Affinage

LONP1

Lon protease homolog, mitochondrial · UniProt P36776

Length
959 aa
Mass
106.5 kDa
Annotated
2026-06-10
100 papers in source corpus 35 papers cited in narrative 34 extracted findings
Cross-family judge vs UniProt: Affinage preferred faithfulness: 9/9 claims corpus-supported (100%)

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

LONP1 is an ATP-dependent AAA+ serine protease of the mitochondrial matrix that governs mitochondrial proteostasis by selectively degrading regulatory, misfolded, and oxidatively damaged proteins while doubling as an ATP-dependent chaperone (PMID:33431889, PMID:30061372, PMID:38932681, PMID:41900996). Its enzyme cycle is built on a bipartite "search-and-shred" architecture: the N-terminal domain acts as a selective vestibule that recruits and initially unfolds substrate, after which polypeptide is threaded through a central channel by aromatic pore-loops in a hand-over-hand mechanism, with a second ATPase-domain spiral staircase engaging substrate during translocation (PMID:34050165, PMID:38932681, PMID:41900996). Critically, ATPase and protease activities are decoupled—substrate occupancy of both the central channel and the protease active site is required to license the activated conformation—and an ADP-bound C3-symmetric intermediate is positioned to sense folded substrate before unfoldase activation (PMID:34050165, PMID:40950177). Through this proteolysis LONP1 sets the levels of a broad regulated substrate set spanning heme synthesis (ALAS-1), steroidogenesis (CYP11A1), mtDNA maintenance and transcription (TFAM, PolG), central carbon and one-carbon metabolism (phospho-PDH E1α, SDHB, ACO2, MPC1, SHMT2), ketogenesis and pyrimidine metabolism (HMGCS2, DHODH), and disease-associated misfolded proteins (DJ-1 mutants) (PMID:21659532, PMID:38870290, PMID:30304514, PMID:37217599, PMID:36629048, PMID:33637676, PMID:38932681, PMID:41900996, PMID:40784490, PMID:40332105). Independently of proteolysis, its ATPase-driven chaperone activity cooperates with the mtHSP70/DNAJA3 system to fold matrix proteins and assemble respiratory complexes (PMID:33431889, PMID:40691304). LONP1 activity is tuned by Akt phosphorylation at Ser173/181, reversible oxidative modifications, Sirt3 deacetylation at K145, and FBXO11-mediated K63-ubiquitination that drives its mitochondrial import (PMID:31406245, PMID:24740269, PMID:36739437, PMID:41289019). Loss-of-function and gain-of-function studies link LONP1 to muscle, cardiac, β-cell, hepatic, and oocyte homeostasis, and to integrated stress and unfolded-protein responses (PMID:35173176, PMID:30625302, PMID:34974310, PMID:35237793, PMID:40691304). Pathogenic LONP1 mutations clustering in the AAA+ and N-terminal domains cause CODAS syndrome and mitochondrial encephalopathy/neurodegeneration through substrate-specific proteolytic defects and imbalance of protease versus chaperone function (PMID:25574826, PMID:30304514, PMID:29518248, PMID:31923470). Beyond the matrix, LONP1 is detected at mitochondria-ER contact sites as a MAM tethering factor and in the nucleus, where it interacts with HSF1 (PMID:37333972, PMID:35760833).

Mechanistic history

Synthesis pass · year-by-year structured walk · 16 steps
  1. 2011 High

    Established that LONP1 is not merely a bulk quality-control protease but a regulated effector of a metabolic pathway, by identifying its role in feedback control of heme biosynthesis.

    Evidence siRNA and pharmacological inhibition with pulse-chase turnover of ALAS-1 in human liver cells

    PMID:21659532

    Open questions at the time
    • Direct in vitro proteolysis of ALAS-1 not reconstituted
    • Mechanism of heme-induced substrate recognition unresolved
  2. 2014 High

    Showed that LONP1 proteolytic activity is itself redox-regulated, linking oxidative damage to its own functional decline in disease.

    Evidence FITC-casein activity assays, oxidative modification immunoblotting, in vivo siRNA, and mitochondria-targeted antioxidant rescue in failing mouse hearts

    PMID:24740269

    Open questions at the time
    • Specific oxidized residues controlling activity not mapped
    • Which ETC substrates accumulate not fully defined
  3. 2015 High

    Connected LONP1 to human Mendelian disease, defining CODAS syndrome and demonstrating that AAA+-domain mutations cause substrate-specific proteolytic and oligomerization defects.

    Evidence Recombinant mutant proteolysis assays, native-gel oligomerization, and electron microscopy of patient lymphoblastoid mitochondria

    PMID:25574826

    Open questions at the time
    • Tissue specificity of clinical phenotype unexplained
    • Full substrate spectrum disrupted by mutations unknown
  4. 2018 High

    Defined a broad LONP1-dependent maturation and turnover network and showed its depletion triggers integrated stress, PINK1 stabilization, and loss of mtDNA/translation, framing LONP1 as central to matrix proteostasis.

    Evidence Catalytic-dead mutant substrate trapping, MPP epistasis, quantitative proteomics, and siRNA

    PMID:30061372

    Open questions at the time
    • Direct versus indirect substrate processing not always distinguished
    • Mechanism linking depletion to ribosome biogenesis loss unclear
  5. 2018 High

    Provided biochemical mechanism for compound heterozygous mitochondrial disease by showing differential proteolytic complementation between mutant LonP1 subunits within hexamers.

    Evidence In vitro proteolysis of purified single and mixed mutant LonP1 proteins

    PMID:29518248

    Open questions at the time
    • Hexamer subunit stoichiometry in patients not measured
    • Cellular consequences not directly assayed
  6. 2019 High

    Identified post-translational control of LONP1 by Akt phosphorylation and a specific substrate (phospho-PDH E1α), linking LONP1 activity to bioenergetics, redox, and tumor cell motility.

    Evidence In vitro kinase assays with phospho-site mutagenesis, patient-fibroblast rescue, and tumor and trafficking readouts

    PMID:30304514 PMID:31406245

    Open questions at the time
    • How phosphorylation alters hexamer conformation not structurally resolved
    • Phosphatase reversing the modification unknown
  7. 2019 High

    Demonstrated dose-dependent cardioprotective and ETC-regulatory roles of LONP1 in ischemia/reperfusion using both gain- and loss-of-function models.

