Literature for M100.001

Summary Alignment Tree Sequences Sequence features Distribution Structure Literature Substrates

(Topics flags: S Structure, M Mutation, V Review. To select only the references relevant to a single topic, click the link above. See explanation.)

    2026
  1. Tomaskovic,I., Prieto-Garcia,C., Boskovic,M., Glumac,M., Tsai,T.L., Mosler,T., Kazi,R., Rathore,R., Andrade,J., Hoffmann,M., Giuliani,G., Jacomin,A.C., Pereira,R.S., Knop,E., Wachsmuth,L., Beli,P., Husnjak,K., Pasparakis,M., Ablasser,A., Krause,D.S., Potente,M., Papathanasiou,S., Terzic,J. and Dikic,I.
    DNA-protein cross-links promote cGAS-STING-driven premature aging and embryonic lethality
    Science (2026) 391, eadx9445-eadx9445. PubMed  Europe PubMed DOI
  2. 2025
  3. Durauer,S., Kang,H.S., Wiebeler,C., Machida,Y., Schnapka,D.S., Yaneva,D., Renz,C., Gotz,M.J., Weickert,P., Major,A.C., Rahmanto,A.S., Gutenthaler-Tietze,S.M., Daumann,L.J., Beli,P., Ulrich,H.D., Sattler,M., Machida,Y.J., Schwierz,N. and Stingele,J.
    Allosteric activation of the SPRTN protease by ubiquitin maintains genome stability
    Nat Commun (2025) 16, 5422-5422. PubMed  Europe PubMed DOI  PMC  EPMC
  4. 2024
  5. Batel,A., Polovic,M., Glumac,M., Suman,O., Jadrijevic,S., Lozic,B., Petrovic,M., Samardzija,B., Bradshaw,N.J., Skube,K., Palada,V., Acman,M. and Marinovic Terzic,I.
    SPRTN is involved in hepatocellular carcinoma development through the ER stress response
    Cancer Gene Ther (2024) 31, 376-386. PubMed  Europe PubMed DOI
  6. Brand,C.L., Oliver,G.T., Farkas,I.Z., Buszczak,M. and Levine,M.T.
    Recurrent duplication and diversification of a vital DNA repair gene family across Drosophila
    Mol Biol Evol (2024) 41, msae113-msae113. PubMed  Europe PubMed DOI
  7. 2023
  8. Anticevic,I., Otten,C., Vinkovic,L., Jukic,L. and Popovic,M.
    Tyrosyl-DNA phosphodiesterase 1 (TDP1) and SPRTN protease repair histone 3 and topoisomerase 1 DNA-protein crosslinks in vivo
    Open Biol (2023) 13, 230113-230113. PubMed  Europe PubMed DOI  PMC  EPMC
  9. Glumac,M., Polovic,M., Batel,A., Gelemanovic,A., Macek,B., Velic,A. and Marinovic-Terzic,I.
    SPRTN-dependent DPC degradation precedes repair of damaged DNA: a proof of concept revealed by the STAR assay
    Nucleic Acids Res (2023) 51, e35-e35. PubMed  Europe PubMed DOI  PMC  EPMC
  10. Noireterre,A., Serbyn,N., Bagdiul,I. and Stutz,F.
    Ubx5-Cdc48 assists the protease Wss1 at DNA-protein crosslink sites in yeast
    EMBO J (2023) 42, e113609-e113609. PubMed  Europe PubMed DOI  PMC  EPMC
  11. Weickert,P., Li,H.Y., Gotz,M.J., Durauer,S., Yaneva,D., Zhao,S., Cordes,J., Acampora,A.C., Forne,I., Imhof,A. and Stingele,J.
    SPRTN patient variants cause global-genome DNA-protein crosslink repair defects
    Nat Commun (2023) 14, 352-352. PubMed  Europe PubMed DOI  PMC  EPMC
  12. 2022
  13. Coleman,K.E., Yin,Y., Lui,S.K.L., Keegan,S., Fenyo,D., Smith,D.J., Rothenberg,E. and Huang,T.T.
    USP1-trapping lesions as a source of DNA replication stress and genomic instability
    Nat Commun (2022) 13, 1740-1740. PubMed  Europe PubMed DOI  PMC  EPMC
  14. Kroning,A., van den Boom,J., Kracht,M., Kueck,A.F. and Meyer,H.
    Ubiquitin-directed AAA+ ATPase p97/VCP unfolds stable proteins crosslinked to DNA for proteolysis by SPRTN
    J Biol Chem (2022) 298, 101976-101976. PubMed  Europe PubMed DOI  PMC  EPMC
  15. Nakano,T., Moriwaki,T., Tsuda,M., Miyakawa,M., Hanaichi,Y., Sasanuma,H., Hirota,K., Kawanishi,M., Ide,H., Tano,K. and Bessho,T.
    SPRTN and TDP1/TDP2 Independently Suppress 5-Aza-2'-deoxycytidine-Induced Genomic Instability in Human TK6 Cell Line
    Chem Res Toxicol (2022) 35, 2059-2067. PubMed  Europe PubMed DOI
  16. Perry,M. and Ghosal,G.
    Mechanisms and Regulation of DNA-Protein Crosslink Repair During DNA Replication by SPRTN Protease
    Front Mol Biosci (2022) 9, 916697-916697. PubMed  Europe PubMed DOI  PMC  EPMC  V
  17. 2021
  18. Hacker,L., Dorn,A., Enderle,J. and Puchta,H.
    The repair of topoisomerase 2 cleavage complexes in Arabidopsis
    Plant Cell (2021) PubMed  Europe PubMed DOI
  19. Na,J., Newman,J.A., Then,C.K., Syed,J., Vendrell,I., Torrecilla,I., Ellermann,S., Ramadan,K., Fischer,R. and Kiltie,A.E.
    SPRTN protease-cleaved MRE11 decreases DNA repair and radiosensitises cancer cells
    Cell Death Dis (2021) 12, 165-165. PubMed  Europe PubMed DOI  PMC  EPMC
  20. Perry,M., Biegert,M., Kollala,S.S., Mallard,H., Su,G., Kodavati,M., Kreiling,N., Holbrook,A. and Ghosal,G.
    USP11 mediates repair of DNA-protein crosslinks by deubiquitinating SPRTN metalloprotease
    J Biol Chem (2021) , 100396-100396. PubMed  Europe PubMed DOI
  21. Reinking,H.K. and Stingele,J.
    Protein-oligonucleotide conjugates as model substrates for DNA-protein crosslink repair proteases
    STAR Protoc (2021) 2, 100591-100591. PubMed  Europe PubMed DOI  PMC  EPMC
  22. Saha,L.K., Murai,Y., Saha,S., Jo,U., Tsuda,M., Takeda,S. and Pommier,Y.
    Replication-dependent cytotoxicity and Spartan-mediated repair of trapped PARP1-DNA complexes
    Nucleic Acids Res (2021) 49, 10493-10506. PubMed  Europe PubMed DOI  PMC  EPMC
  23. Zhao,S., Kieser,A., Li,H.Y., Reinking,H.K., Weickert,P., Euteneuer,S., Yaneva,D., Acampora,A.C., Gotz,M.J., Feederle,R. and Stingele,J.
    A ubiquitin switch controls autocatalytic inactivation of the DNA-protein crosslink repair protease SPRTN
    Nucleic Acids Res (2021) 49, 902-915. PubMed  Europe PubMed DOI
  24. 2020
  25. Dokshin,G.A., Davis,G.M., Sawle,A.D., Eldridge,M.D., Nicholls,P.K., Gourley,T.E., Romer,K.A., Molesworth,L.W., Tatnell,H.R., Ozturk,A.R., de Rooij,D.G., Hannon,G.J., Page,D.C., Mello,C.C. and Carmell,M.A.
    GCNA Interacts with spartan and topoisomerase II to regulate genome stability
    Dev Cell (2020) 52, 53-68. PubMed  Europe PubMed DOI
  26. Fielden,J., Wiseman,K., Torrecilla,I., