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USP53

Ubiquitin carboxyl-terminal hydrolase 53 · UniProt Q70EK8

Length
1073 aa
Mass
120.8 kDa
Annotated
2026-04-28
33 papers in source corpus 20 papers cited in narrative 20 extracted findings

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

USP53 is a K63-linkage-selective deubiquitinase that operates at the intersection of tight junction maintenance, bile acid secretion, cell death signaling, bone homeostasis, and tumor suppression across multiple tissues. Despite its prior annotation as a catalytically inactive pseudo-DUB, USP53 harbors an active USP domain with K63-specific S2-ubiquitin-binding sites and deubiquitinates diverse substrates including TJP1/TJP2 (tight junction scaffolding), MYO5B (BSEP trafficking to the canalicular membrane), RIPK1 (K63-deubiquitination at K377 triggering necroptosis/apoptosis), cytochrome c (apoptosis), FKBP51 (AKT1 suppression), NOTCH2 (NF-κB signaling in microglia), MORF4L1, ZMYND11, SMAD5, and SR-A (PMID:26609154, PMID:40828662, PMID:40822127, PMID:35654790, PMID:39044157, PMID:38904030). In bone, USP53 enhances VDR–SMAD3 interaction to suppress RANKL, coupling osteoblast and osteoclast activity, and Usp53-null mice display low bone mass (PMID:36726200). Biallelic loss-of-function variants in USP53 cause low-GGT intrahepatic cholestasis with sensorineural hearing loss, reflecting its essential roles in hepatocyte tight junction integrity and bile transporter expression (PMID:30250217, PMID:39705897).

Mechanistic history

Synthesis pass · year-by-year structured walk · 12 steps
  1. 2015 High

    Forward genetics revealed that USP53 — then annotated as a catalytically dead pseudo-DUB — physically associates with tight junction scaffolds TJP1/TJP2 and is essential for auditory hair cell survival and endocochlear potential maintenance, establishing it as a functional component of epithelial tight junctions.

    Evidence ENU mutagenesis (mambo mouse), reciprocal Co-IP, colocalization imaging, cochlear organotypic cultures, biotin tracer assay

    PMID:26609154

    Open questions at the time
    • Catalytic activity was not tested and assumed absent
    • The mechanism by which USP53 supports tight junction integrity was unknown
    • No liver or bone phenotype examined
  2. 2018 Medium

    Human genetics linked biallelic USP53 truncating variants to low-GGT intrahepatic cholestasis and hearing loss, extending USP53's tight-junction role from the inner ear to hepatocytes and establishing a Mendelian disease association.

    Evidence Exome sequencing and segregation analysis in multiple families

    PMID:30250217

    Open questions at the time
    • No biochemical or cell-biological experiments in this study
    • Mechanism linking USP53 loss to cholestasis was unresolved
    • No animal model of liver phenotype
  3. 2020 Medium

    USP53 was shown to deubiquitinate specific substrates — FKBP51 (suppressing AKT1/glycolysis in lung adenocarcinoma) and cytochrome c (promoting apoptosis in HCC) — establishing that it functions as an active deubiquitinase with tumor-suppressive roles, contradicting its pseudo-enzyme annotation.

    Evidence Co-IP, ubiquitination assays, cycloheximide chase, GSEA, in vivo xenograft, rescue experiments

    PMID:32511815 PMID:35654790

    Open questions at the time
    • Linkage specificity (K48 vs K63) not resolved
    • No in vitro reconstitution of catalytic activity
    • Single-lab observations without structural confirmation of active site
  4. 2020 Medium

    Ultrastructural analysis of USP53-mutant patient livers revealed elongated hepatocyte tight junctions, providing direct morphological evidence that USP53 regulates tight junction architecture in human liver.

    Evidence Transmission electron microscopy of liver biopsies from patients with biallelic USP53 variants

    PMID:32124521

    Open questions at the time
    • Mechanism of junction elongation unclear
    • No molecular targets identified in this study
    • Small patient cohort
  5. 2021 High

    USP53 was placed downstream of PTH/NACA signaling in osteoblasts; its knockdown promoted osteoblast over adipocyte commitment, revealing a role in mesenchymal lineage determination.

    Evidence ChIP-seq, EMSA, luciferase reporter, shRNA knockdown in ST2 stromal cells, in vivo implantation

    PMID:33875709

    Open questions at the time
    • No DUB substrates identified in bone context at this point
    • No Usp53 knockout bone phenotype yet reported
  6. 2023 High

    Usp53-null mice exhibited low bone mass and elevated RANKL due to loss of USP53-mediated enhancement of VDR–SMAD3 interaction, revealing a specific molecular mechanism for USP53's role in osteoblast-osteoclast coupling.

    Evidence Usp53 knockout mice, micro-CT, histomorphometry, serum RANKL ELISA, VDR–SMAD3 Co-IP

    PMID:36726200

    Open questions at the time
    • Whether USP53 directly deubiquitinates VDR or SMAD3 was not tested
    • Bone phenotype rescue not demonstrated
    • Contribution of adipocyte vs osteoblast compartment not dissected
  7. 2024 High

    Liver-specific Usp53 knockout confirmed that hepatic USP53 interacts with TJP2 and regulates tight junction length and bile acid transporter expression (BSEP/NTCP/MRP2), providing a mechanistic bridge between tight junction biology and cholestasis.

    Evidence Alb-cre × Usp53fl/fl mice, Co-IP, immunofluorescence, electron microscopy, DDC dietary challenge

    PMID:39705897

    Open questions at the time
    • Whether USP53 directly deubiquitinates TJP2 or another junction component unclear
    • Compensatory mechanisms in liver-specific KO not defined
  8. 2024 High

    Biochemical reconstitution overturned the pseudo-enzyme annotation: USP53 is an active deubiquitinase with high selectivity for K63-linked polyubiquitin, with structural K63-specific S2-ubiquitin-binding sites, and patient-associated mutations abolish catalytic activity.

    Evidence (preprint) In vitro DUB assays, structural analysis, site-directed mutagenesis of patient variants, K63-ubiquitination profiling of tricellular junction components

    PMID:bio_10.1101_2024.07.07.602376

    Open questions at the time
    • Awaits peer review
    • Full crystal/cryo-EM structure not yet deposited
    • In vivo validation of K63-selectivity for specific substrates needed
  9. 2024 High

    USP53 was shown to deubiquitinate SR-A via its USP domain (Cys41-dependent) removing K48-linked ubiquitin, promoting foam cell formation during atherosclerosis — notably demonstrating K48-directed activity alongside the predominant K63 specificity.

    Evidence Co-IP, Cys41 mutagenesis, ChIP, AAV-SMC-specific OE in DKK1ECKO/APOE−/− mice, parallel-plate co-culture

    PMID:38904030

    Open questions at the time
    • Apparent K48 activity on SR-A conflicts with predominant K63 selectivity; reconciliation needed
    • Single disease context (atherosclerosis)
  10. 2025 High

    The cholestasis mechanism was resolved at the trafficking level: USP53 loss causes MYO5B hyperubiquitination and BSEP mistrafficking to recycling endosomes; USP53 binds the MYO5B IQ domain, and a cholestasis-associated MYO5B variant disrupts this interaction.

    Evidence CRISPR KO, surface biotinylation, FRAP, Co-IP with mutagenesis, confocal imaging in patient tissue

    PMID:40828662

    Open questions at the time
    • Ubiquitin linkage type on MYO5B not specified
    • Whether USP53 regulates other apical transporters via MYO5B not tested
  11. 2025 High

    USP53 was shown to remove K63-ubiquitin from RIPK1 at K377, licensing RIPK1 autophosphorylation and triggering necroptosis/apoptosis in cardiomyocytes, expanding USP53's cell death regulatory repertoire beyond cytochrome c and FKBP51.

    Evidence Co-IP/LC-MS/MS, site-specific K63-ubiquitination mapping, cardiomyocyte-specific USP53 KO mice with alcohol feeding

    PMID:40822127

    Open questions at the time
    • Whether RIPK1-K377 deubiquitination by USP53 occurs in non-cardiac tissues unknown
    • Kinetics and regulation of USP53 recruitment to complex I/II not defined
  12. 2025 Medium

    Additional substrates were identified — NOTCH2 (microglia/neuroinflammation), MORF4L1 (colorectal cancer), ZMYND11 (breast cancer), and SMAD5 (osteogenic differentiation) — broadening the substrate repertoire and reinforcing USP53's role as a multi-tissue DUB acting on both signaling and chromatin-associated proteins.

    Evidence Co-IP, ubiquitination assays with site mapping (K249/K227 for MORF4L1), domain mutagenesis (Cys-box for ZMYND11), xenografts, in vivo KO (5XFAD mice for NOTCH2)

    PMID:39044157 PMID:40481141 PMID:41061828 PMID:41482165

    Open questions at the time
    • Substrate prioritization unclear — which are physiologically dominant
    • K63 vs K48 linkage not determined for all new substrates
    • SMAD5 result from single lab without in vivo validation

Open questions

Synthesis pass · forward-looking unresolved questions
  • How USP53 achieves substrate selectivity across such diverse targets, whether its K63 selectivity is absolute or context-dependent (given reported K48 activity on SR-A), and the structural basis for substrate recognition outside the catalytic domain remain open questions.
  • No high-resolution structure of USP53 bound to a substrate or ubiquitin chain
  • Systematic profiling of linkage preference across substrates not performed
  • Tissue-specific regulation of USP53 expression and activation incompletely mapped

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0140096 catalytic activity, acting on a protein 11 GO:0016787 hydrolase activity 2
Localization
GO:0005886 plasma membrane 4 GO:0005634 nucleus 2 GO:0005829 cytosol 2 GO:0005768 endosome 1
Pathway
R-HSA-1500931 Cell-Cell communication 4 R-HSA-5357801 Programmed Cell Death 4 R-HSA-162582 Signal Transduction 3 R-HSA-1643685 Disease 3 R-HSA-382551 Transport of small molecules 3 R-HSA-392499 Metabolism of proteins 3
Partners
FKBP51MORF4L1MYO5BNOTCH2RIPK1TJP1TJP2ZMYND11

Evidence

Reading pass · 20 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
2015 USP53 contains a catalytically inactive ubiquitin-specific protease domain (annotated as pseudo-enzyme) and colocalizes and physically interacts with tight junction scaffolding proteins TJP1 and TJP2 in polarized epithelial cells, placing USP53 as a component of the tight junction complex essential for auditory hair cell survival. Co-immunoprecipitation, colocalization imaging, forward genetics (ENU mutagenesis mambo mouse line), cochlear organotypic cultures, biotin tracer assay, electrophysiology (endocochlear potential measurement) The Journal of neuroscience High 26609154
2018 USP53 was identified as an interactor of TJP2, and homozygous truncating variants in USP53 cause low-GGT intrahepatic cholestasis and hearing loss, linking USP53 function to tight junction integrity in the liver and inner ear. Exome sequencing, positional mapping, human genetics (segregation analysis) Genetics in medicine Medium 30250217
2020 USP53 deubiquitinates FKBP51, stabilizing it; FKBP51 in turn dephosphorylates AKT1, thereby suppressing AKT1 pathway activity, inhibiting glycolysis, and promoting apoptosis in lung adenocarcinoma cells. Co-immunoprecipitation, ubiquitination assay, stable overexpression/shRNA knockdown, AKT pathway inhibitor (LY294002), in vivo xenograft Molecular carcinogenesis Medium 32511815
2020 Ultrastructural study of liver biopsies from patients with USP53 mutations revealed elongated hepatocyte-hepatocyte tight junctions, implicating USP53 in tight junction structure/length regulation in hepatocytes. Transmission electron microscopy of liver biopsies from patients with biallelic USP53 variants Liver international Medium 32124521
2021 USP53 is identified as a target gene of PTH-activated NACA in osteoblasts; NACA binds the USP53 promoter (confirmed by EMSA), and USP53 promoter activity is regulated by the JUN-CREB complex via activated PKA and NACA. Usp53 knockdown in stromal cells promotes osteoblast markers and inhibits adipogenic markers, establishing a role in mesenchymal lineage commitment. ChIP-seq, RNA-seq, EMSA, luciferase reporter assay, shRNA knockdown in ST2 stromal cells, in vivo implantation of knockdown cells in immunocompromised mice Scientific reports High 33875709
2022 USP53 interacts with cytochrome c (CYCS) and deubiquitinates it, increasing CYCS stability; overexpression of CYCS rescues the decreased apoptosis caused by USP53 knockdown in hepatocellular carcinoma cells, establishing a USP53-CYCS apoptotic axis. Co-immunoprecipitation, mass spectrometry, cycloheximide chase assay, GSEA, rescue experiment with CYCS overexpression Oncogenesis Medium 35654790
2023 USP53 regulates RANKL expression by enhancing the interaction between VDR and SMAD3 in osteoblasts and bone marrow adipocytes; Usp53 null mice display low bone mass, increased osteoclastogenesis, and elevated serum RANKL, establishing USP53 as a regulator of osteoblast-osteoclast coupling. Usp53 knockout mice, micro-CT (trabecular and cortical bone quantification), histomorphometry, serum RANKL ELISA, mechanistic interaction assay (VDR-SMAD3 co-immunoprecipitation) Journal of bone and mineral research High 36726200
2023 USP53 directly binds and deubiquitinates CRKL to stabilize it, thereby promoting TNBC cell proliferation, migration, invasion, EMT, and chemoresistance. Co-immunoprecipitation, ubiquitination assay, gain/loss-of-function cell experiments Cancers Low 37894400
2023 USP53 localizes to hippocampal CA1-3 regions and granular dentate, and co-immunoprecipitates with GRIA2/GluA2 (AMPA receptor subunit) and GRIP2, placing USP53 within the AMPA receptor interactome in the brain. Immunofluorescence (co-localization with GRIA2/GluA2 and GRIP2 antibodies), co-immunoprecipitation Genes Low 37895270
2024 USP53 binds SR-A via its USP domain (requiring Cys41) and removes K48-linked ubiquitin chains from SR-A, preventing its proteasomal degradation and thereby promoting foam cell formation in smooth muscle cells during atherosclerosis. DKK1 regulates USP53 transcription by facilitating CREB binding to the USP53 promoter. Co-immunoprecipitation, RNA-seq, site-directed mutagenesis (Cys41), ChIP, adeno-associated virus SMC-specific overexpression in DKK1ECKO/APOE-/- mice, parallel-plate co-culture flow system, endothelium-specific KO and OE mouse models International journal of biological sciences High 38904030
2024 USP53 interacts with ZMYND11 and catalyzes its deubiquitination and stabilization; the 33–50 amino acid Cys-box domain is required for USP53 enzymatic activity but not for ZMYND11 binding; USP53-mediated stabilization of ZMYND11 mediates USP53's anti-tumor effects in breast cancer. Co-immunoprecipitation, domain deletion/mutagenesis (Cys-box), ubiquitination assay, rescue experiments with ZMYND11 overexpression, xenograft Biological procedures online Medium 39044157
