and EGFR signaling. Co-IP assay (TUFT1-ATF1 interaction), RT-qPCR and western blot (ATF1 expression after TUFT1 silencing), epistasis rescue (co-transfection of TUFT1 siRNA + ATF1 overexpression), functional assays Cell biochemistry and biophysics Low 39948288
2022 ATF2 transcription factor directly targets the miR-548p promoter (confirmed by ChIP and luciferase assay), and miR-548p directly binds the 3'-UTR of TUFT1 to suppress its expression, thereby suppressing PI3K/AKT signaling in HCC. Chromatin immunoprecipitation (ATF2 binding to miR-548p promoter), luciferase reporter assay (miR-548p binding to TUFT1 3'-UTR), western blot for PI3K/AKT signaling, functional assays Hepatology research Low 34904343

Source papers

Stage 0 corpus · 34 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
2018 Hypoxia-induced TUFT1 promotes the growth and metastasis of hepatocellular carcinoma by activating the Ca2+/PI3K/AKT pathway. Oncogene 105 30250300
2019 Exosomal lncRNA HNF1A-AS1 affects cisplatin resistance in cervical cancer cells through regulating microRNA-34b/TUFT1 axis. Cancer cell international 74 31827397
2016 TUFT1 regulates metastasis of pancreatic cancer through HIF1-Snail pathway induced epithelial-mesenchymal transition. Cancer letters 47 27566398
2018 TUFT1 interacts with RABGAP1 and regulates mTORC1 signaling. Cell discovery 40 29423269
2020 Long noncoding RNA LINC01123 promotes the proliferation and invasion of hepatocellular carcinoma cells by modulating the miR-34a-5p/TUFT1 axis. International journal of biological sciences 36 32760198
2020 Bromodomain-containing protein 9 promotes the growth and metastasis of human hepatocellular carcinoma by activating the TUFT1/AKT pathway. Cell death & disease 35 32908135
2020 LncRNA DANCR upregulation induced by TUFT1 promotes malignant progression in triple negative breast cancer via miR-874-3p-SOX2 axis. Experimental cell research 33 33058834
2019 TUFT1 Promotes Triple Negative Breast Cancer Metastasis, Stemness, and Chemoresistance by Up-Regulating the Rac1/β-Catenin Pathway. Frontiers in oncology 32 31338333
2017 TUFT1, a novel candidate gene for metatarsophalangeal osteoarthritis, plays a role in chondrogenesis on a calcium-related pathway. PloS one 25 28410428
2023 m6A modified BACE1-AS contributes to liver metastasis and stemness-like properties in colorectal cancer through TUFT1 dependent activation of Wnt signaling. Journal of experimental & clinical cancer research : CR 24 37986103
2017 TUFT1 is expressed in breast cancer and involved in cancer cell proliferation and survival. Oncotarget 22 29088838
2022 Hsa_circ_0074269-mediated Upregulation of TUFT1 Through miR-485-5p Increases Cisplatin Resistance in Cervical Cancer. Reproductive sciences (Thousand Oaks, Calif.) 20 35075616
2019 TUFT1 promotes metastasis and chemoresistance in triple negative breast cancer through the TUFT1/Rab5/Rac1 pathway. Cancer cell international 19 31572059
2021 Tuftelin 1 (TUFT1) Promotes the Proliferation and Migration of Renal Cell Carcinoma via PI3K/AKT Signaling Pathway. Pathology oncology research : POR 18 34257606
2019 Association of ENAM, TUFT1, MMP13, IL1B, IL10 and IL1RN gene polymorphism and dental caries susceptibility in Chinese children. The Journal of international medical research 18 30803280
2018 Tuft1 promotes thyroid carcinoma cell invasion and proliferation and suppresses apoptosis through the Akt-mTOR/GSK3β signaling pathway. American journal of translational research 17 30662679
2020 MiR-671-5p plays a promising role in restraining osteosarcoma cell characteristics through targeting TUFT1. Journal of biochemical and molecular toxicology 16 32115852
2022 LINC00960 regulates cell proliferation and glycolysis in pancreatic cancer through the miR-326-3p/TUFT1/AKT-mTOR axis. The Kaohsiung journal of medical sciences 12 36149758
2022 SUMOylation of TUFT1 is essential for gastric cancer progression through AKT/mTOR signaling pathway activation. Cancer science 11 36380570
2021 TUFT1 Facilitates Metastasis, Stemness, and Vincristine Resistance in Colorectal Cancer via Activation of PI3K/AKT Pathway. Biochemical genetics 11 33634374
2023 Biallelic TUFT1 variants cause woolly hair, superficial skin fragility and desmosomal defects. The British journal of dermatology 9 36689522
2023 microRNA-128-3p inhibits proliferation and accelerates apoptosis of gastric cancer cells via inhibition of TUFT1. World journal of surgical oncology 8 36797791
2022 Construction and protective efficacy of a novel Streptococcus pneumoniae fusion protein vaccine NanAT1-TufT1-PlyD4. Frontiers in immunology 8 36389808
2023 In silico screening of non-synonymous SNPs in human TUFT1 gene. Journal, genetic engineering & biotechnology 6 37801178
2023 Disruption of TUFT1, a Desmosome-Associated Protein, Causes Skin Fragility, Woolly Hair, and Palmoplantar Keratoderma. The Journal of investigative dermatology 3 37716648
2022 ATF2 accelerates the invasion and metastasis of hepatocellular carcinoma through targeting the miR-548p/TUFT1 axis. Hepatology research : the official journal of the Japan Society of Hepatology 3 34904343
2022 MicroRNA-145 regulates the proliferation of the human gastric cancer cells by targeting tuftelin 1 (TUFT1). Acta biochimica Polonica 2 35616620
2021 Erratum: Long noncoding RNA LINC01123 promotes the proliferation and invasion of hepatocellular carcinoma cells by modulating the miR-34a-5p/TUFT1 axis: Erratum. International journal of biological sciences 2 34239359
2025 TUFT1 Modulates Cell Proliferation, Migration, Invasion, Stemness and EGFR Signaling in Cervical Cancer through Interacting with and Targeting ATF1. Cell biochemistry and biophysics 1 39948288
2021 Erratum: Long noncoding RNA LINC01123 promotes the proliferation and invasion of hepatocellular carcinoma cells by modulating the miR-34a-5p/TUFT1 axis: Erratum. International journal of biological sciences 1 33994858
2026 TUFT1 stabilizes TGF-β receptor II protein and facilitates activation of hepatic stellate cells into metastasis-promoting myofibroblasts. Cell death and differentiation 0 41593321
2025 Expression of AMELX, AMBN, ENAM, TUFT1, FAM83H and MMP20 Genes in Buccal Epithelial Cells from Patients with Molar Incisor Hypomineralization (MIH)-A Pilot Study. International journal of molecular sciences 0 39859478
2025 TUFT1 regulates cancer progression by suppressing centrosome amplification and mitotic spindle multipolarity. Cell death & disease 0 41022752
2024 Assessment of TUFT1 and Rac1-GTP levels in triple-negative breast cancer patients: clinical and pathological correlations. Clinical & translational oncology : official publication of the Federation of Spanish Oncology Societies and of the National Cancer Institute of Mexico 0 38478261

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