    Evidence Cardiac transgenic overexpression and haploinsufficient mice, NRVM knockdown, in vivo I/R, and respirometry

    PMID:30625302

    Open questions at the time
    • Direct substrates mediating Complex I downregulation not all identified
    • Relationship to preconditioning signaling incomplete
  8. 2020 Medium

    Linked LONP1 to differentiation and to a gain-of-function neurodegeneration mechanism, showing that imbalance toward excess proteolysis at the expense of chaperone function is itself pathogenic.

    Evidence Patient-fibroblast substrate immunoblotting and hexamer stability assays for p.R301W; C2C12 siRNA and PINK1/Parkin pathway analysis

    PMID:31923470 PMID:32936696

    Open questions at the time
    • N-terminal domain conformational control inferred indirectly
    • Single-lab functional characterization without structural confirmation
  9. 2021 High

    Resolved the structural basis of LONP1 catalysis, showing dual spiral staircases and the requirement of both ATPase-channel and protease-site substrate engagement to activate proteolysis, explaining ATPase/protease decoupling.

    Evidence Cryo-EM in multiple conformational states with bortezomib active-site trapping

    PMID:34050165

    Open questions at the time
    • Native folded-substrate engagement not captured in this study
    • How specific substrates select between states unresolved
  10. 2021 High

    Separated LONP1 chaperone function from its protease activity, showing ATPase-dependent collaboration with mtHSP70/DNAJA3 to fold matrix proteins.

    Evidence siRNA, in vitro chaperone reconstitution with OXA1L, and ATPase-dead versus protease-dead mutants

    PMID:33431889

    Open questions at the time
    • Structural basis of chaperone handoff to mtHSP70 unknown
    • Full chaperone client set undefined
  11. 2021 Medium

    Expanded the substrate landscape and partner network, defining functional overlap with ClpP (shared substrate SHMT2), the NEK5-LonP1-TFAM module, AIFM1 control of oocyte survival, and degradation of mitochondria-associated DJ-1 mutants.

    Evidence APEX proximity proteomics with dual protease depletion; Co-IP of NEK5 and AIFM1; oocyte conditional KO; siRNA pulse-chase of DJ-1 mutants

    PMID:33547867 PMID:33637676 PMID:33795807 PMID:34974310

    Open questions at the time
    • NEK5 phosphorylation of LonP1 not directly shown
    • DJ-1 mutant degradation not reconstituted in vitro
    • Several interactions rest on single Co-IP
  12. 2022 High

    Extended LONP1 beyond matrix proteolysis to ATFS-1/UPRmt-coupled mtDNA quality control, MAM tethering, and ER-stress/development signaling, and tied substrate accumulation to autophagy and muscle wasting.

    Evidence C. elegans/cybrid genetics with ChIP and heteroplasmy; muscle and cardiac conditional KO with ΔOTC substrate epistasis; embryonic cardiac KO with ATF4-Tfam-Glut1 analysis; MAM fractionation

    PMID:35165413 PMID:35173176 PMID:35237793 PMID:37333972

    Open questions at the time
    • MAM tethering mechanism is single-lab/Medium evidence
    • How matrix protease activity produces ER/MAM phenotypes mechanistically unclear
  13. 2022 Medium

    Defined selective small-molecule pharmacology of LonP1 and detected a nuclear LONP1 pool interacting with HSF1, broadening its regulatory reach.

    Evidence In vitro ATPase/protease kinetics and cryo-EM docking of CDDO derivatives with mutagenesis; subcellular fractionation and Co-IP of nuclear LONP1 with HSF1

    PMID:35151690 PMID:35760833

    Open questions at the time
    • Functional consequence of LONP1-HSF1 interaction rests on Co-IP
    • Nuclear LONP1 import/processing route unknown
  14. 2023 High

    Identified additional regulated substrates that couple LONP1 to metabolic and epigenetic programming (SDHB-succinate-histone methylation in beiging; HMGCS2 in kidney) and a deacetylation/ubiquitination switch controlling LONP1 stability.

    Evidence Adipocyte and renal conditional KO/overexpression with proteomics and metabolomics; MS site mapping of K145, Sirt3 KO, and K63-ubiquitination assays

    PMID:36629048 PMID:36739437 PMID:37217599

    Open questions at the time
    • ESCRT0-mediated LONP1 turnover route incompletely defined
    • Tissue specificity of substrate selection unexplained
  15. 2024 High

    Captured LonP1 engaging a native substrate (TFAM) to define the search-and-shred mechanism and mapped PolG engagement, linking LONP1 to mitochondrial polymerase regulation and drug-induced (artemisinin) substrate degradation.

    Evidence Cryo-EM of LonP1-TFAM, crosslinking MS and mutagenesis on PolG, in vitro proteolysis; biochemical binding and Co-IP for artemisinin-enhanced CYP11A1 degradation in PCOS models

    PMID:38870290 PMID:38932681 PMID:41900996

    Open questions at the time
    • Generalizability of N-domain vestibule selectivity to all substrates untested
    • Artemisinin binding site on LONP1 not structurally resolved
  16. 2025 High

    Resolved a fold-sensing C3-symmetric intermediate and defined import-coupled and metabolic roles, including FBXO11-driven mitochondrial import, chaperone-dependent β-cell survival, and proteolytic control of DHODH, MPC1, and ACO2 across disease contexts.

    Evidence Cryo-EM intermediate states (preprint); Co-IP and K63-ubiquitination with respirometry (FBXO11); conditional KO/overexpression with protease-dead vs chaperone mutants and human islet/MASH/PH/HCC models

    PMID:40332105 PMID:40691304 PMID:40784490 PMID:40917061 PMID:40950177 PMID:41289019

    Open questions at the time
    • Intermediate-state model is preprint and awaits peer review
    • ACO2 substrate relationship inferred without in vitro reconstitution
    • How FBXO11 K63-ubiquitination mechanistically promotes import unclear

Open questions

Synthesis pass · forward-looking unresolved questions
  • How LONP1 integrates its multiple post-translational regulatory inputs (phosphorylation, oxidation, acetylation, ubiquitination) with conformational state to select among its diverse substrates, and how nuclear/MAM pools are established and function, remains unresolved.
  • No unified model linking modification state to substrate choice
  • Nuclear LONP1 function beyond HSF1 Co-IP undefined
  • Structural basis of extramitochondrial localization unknown

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0140096 catalytic activity, acting on a protein 7 GO:0016787 hydrolase activity 4 GO:0140657 ATP-dependent activity 4 GO:0003677 DNA binding 2 GO:0044183 protein folding chaperone 2
Localization
GO:0005739 mitochondrion 4 GO:0005634 nucleus 1
Pathway
R-HSA-1430728 Metabolism 6 R-HSA-1643685 Disease 4 R-HSA-392499 Metabolism of proteins 4 R-HSA-8953897 Cellular responses to stimuli 3
Partners
AIFM1CYP11A1DNAJA3FBXO11HSF1HSPA9NEK5POLG

Evidence

Reading pass · 34 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
2021 LONP1 cryo-EM structures reveal that human LONP1 adopts both open and closed spiral staircase orientations dictated by substrate and nucleotide; unlike bacterial Lon, it contains a second spiral staircase within the ATPase domain that engages substrate during translocation. Critically, substrate binding within the central ATPase channel alone is insufficient to induce the activated protease conformation—substrate binding within the protease active site is also required, demonstrating that LONP1 can decouple ATPase and protease activities. Cryo-electron microscopy structural determination; peptidomimetic active-site inhibitor bortezomib used to trap active conformation Nature communications High 34050165
2021 LONP1 works with the mtHSP70 chaperone system (specifically co-chaperone DNAJA3) to promote mitochondrial protein folding. LONP1 is required for DNAJA3 and mtHSP70 solubility; its ATPase activity (but not protease activity) is required for this chaperone function. In vitro, LONP1 shows intrinsic chaperone-like activity and collaborates with mtHSP70 to stabilize a folding intermediate of OXA1L. siRNA knockdown, in vitro chaperone reconstitution assay, ATPase-dead and protease-dead mutants, mass spectrometry of aggregated proteins Nature communications High 33431889
2015 LONP1 pathogenic mutations causing CODAS syndrome cluster within the AAA+ domain near the ATP-binding pocket; biochemical assays show substrate-specific defects in ATP-dependent proteolysis, and one Amish variant (p.Arg721Gly) homo-oligomerizes poorly in vitro. Patient lymphoblastoid cells show swollen mitochondria with electron-dense inclusions, aggregated MT-CO2 (mtDNA-encoded COX subunit II), and reduced spare respiratory capacity. Biochemical proteolysis assays with recombinant mutant proteins; native gel oligomerization assay; electron microscopy of patient lymphoblastoid cell mitochondria; immunoblotting American journal of human genetics High 25574826
2011 LONP1 mediates heme-induced proteolysis of mature ALAS-1 (5-aminolevulinic acid synthase) in the mitochondrial matrix. Inhibition of LONP1 with inhibitors or siRNA knockdown blocks heme- and metalloporphyrin-induced degradation of ALAS-1 protein, establishing LONP1 as the protease responsible for this feedback regulatory step in heme biosynthesis. siRNA knockdown of LONP1; pharmacological inhibition; pulse-chase protein turnover assays in human liver cells The Journal of biological chemistry High 21659532
2024 Artemisinins directly target LONP1, enhance the LONP1–CYP11A1 protein interaction, and facilitate LONP1-catalyzed degradation of CYP11A1, thereby suppressing ovarian androgen synthesis. Overexpression of LONP1 alone replicates the androgen-lowering effect. Biochemical binding assays, Co-IP, LONP1 overexpression in rodent PCOS models and human patients, mass spectrometry Science (New York, N.Y.) High 38870290
2018 LONP1 and the mitochondrial processing peptidase MPPαβ are together required for maturation of a subset of mitochondrial matrix proteins (SSBP1, MTERFD3, FASTKD2, CLPX); these proteins accumulate in unprocessed, insoluble form when LONP1 is absent. Prolonged LONP1 depletion also causes loss of mtDNA and suppression of mitochondrial translation via impaired ribosome biogenesis, activates the integrated stress response (ISR), and stabilizes PINK1. Catalytically dead LONP1 mutant expression; MPP depletion; quantitative proteomics; siRNA knockdown; immunoblotting Molecular and cellular biology High 30061372
2019 Mitochondrial LonP1 is phosphorylated by Akt at Ser173 and Ser181, which enhances its protease activity. Interference with this Akt–LonP1 pathway causes accumulation of misfolded subunits of ETC complex II and complex V, impairs oxidative bioenergetics, increases ROS, suppresses mitochondrial trafficking to the cortical cytoskeleton, and inhibits tumor cell migration and invasion. In vitro kinase assay, site-directed mutagenesis of phosphorylation sites, co-immunoprecipitation, mitochondrial trafficking imaging, tumor growth assays in vivo Oncogene High 31406245
2019 Purified LonP1-P761L (LONP1 pathogenic variant causing neurodegeneration) fails to degrade phosphorylated PDH E1α subunit in vitro, while wild-type LonP1 degrades it. Overexpressing wild-type LonP1 in patient fibroblasts down-regulates phospho-E1α and rescues PDH activity, demonstrating that LONP1 degrades phospho-E1α to maintain pyruvate dehydrogenase function. In vitro proteolysis assay with purified recombinant proteins; LonP1 overexpression in patient fibroblasts; PDH activity and lactate/pyruvate ratio measurements Human molecular genetics High 30304514
2018 LonP1 mutant p.Tyr565His cannot bind or degrade a substrate in vitro, consistent with the predicted role of this residue. Mixing p.Tyr565His with wild-type LonP1 retains partial protease activity, but mixing p.Tyr565His with p.Glu733Lys severely depletes activity, consistent with compound heterozygosity causing classical mitochondrial disease. In vitro proteolysis assay with purified recombinant single and mixed mutant LonP1 proteins Human molecular genetics High 29518248
2014 Oxidative post-translational modifications (decreased reduced cysteine, increased tyrosine nitration and protein carbonylation) of LONP1 attenuate its ATP-dependent proteolytic activity in failing mouse hearts. Mitochondria-targeted antioxidants (mitoTEMPO, mitochondria-targeted human catalase) reversed these oxidative modifications and restored LONP1 proteolytic activity; siRNA repression of LONP1 partially canceled the protective antioxidant effects, confirming LONP1 mediates redox-dependent ETC protein turnover. FITC-casein proteolytic activity assay; immunoblotting for oxidative modifications; in vivo siRNA knockdown; mitochondria-targeted antioxidant treatment; cardiac function measurement Circulation. Heart failure High 24740269
2022 In C. elegans, LONP-1 (the LONP1 ortholog) degrades ATFS-1 in healthy mitochondria, preventing its accumulation. In dysfunctional mitochondria, LONP-1-mediated ATFS-1 degradation is impaired, allowing ATFS-1 to accumulate and bind the replicative polymerase POLG to ∆mtDNA. LONP-1 inhibition in C. elegans and human cybrid cells increased ATFS-1/POLG binding to wild-type mtDNAs, improving heteroplasmy ratios and restoring oxidative phosphorylation. Genetic loss-of-function (lonp-1 deletion), ChIP for POLG-mtDNA binding, heteroplasmy quantification, LONP-1 inhibitor treatment in C. elegans and human cybrids, respirometry Nature cell biology High 35165413
2022 Skeletal muscle-specific ablation of LONP1 in mice causes impaired mitochondrial protein turnover leading to mitochondrial dysfunction, reduced muscle fiber size and strength. Mechanistically, accumulation of mitochondrial-retained protein activates the autophagy-lysosome degradation program. Overexpression of ΔOTC (a known LONP1 substrate) in skeletal muscle recapitulates mitochondrial dysfunction, autophagy activation, muscle loss, and weakness. Conditional muscle-specific LONP1 knockout mice; ΔOTC overexpression; respirometry; autophagy markers; muscle fiber histology and strength testing Nature communications High 35173176
2023 LONP1 selectively degrades succinate dehydrogenase complex iron sulfur subunit B (SDHB) in mature white adipocytes during thermogenic stimulation. LONP1-mediated SDHB degradation raises intracellular succinate levels, altering histone methylation status on thermogenic genes and enabling white-to-beige adipocyte cell fate programming. Disruption of LONP1-dependent proteolysis impairs cold- or β3 adrenergic agonist-induced beige conversion. Conditional LONP1 disruption in mature adipocytes; mass spectrometry substrate identification; succinate metabolomics; histone methylation ChIP; cold/β3-agonist treatment in vivo and in vitro Nature cell biology High 37217599
2023 LONP1 degrades HMGCS2 (3-hydroxy-3-methylglutaryl-CoA synthase 2) in the mitochondrial matrix; LONP1 downregulation causes aberrant HMGCS2 accumulation that disrupts mitochondrial function and accelerates chronic kidney disease progression. Tubular-specific Lonp1 overexpression and deletion in CKD mouse models; in vitro proteolysis validation; mass spectrometry substrate identification; renal function and mitochondrial function assays EMBO molecular medicine High 36629048
2019 LonP1 overexpression in mouse hearts protects against ischemia/reperfusion injury by reducing oxidative protein damage, maintaining mitochondrial redox homeostasis, and downregulating Complex I protein levels and activity. LonP1 haploinsufficiency abrogates ischemic preconditioning-mediated cardioprotection and increases infarct size. In NRVMs, LonP1 knockdown upregulates Complex I subunits and ETC activities, increases superoxide production, and causes mitochondrial inner membrane dysmorphology and hyperpolarization. Cardiac-specific LonP1 transgenic overexpression mice; LonP1+/− haploinsufficient mice; siRNA knockdown in NRVMs; in vivo I/R injury model; respirometry; ETC activity assays Journal of molecular and cellular cardiology High 30625302
2021 LONP1 directly interacts with AIFM1 (apoptosis-inducing factor, mitochondria-associated 1); LONP1 ablation in mouse oocytes causes translocation of AIFM1 from cytoplasm to nucleus, triggering apoptosis and progressive oocyte death. Conditional knockout of Lonp1 in oocytes impairs follicular development and causes infertility. Conditional oocyte-specific Lonp1 knockout mice; Co-IP/LC-MS; immunofluorescence for AIFM1 localization; follicle counting; fertility assays EBioMedicine High 34974310
2022 LONP1 is identified as a novel mitochondria-associated membrane (MAM) tethering protein; removal of LonP1 substantially reduces MAM formation and causes mitochondrial fragmentation. Cardiac-specific LonP1 deletion impairs MAM integrity, mitochondrial fusion, and activates ER unfolded protein response (UPRER), causing metabolic reprogramming and pathological heart remodeling. Conditional cardiac-specific LonP1 knockout mice; MAM fractionation; mitochondrial morphology imaging; ER stress markers; cardiac phenotyping Research (Washington, D.C.) Medium 37333972
2022 CDDO and its methyl and imidazole derivatives reversibly inhibit LonP1 by a noncompetitive mechanism, blocking ATP hydrolysis and thus proteolysis (allosteric inhibition). CDDO-anhydride inhibits LonP1 ATPase competitively. Docking of CDDO derivatives in the cryo-EM structure identifies a hydrophobic pocket adjacent to the ATP-binding site. A CODAS-causing pathogenic mutation ablates CDDO inhibition. CDDO fails to inhibit the 26S proteasome ATPase, showing selectivity within the AAA+ family. In vitro ATPase and protease activity assays; competitive vs. noncompetitive kinetic analysis; cryo-EM structure-based docking; site-directed mutagenesis validation; cell-based inhibition assays The Journal of biological chemistry High 35151690
2023 Sirt3 deacetylates LONP1 at lysine 145 (K145) in the N-terminal domain; deacetylation subsequently facilitates ESCRT0 complex sorting and K63-linked ubiquitination of LONP1, resulting in its degradation. Hyperacetylation-mimetic LONP1-K145Q enhances oxidative phosphorylation and accelerates tumor growth, while deacetylation-mimetic K145R produces a calorie-restriction-like phenotype restraining tumorigenesis. Mass spectrometry identification of deacetylation site; K145Q/K145R mutagenesis; Sirt3 conditional intestinal knockout mice; Seahorse metabolic analysis; ubiquitination assays Journal of translational medicine High 36739437
2020 De novo LONP1 mutation p.R301W (located in the N-terminal domain) causes a mitochondrial encephalopathy phenotype characterized by excessive LONP1 proteolytic activity (degrading known substrates TFAM, PINK1, phospho-PDH E1α, mitoribosome subunits MRPL44/MRPL11) combined with loss of LONP1 chaperone activity. The N-terminal domain is required for hexamer stability and for conformational changes necessary to balance proteolytic and chaperone activities. Functional studies in patient fibroblasts; immunoblotting for proteolytic substrates; respiratory chain activity assays; hexamer stability assays with N-terminal domain mutations Mitochondrion Medium 31923470
2021 NEK5 kinase interacts with LonP1 within mitochondria and may be involved in the LonP1-TFAM signaling module. NEK5 kinase activity is required for maintaining mitochondrial mass, functionality, and mtDNA integrity after oxidative damage. Co-immunoprecipitation of NEK5 and LonP1; NEK5 kinase-dead mutants; mitochondrial mass and mtDNA integrity assays after oxidative damage FEBS open bio Medium 33547867
2021 LONP1 and ClpP share substrates including SHMT2; depletion of both proteases synergistically increases accumulation of unfolded SHMT2, confirming functional substrate overlap. APEX-mediated proximity proteomics identified the specific substrate sets of LONP1 and ClpP within the mitochondrial matrix, including components of OXPHOS, TCA cycle, amino acid and lipid metabolism. APEX-mediated proximity biotinylation/proteomics; siRNA depletion of LONP1 and ClpP individually and combined; immunoblotting for SHMT2 folding status; cell growth and death assays Oncogenesis Medium 33637676
2021 Mitochondrial LonP1 protease is implicated in the degradation of unstable Parkinson's disease-associated DJ-1/PARK7 missense mutants (A107P, P158Δ, L166P, E163K, L172Q) that associate with mitochondria. siRNA silencing of LONP1 strongly reduces degradation of these mitochondria-associated DJ-1 mutants. siRNA knockdown of LONP1; immunofluorescence and biochemical fractionation to assess DJ-1 mutant localization; pulse-chase degradation assays in DJ-1-null MEFs Scientific reports Medium 33795807
2020 LONP1 depletion in mouse oocytes during myoblast differentiation causes mitochondrial depolarization, suppresses the PINK1/Parkin pathway, and reduces Mfn2 and Drp1 levels, blocking mitochondrial remodeling and myoblast differentiation into myotubes. LonP1 promotes myoblast differentiation by regulating PINK1/Parkin-mediated mitochondrial network remodeling. siRNA knockdown of LonP1 in C2C12 myoblasts; PINK1/Parkin pathway immunoblotting; mitochondrial membrane potential assay; fusion/fission protein levels; differentiation assays American journal of physiology. Cell physiology Medium 32936696
2022 LONP1 is detected in the nucleus (up to 22% of total cellular LONP1). Nuclear LONP1 interacts with heat shock factor 1 (HSF1) and modulates the heat shock response. Nuclear localization is detectable under all conditions but increases in response to heat shock. Subcellular fractionation; immunofluorescence; Co-immunoprecipitation of LONP1 with HSF1; quantification of nuclear fraction under heat shock Scientific reports Medium 35760833
2024 Cryo-EM structure of human LonP1 engaging its native substrate TFAM reveals a bipartite search-and-shred mechanism: the N-terminal domain acts as a selective vestibule to recruit and initially unfold substrate tertiary structure, then polypeptide is threaded through the central channel via a hand-over-hand mechanism driven by aromatic pore-loops. Chemical crosslinking mass spectrometry and site-directed mutagenesis uncovered the region of LonP1 engagement of PolG (the catalytic subunit of the mitochondrial replicative polymerase), which promotes proteolysis and regulation of PolG protein levels; PolG2 clinical variants that disrupt a stable Polγ complex lead to enhanced LonP1-mediated PolG degradation. Single-particle cryo-EM structure of LonP1–TFAM complex; chemical crosslinking mass spectrometry; site-directed mutagenesis; in vitro proteolysis assay of PolG Nucleic acids research / Life (Basel) High 38932681 41900996
2025 A novel ADP-bound, C3-symmetric intermediate state of LONP1 (LONP1C3) is resolved by cryo-EM. This intermediate is stabilized by interaction with folded substrates and is proposed to have 'fold-sensing' capabilities. Additional symmetric and asymmetric states, including a two-fold symmetric split-hexamer conformation, are associated with the transition from LONP1C3 to the active enzyme (LONP1ENZ), providing mechanistic insight into how substrate selection and engagement regulate unfoldase activation. Cryo-electron microscopy; biochemical ATPase and protease assays bioRxivpreprint Medium 40950177
2025 FBXO11 interacts with LONP1 in the cytosol and catalyzes K63-linked ubiquitination of LONP1 to promote its entry into mitochondria. FBXO11 or LONP1 depletion reduces mitochondrial respiration through impaired LONP1 chaperone activity for assembling ETC Complex IV. This reduced mitochondrial respiration imparts myeloid-biased stem cell properties in CD34+ HSPCs. Co-IP of FBXO11 and LONP1; ubiquitination assay specifying K63-linkage; shRNA depletion; respirometry; Complex IV assembly assay; human xenograft model The Journal of clinical investigation High 41289019
2025 LONP1 degrades DHODH (dihydroorotate dehydrogenase) in hepatocytes in an ATP-dependent manner, thereby lowering orotic acid levels and suppressing ATF3-mediated activation of hepatic stellate cells. Hepatocyte-specific LONP1 deficiency causes DHODH accumulation, elevated orotic acid, and aggravated MASH-induced liver fibrosis; LONP1 overexpression or DHODH inhibition reverses this. Hepatocyte-specific Lonp1 knockout mice; LONP1 overexpression; transcriptomics/proteomics/metabolomics; in vitro ATP-dependent proteolysis of DHODH; liver biopsy correlation in human MASH patients Journal of hepatology High 40784490
2025 LONP1 degrades MPC1 (mitochondrial pyruvate carrier 1) directly; LONP1-mediated MPC1 degradation promotes glycolytic reprogramming and proliferation/migration of pulmonary artery smooth muscle cells in pulmonary hypertension. Rescue experiments confirm MPC1 knockdown abrogates suppressive effects of LONP1 knockdown on glycolysis and proliferation. LONP1 knockdown and overexpression in PASMCs; MPC1 knockdown and overexpression rescue experiments; in vivo LONP1 knockdown in rat PH models; glycolysis and proliferation assays Clinical science (London, England) Medium 40332105
2022 Cardiac-specific deletion of Lonp1 in mouse embryos causes severely impaired heart development and embryonic lethality, with mitochondrial swelling, cristae loss, and abnormal protein aggregates. Mechanistically, loss of LONP1 triggers the p-eIF2α–ATF4 pathway; nuclear ATF4 negatively regulates Tfam and promotes Glut1, disrupting the metabolic shift from glycolysis to oxidative phosphorylation required for heart development. Cardiac-specific conditional Lonp1 knockout mice (embryonic); electron microscopy; ATF4 nuclear translocation assay; Tfam and Glut1 gene expression analysis; metabolic function assays Development (Cambridge, England) High 35237793
2025 LONP1 promotes β-cell survival and prevents hyperglycemia by facilitating mitochondrial protein folding via its chaperone activity in cooperation with mtHSP70. LONP1 deletion causes mitochondrial protein misfolding, reduced respiratory function, β-cell apoptosis, and hyperglycemia. LONP1 gain-of-function restores human β-cell survival after glucolipotoxicity via a protease-independent mechanism requiring LONP1–mtHSP70 chaperone activity. Conditional LONP1 knockout mice; LONP1 overexpression in human islets; quantitative proteomics of protein aggregates; respirometry; β-cell apoptosis assays; protease-dead vs. chaperone mutants Nature metabolism High 40691304
2024 LONP1 degrades ACO2 (aconitase 2) to promote hepatocellular carcinoma cell proliferation, migration, and reduced susceptibility to ferroptosis and cuproptosis. LONP1 overexpression leads to decreased mitochondrial membrane potential (mitochondrial dysfunction) and reduced ACO2 protein, inhibiting ferroptosis. LONP1 overexpression and knockdown in HCC cell lines; subcutaneous tumor xenografts; histological co-staining of LONP1 and ACO2; mitochondrial membrane potential assay; ferroptosis/cuproptosis assays Frontiers in bioscience (Landmark edition) Low 40917061
2026 LONP-1 (C. elegans LONP1 ortholog) deficiency causes dysregulation of mitochondrial DNA replication, mitochondrial RNA transcription, and protein synthesis. Suppressor mutations in genes encoding mitochondrial ribosome proteins (MRPS-38, MRPS-15) restore mitochondrial function and oxidative phosphorylation in lonp-1 mutants, suggesting LONP-1 regulates mitochondrial protein synthesis and that slowing translation corrects OXPHOS complex assembly defects. Forward genetic suppressor screen in C. elegans; lonp-1 deletion mutants; mitoribosome gene mutagenesis; respirometry; mtDNA replication and transcription assays bioRxivpreprint Medium 41648325

Source papers

Stage 0 corpus · 100 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
2015 CODAS syndrome is associated with mutations of LONP1, encoding mitochondrial AAA+ Lon protease. American journal of human genetics 130 25574826
2021 LONP1 and mtHSP70 cooperate to promote mitochondrial protein folding. Nature communications 110 33431889
2011 Lon peptidase 1 (LONP1)-dependent breakdown of mitochondrial 5-aminolevulinic acid synthase protein by heme in human liver cells. The Journal of biological chemistry 106 21659532
2024 Artemisinins ameliorate polycystic ovarian syndrome by mediating LONP1-CYP11A1 interaction. Science (New York, N.Y.) 101 38870290
2018 LONP1 Is Required for Maturation of a Subset of Mitochondrial Proteins, and Its Loss Elicits an Integrated Stress Response. Molecular and cellular biology 89 30061372
2022 LONP-1 and ATFS-1 sustain deleterious heteroplasmy by promoting mtDNA replication in dysfunctional mitochondria. Nature cell biology 80 35165413
2019 Mitochondrial LonP1 protects cardiomyocytes from ischemia/reperfusion injury in vivo. Journal of molecular and cellular cardiology 78 30625302
2022 Disuse-associated loss of the protease LONP1 in muscle impairs mitochondrial function and causes reduced skeletal muscle mass and strength. Nature communications 72 35173176
2015 Mutations in LONP1, a mitochondrial matrix protease, cause CODAS syndrome. American journal of medical genetics. Part A 68 25808063
2020 The biology of Lonp1: More than a mitochondrial protease. International review of cell and molecular biology 63 32475470
2021 Structures of the human LONP1 protease reveal regulatory steps involved in protease activation. Nature communications 62 34050165
2014 Oxidative post-translational modifications develop LONP1 dysfunction in pressure overload heart failure. Circulation. Heart failure 61 24740269
2023 LONP1 targets HMGCS2 to protect mitochondrial function and attenuate chronic kidney disease. EMBO molecular medicine 59 36629048
2018 Defective mitochondrial protease LonP1 can cause classical mitochondrial disease. Human molecular genetics 57 29518248
2021 LONP1 and ClpP cooperatively regulate mitochondrial proteostasis for cancer cell survival. Oncogenesis 56 33637676
2018 LonP1 Differently Modulates Mitochondrial Function and Bioenergetics of Primary Versus Metastatic Colon Cancer Cells. Frontiers in oncology 51 30038898
2019 Akt phosphorylation of mitochondrial Lonp1 protease enables oxidative metabolism and advanced tumor traits. Oncogene 50 31406245
2021 The mitochondrial protease LONP1 maintains oocyte development and survival by suppressing nuclear translocation of AIFM1 in mammals. EBioMedicine 48 34974310
2021 Rare and de novo variants in 827 congenital diaphragmatic hernia probands implicate LONP1 as candidate risk gene. American journal of human genetics 44 34547244
2016 The peroxisomal Lon protease LonP2 in aging and disease: functions and comparisons with mitochondrial Lon protease LonP1. Biological reviews of the Cambridge Philosophical Society 39 26852705
2023 The Role of Lonp1 on Mitochondrial Functions during Cardiovascular and Muscular Diseases. Antioxidants (Basel, Switzerland) 38 36978846
2023 LonP1 Links Mitochondria-ER Interaction to Regulate Heart Function. Research (Washington, D.C.) 37 37333972
2019 Inhibition of LONP1 protects against erastin-induced ferroptosis in Pancreatic ductal adenocarcinoma PANC1 cells. Biochemical and biophysical research communications 36 31822343
2019 Bi-allelic mutations of LONP1 encoding the mitochondrial LonP1 protease cause pyruvate dehydrogenase deficiency and profound neurodegeneration with progressive cerebellar atrophy. Human molecular genetics 33 30304514
2022 LONP1 downregulation with ageing contributes to osteoarthritis via mitochondrial dysfunction. Free radical biology & medicine 31 36064070
2022 LONP1-mediated mitochondrial quality control safeguards metabolic shifts in heart development. Development (Cambridge, England) 30 35237793
2020 Chronic hyperglycemia impairs mitochondrial unfolded protein response and precipitates proteotoxicity in experimental diabetic neuropathy: focus on LonP1 mediated mitochondrial regulation. Pharmacological reports : PR 30 32720218
2023 Proteolytic rewiring of mitochondria by LONP1 directs cell identity switching of adipocytes. Nature cell biology 29 37217599
2019 Ameliorative effect of ursolic acid on ochratoxin A-induced renal cytotoxicity mediated by Lonp1/Aco2/Hsp75. Toxicon : official journal of the International Society on Toxinology 27 31356822
2019 CDDO-Me Selectively Attenuates CA1 Neuronal Death Induced by Status Epilepticus via Facilitating Mitochondrial Fission Independent of LONP1. Cells 25 31387295
2023 Sirt3 restricts tumor initiation via promoting LONP1 deacetylation and K63 ubiquitination. Journal of translational medicine 24 36739437
2021 The Mitochondrial Protease LonP1 Promotes Proteasome Inhibitor Resistance in Multiple Myeloma. Cancers 24 33671345
2020 Impaired Mitochondrial Morphology and Functionality in Lonp1wt/- Mice. Journal of clinical medicine 24 32521756
2022 Lonp1 and Sig-1R contribute to the counteraction of ursolic acid against ochratoxin A-induced mitochondrial apoptosis. Food and chemical toxicology : an international journal published for the British Industrial Biological Research Association 23 36587836
2019 LONP1 induction by SRT1720 attenuates mitochondrial dysfunction against high glucose induced neurotoxicity in PC12 cells. Toxicology in vitro : an international journal published in association with BIBRA 23 31639451
2022 Inhibition of mitochondrial LonP1 protease by allosteric blockade of ATP binding and hydrolysis via CDDO and its derivatives. The Journal of biological chemistry 22 35151690
2021 Proteomic analysis demonstrates the role of the quality control protease LONP1 in mitochondrial protein aggregation. The Journal of biological chemistry 22 34461102
2022 Transcranial Direct-Current Stimulation Regulates MCT1-PPA-PTEN-LONP1 Signaling to Confer Neuroprotection After Rat Cerebral Ischemia-Reperfusion Injury. Molecular neurobiology 21 36190692
2020 LONP1 de novo dominant mutation causes mitochondrial encephalopathy with loss of LONP1 chaperone activity and excessive LONP1 proteolytic activity. Mitochondrion 21 31923470
2022 Organismal and Cellular Stress Responses upon Disruption of Mitochondrial Lonp1 Protease. Cells 20 35456042
2021 LONP1 Regulates Mitochondrial Accumulations of HMGB1 and Caspase-3 in CA1 and PV Neurons Following Status Epilepticus. International journal of molecular sciences 20 33668863
2017 A novel mutation in the proteolytic domain of LONP1 causes atypical CODAS syndrome. Journal of human genetics 20 28148925
2021 NEK5 interacts with LonP1 and its kinase activity is essential for the regulation of mitochondrial functions and mtDNA maintenance. FEBS open bio 19 33547867
2023 Powering down the mitochondrial LonP1 protease: a novel strategy for anticancer therapeutics. Expert opinion on therapeutic targets 18 38156441
2021 Structure-Based Design of Selective LONP1 Inhibitors for Probing In Vitro Biology. Journal of medicinal chemistry 18 33821636
2019 Acute increases in O-GlcNAc indirectly impair mitochondrial bioenergetics through dysregulation of LonP1-mediated mitochondrial protein complex turnover. American journal of physiology. Cell physiology 18 30865517
2020 Loss of mitochondrial ClpP, Lonp1, and Tfam triggers transcriptional induction of Rnf213, a susceptibility factor for moyamoya disease. Neurogenetics 17 32342250
2019 Cell stress management by the mitochondrial LonP1 protease - Insights into mitigating developmental, oncogenic and cardiac stress. Mitochondrion 16 31756517
2023 Intraneuronal β-amyloid impaired mitochondrial proteostasis through the impact on LONP1. Proceedings of the National Academy of Sciences of the United States of America 15 38091289
2022 Evidence for mitochondrial Lonp1 expression in the nucleus. Scientific reports 15 35760833
2019 Inhibition of LONP1 Suppresses Pancreatic Cancer Progression Via c-Jun N-Terminal Kinase Pathway-Meditated Epithelial-Mesenchymal Transition. Pancreas 15 31091208
2019 Global Proteome of LonP1+/- Mouse Embryonal Fibroblasts Reveals Impact on Respiratory Chain, but No Interdependence between Eral1 and Mitoribosomes. International journal of molecular sciences 15 31547314
2025 LONP1 regulation of mitochondrial protein folding provides insight into beta cell failure in type 2 diabetes. Nature metabolism 14 40691304
2021 Mitochondrial LonP1 protease is implicated in the degradation of unstable Parkinson's disease-associated DJ-1/PARK 7 missense mutants. Scientific reports 13 33795807
2019 Expanding the Clinical Spectrum of LONP1-Related Mitochondrial Cytopathy. Frontiers in neurology 13 31636596
2024 m6A-methylated Lonp1 drives mitochondrial proteostasis stress to induce testicular pyroptosis upon environmental cadmium exposure. The Science of the total environment 11 38703850
2021 Expression of LONP1 Is High in Visceral Adipose Tissue in Obesity, and Is Associated with Glucose and Lipid Metabolism. Endocrinology and metabolism (Seoul, Korea) 11 34154043
2020 LonP1 regulates mitochondrial network remodeling through the PINK1/Parkin pathway during myoblast differentiation. American journal of physiology. Cell physiology 11 32936696
2024 Context-dependent roles of mitochondrial LONP1 in orchestrating the balance between airway progenitor versus progeny cells. Cell stem cell 10 39181129
2024 LONP1 alleviates ageing-related renal fibrosis by maintaining mitochondrial homeostasis. Journal of cellular and molecular medicine 10 39261902
2023 Mitochondrial oxidative stress regulates LonP1-TDP-43 pathway and rises mitochondrial damage in carbon tetrachloride-induced liver fibrosis. Ecotoxicology and environmental safety 10 37647804
2018 Clinical features of LONP1-related infantile cataract. Journal of AAPOS : the official publication of the American Association for Pediatric Ophthalmology and Strabismus 10 29408517
2025 LONP1 facilitates pulmonary artery smooth muscle cell glycolytic reprogramming by degrading MPC1 in pulmonary hypertension. Clinical science (London, England : 1979) 8 40332105
2022 Inhibition of Lonp1 induces mitochondrial remodeling and autophagy suppression in cervical cancer cells. Acta histochemica 8 36508908
2025 The mitochondrial LONP1 protease: molecular targets and role in pathophysiology. Molecular biology reports 7 40249453
2022 Differential Expression of Lonp1 Isoforms in Cancer Cells. Cells 7 36497197
2024 LONP1 ameliorates liver injury and improves gluconeogenesis dysfunction in acute-on-chronic liver failure. Chinese medical journal 6 38184784
2023 The first case report of CODAS syndrome in Chinese population caused by two LONP1 pathogenic mutations. Frontiers in genetics 6 36685982
2025 Targeting mitochondrial proteases CLPP and LONP1 via disruption of mitochondrial redox homeostasis induces proteotoxic stress and suppresses tumor progression. Cell communication and signaling : CCS 5 40903791
2024 Coordinated DNA polymerization by Polγ and the region of LonP1 regulated proteolysis. Nucleic acids research 5 38932681
2024 Activation of LONP1 by 84-B10 alleviates aristolochic acid nephropathy via re-establishing mitochondrial and peroxisomal homeostasis. Chinese journal of natural medicines 5 39326975
2024 Readdressing the Localization of Apolipoprotein E (APOE) in Mitochondria-Associated Endoplasmic Reticulum (ER) Membranes (MAMs): An Investigation of the Hepatic Protein-Protein Interactions of APOE with the Mitochondrial Proteins Lon Protease (LONP1), Mitochondrial Import Receptor Subunit TOM40 (TOMM40) and Voltage-Dependent Anion-Selective Channel 1 (VDAC1). International journal of molecular sciences 5 39408926
2024 Aging of alveolar type 2 cells induced by Lonp1 deficiency exacerbates pulmonary fibrosis. Biomolecules & biomedicine 4 38625722
2023 Mitochondrial p38 Mitogen-Activated Protein Kinase: Insights into Its Regulation of and Role in LONP1-Deficient Nematodes. International journal of molecular sciences 4 38139038
2022 Roles of LonP1 in Oral-Maxillofacial Developmental Defects and Tumors: A Novel Insight. International journal of molecular sciences 4 36362158
2025 Modulation of Lonp1 Activity by Small Compounds. Biomolecules 3 40305312
2023 Decreased LONP1 expression contributes to DNA damage and meiotic defects in oocytes. Molecular reproduction and development 3 37392095
2025 Dual regulation of Atf3 and Lonp1 as therapeutic targets in cerebral ischaemia-reperfusion injury. Stroke and vascular neurology 2 40044488
2025 LONP1 loss causes mitochondrial mayhem in β-cells. Nature metabolism 2 40691303
2025 Wen-Shen-Tong-Luo-Zhi-Tong Decoction alleviates bone loss in aged mice by suppressing LONP1-mediated macrophage senescence. Pharmaceutical biology 2 40719285
2025 Decreased LONP1 expression exacerbates MASH-induced liver fibrosis via elevated orotic acid levels. Journal of hepatology 2 40784490
2025 LONP1 Variants Are Associated With Clinically Diverse Phenotypes. Clinical genetics 2 40931319
2025 Single-cell transcriptomics uncovering a critical AKT-LONP1-STAR axis in ovarian hyperandrogenism of PCOS. Journal of ovarian research 2 41257877
2024 Association of LONP1 gene with epilepsy and the sub-regional effect. Scientific reports 2 39462050
2025 Peste des petits ruminants virus (PPRV) induces ferroptosis via LONP1-mediated mitochondrial GPX4 degradation in cell culture. Journal of virology 1 40197059
2025 Systemic Lonp1 Haploinsufficiency Mitigates Cardiac Mitochondrial Dysfunction Induced by Cardiomyocyte-Specific Lonp1 Haploinsufficiency via Potential Inter-Organ Crosstalk. Biomolecules 1 40867604
2025 Identifying a novel Mecp2-mediated epigenetic mechanism controlling Lonp1 in the hippocampus and its disruption by aging. Scientific reports 1 41258406
2024 LONP1 regulation of mitochondrial protein folding provides insight into beta cell failure in type 2 diabetes. bioRxiv : the preprint server for biology 1 38895283
2026 Role of LONP1 in human diseases: molecular mechanisms and therapeutic potential. Cellular & molecular biology letters 0 41620670
2026 LONP-1 deficiency causes dysregulated protein synthesis within mitochondria that is restored by mitoribosomal mutations. bioRxiv : the preprint server for biology 0 41648325
2026 Structural Analysis of Human LonP1 Protease Bound with the Native Substrate. Life (Basel, Switzerland) 0 41900996
2026 Lonp1 inhibition disrupts mitochondrial function in the hippocampus and drives an aging-like synaptic and cognitive phenotype in adult SAMP8 mice. Free radical biology & medicine 0 41903616
2026 Rare ginsenoside Rk1 protects against cisplatin-induced auditory damage by regulating the MST1/LONP1 pathway. Phytomedicine : international journal of phytotherapy and phytopharmacology 0 42097003
2026 The Multiple Roles and Targeting Strategies of LonP1 in the Occurrence and Development of Cancer. BioFactors (Oxford, England) 0 42219609
2025 Sulforaphane downregulates mitochondrial TIGAR via inhibiting mitochondrial transmembrane assembly and LONP1/CASP3 axis causing apoptosis. Biochemical and biophysical research communications 0 40157290
2025 LONP1 Promotes Hepatocarcinogenesis by Degrading ACO2 to Alleviate Ferroptosis. Frontiers in bioscience (Landmark edition) 0 40917061
2025 Cryo-EM Reveals Regulatory Mechanisms Governing Substrate Selection and Activation of Human LONP1. bioRxiv : the preprint server for biology 0 40950177
2025 Loss of FBXO11 establishes a stem cell program in acute myeloid leukemia by dysregulating LONP1. The Journal of clinical investigation 0 41289019
2025 N6-methyladenosine (m6A) modification regulates LONP1 to inhibit coxsackievirus B3 replication by suppressing virus-induced ferroptosis. International journal of biological macromolecules 0 41317762
2025 Hypoxia-induced LONP1 overexpression and mtDNA damage may serve as biomarkers for death from mechanical asphyxia. Legal medicine (Tokyo, Japan) 0 41455337

Missed literature

Know a paper Affinage missed for LONP1? Flag it for the maintainers and the community.

No submissions yet.