2024 USP53 interacts with TJP2 in hepatocytes (confirmed by co-immunoprecipitation and co-immunofluorescence); loss of hepatic USP53 causes upregulation of Tjp2 mRNA, lengthening of hepatocellular tight junctions, and downregulation of bile acid transporters (Abcb11, Ntcp, Abcc2), protecting mice from DDC-induced liver injury. Usp53 liver-specific knockout mice (Alb-cre × Usp53fl/fl), co-immunoprecipitation, immunofluorescence, electron microscopy (tight junction length), qRT-PCR, dietary challenge (DDC) Biochimica et biophysica acta. Molecular basis of disease High 39705897
2024 USP53 and USP54, previously annotated as catalytically inactive pseudo-DUBs, are in fact active deubiquitinases with high selectivity for K63-linked polyubiquitin chains. USP53 can deubiquitinate a substrate in a K63-linkage-dependent manner. Patient-associated mutations in the USP domain of USP53 abrogate this catalytic activity. Depletion of USP53 increases K63-linked ubiquitination of tricellular junction components. Structural analyses identified K63-specific S2-ubiquitin-binding sites within the catalytic domain. Biochemical in vitro DUB activity assays, structural analysis, site-directed mutagenesis of patient variants, K63-linkage-specific ubiquitin chain assays, mass spectrometry, depletion of USP53 followed by K63-ubiquitination profiling of tricellular junction components bioRxivpreprint High bio_10.1101_2024.07.07.602376
2025 Loss of USP53 causes mislocalization of the bile salt export pump (BSEP) to MYO5B-positive and RAB11A-positive recycling endosomes, impairing BSEP trafficking to the plasma membrane. USP53 colocalizes with MYO5B and interacts with its IQ domain; the cholestasis-associated MYO5B p.(Arg824Cys) variant (located in the IQ domain) fails to interact with USP53. Loss of USP53 increases ubiquitination of MYO5B and disrupts its endosomal recruitment. Immunohistochemistry of patient tissue, confocal immunofluorescence, surface protein biotinylation, siRNA knockdown, CRISPR-Cas9 knockout, site-directed mutagenesis, co-immunoprecipitation, live cell imaging, FRAP Hepatology High 40828662
2025 USP53 interacts with the intermediate domain of RIPK1 and removes K63-linked ubiquitin chains at RIPK1 lysine-377, facilitating RIPK1 autophosphorylation and triggering downstream apoptotic and necroptotic pathways in cardiomyocytes during ethanol-induced injury. USP53 transcription is induced by EGR1 in response to ethanol. Co-immunoprecipitation combined with LC-MS/MS, site-specific ubiquitination mapping, cardiomyocyte-specific USP53 knockout (USP53CKO) mice, in vivo alcohol feeding model, western blot, ChIP Research (Washington, D.C.) High 40822127
2025 USP53 interacts with and deubiquitinates NOTCH2 in microglia; USP53 knockout reduces Aβ-induced NOTCH2 deubiquitination, suppresses IKKβ/NFκB signaling, reduces neuroinflammatory mediators (IL-1β, TNF-α), and improves learning/memory in 5XFAD Alzheimer's disease mice. Co-immunoprecipitation, USP53 knockout in 5XFAD mice, Morris Water Maze, western blot, immunofluorescence, qRT-PCR Neurochemistry international Medium 41482165
2025 USP53 deubiquitinates MORF4L1 at K249 and K227, preventing its ubiquitination and proteasomal degradation, thereby suppressing colorectal cancer cell proliferation; MORF4L1 overexpression rescues the pro-tumorigenic effects of USP53 silencing. Immunoprecipitation–LC/MS (ubiquitylome), Co-IP, ubiquitination assay with site identification (K249, K227), gain/loss-of-function experiments, xenograft Biochimica et biophysica acta. Molecular basis of disease Medium 41061828
2025 USP53 deubiquitinates SMAD5 to stabilize its protein expression in bone marrow stromal cells, promoting their viability, invasion, and osteogenic differentiation; EIF4A3 stabilizes USP53 mRNA to maintain USP53 protein levels, forming an EIF4A3-USP53-SMAD5 regulatory axis. Co-immunoprecipitation, western blot, knockdown/overexpression experiments, ALP activity assay, Alizarin red staining, qRT-PCR Scientific reports Low 40481141
2025 FOS transcription factor directly regulates USP53 expression; juglone suppresses FOS, downregulating USP53, which reduces USP53-dependent deubiquitination of MLKL and GSDMD, promoting their ubiquitination and degradation and thereby alleviating necroptosis and pyroptosis in injured neurons. RNA-seq, immunoprecipitation, western blot, qRT-PCR, ChIP-qPCR, OGD/R neuronal model, in vivo spinal cord injury model Phytomedicine Medium 41045624
2025 ATF3 transcriptionally represses USP53 by binding its promoter; USP53 promotes adipogenesis and activates the RhoA/ROCK signaling pathway; ATF3-mediated USP53 repression inhibits adipocyte differentiation of 3T3-L1 cells. ChIP assay, luciferase reporter assay, Oil Red O staining, triglyceride measurement, western blot (RhoA/ROCK pathway), ATF3 and USP53 overexpression/knockdown Journal of molecular endocrinology Medium 39641389

Source papers

Stage 0 corpus · 33 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
2018 Identification of novel loci for pediatric cholestatic liver disease defined by KIF12, PPM1F, USP53, LSR, and WDR83OS pathogenic variants. Genetics in medicine : official journal of the American College of Medical Genetics 85 30250217
2015 Progressive Hearing Loss in Mice Carrying a Mutation in Usp53. The Journal of neuroscience : the official journal of the Society for Neuroscience 56 26609154
2020 Low-GGT intrahepatic cholestasis associated with biallelic USP53 variants: Clinical, histological and ultrastructural characterization. Liver international : official journal of the International Association for the Study of the Liver 41 32124521
2020 USP53 promotes apoptosis and inhibits glycolysis in lung adenocarcinoma through FKBP51-AKT1 signaling. Molecular carcinogenesis 31 32511815
2021 Cholestasis Due to USP53 Deficiency. Journal of pediatric gastroenterology and nutrition 29 33075013
2020 New paradigms of USP53 disease: normal GGT cholestasis, BRIC, cholangiopathy, and responsiveness to rifampicin. Journal of human genetics 29 32759993
2023 USP53 Regulates Bone Homeostasis by Controlling Rankl Expression in Osteoblasts and Bone Marrow Adipocytes. Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research 20 36726200
2022 USP53 plays an antitumor role in hepatocellular carcinoma through deubiquitination of cytochrome c. Oncogenesis 19 35654790
2021 Ubiquitin specific peptidase Usp53 regulates osteoblast versus adipocyte lineage commitment. Scientific reports 16 33875709
2022 USP53 activated by H3K27 acetylation regulates cell viability, apoptosis and metabolism in esophageal carcinoma via the AMPK signaling pathway. Carcinogenesis 13 34919659
2021 Progressive Familial Intrahepatic Cholestasis Associated With USP53 Gene Mutation in a Brazilian Child. Journal of pediatric gastroenterology and nutrition 11 33661244
2021 A Two-Year Clinical Description of a Patient with a Rare Type of Low-GGT Cholestasis Caused by a Novel Variant of USP53. Genes 9 34681012
2021 Biallelic Mutations in Ubiquitin-Specific Peptidase 53 (USP53) Causing Progressive Intrahepatic Cholestasis. Report of a Case With Review of Literature. Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society 9 34809518
2024 USP53 Affects the Proliferation and Apoptosis of Breast Cancer Cells by Regulating the Ubiquitination Level of ZMYND11. Biological procedures online 8 39044157
2024 An Overview of the Deubiquitinase USP53: A Promising Diagnostic Marker and Therapeutic Target. Current protein & peptide science 8 39300775
2022 Biallelic Novel USP53 Splicing Variant Disrupting the Gene Function that Causes Cholestasis Phenotype and Review of the Literature. Molecular syndromology 8 36660033
2024 Endothelial Dickkopf-1 Promotes Smooth Muscle Cell-derived Foam Cell Formation via USP53-mediated Deubiquitination of SR-A During Atherosclerosis. International journal of biological sciences 7 38904030
2023 USP53 Exerts Tumor-Promoting Effects in Triple-Negative Breast Cancer by Deubiquitinating CRKL. Cancers 4 37894400
2024 Loss of hepatocyte Usp53 protects mice from a form of xenobiotic-induced liver injury. Biochimica et biophysica acta. Molecular basis of disease 3 39705897
2023 Genome Sequencing of Consanguineous Family Implicates Ubiquitin-Specific Protease 53 (USP53) Variant in Psychosis/Schizophrenia: Wild-Type Expression in Murine Hippocampal CA 1-3 and Granular Dentate with AMPA Synapse Interactions. Genes 3 37895270
2025 USP53 Drives Ethanol-Induced Myocardial Injury by Promoting K63 Deubiquitination-Dependent RIPK1 Activation at K377. Research (Washington, D.C.) 2 40822127
2025 Juglone promotes spinal cord injury recovery by suppressing pyroptosis and necroptosis through FOS/USP53/ubiquitination. Phytomedicine : international journal of phytotherapy and phytopharmacology 2 41045624
2024 Natural course and outcomes of children with ubiquitin-specific protease 53 (USP53)-related genetic chronic cholestasis. Journal of pediatric gastroenterology and nutrition 2 39440620
2023 A novel homozygous mutation in the USP53 gene as the cause of benign recurrent intrahepatic cholestasis in children: a case report. The Turkish journal of pediatrics 2 38204320
2023 Cholestatic Liver Disease due to Novel USP53 Mutations: A Case Series of Three Indian Children. Journal of clinical and experimental hepatology 2 38544763
2025 EIF4A3 enhances the viability, invasion and osteogenic differentiation of BMSCs via the USP53/SMAD5 pathway. Scientific reports 1 40481141
2023 The First Korean Adult Case of Progressive Familial Intrahepatic Cholestasis Type 7 with Novel USP53 Splicing Variants by Next Generation Sequencing. Yonsei medical journal 1 37992747
2025 ATF3 suppresses 3T3-L1 adipocyte adipogenesis by transcriptionally repressing USP53. Journal of molecular endocrinology 0 39641389
2025 A novel mechanism involving USP53-regulated BSEP trafficking underlies low-GGT intrahepatic cholestasis. Hepatology (Baltimore, Md.) 0 40828662
2025 Deubiquitinase USP53 suppressed tumorigenesis of colorectal cancer cells by mediating deubiquitination of MORF4L1. Biochimica et biophysica acta. Molecular basis of disease 0 41061828
2025 Case Report: Mild BRIC-like cholestasis despite a gross USP53 deletion-novel findings and literature review. Frontiers in genetics 0 41356217
2025 USP53 promotes NOTCH2-induced neuroinflammation in Alzheimer's disease. Neurochemistry international 0 41482165
2024 A novel case report of benign recurrent intrahepatic cholestasis-associated USP53 genetic mutation in a Pakistani girl. SAGE open medical case reports 0 39071191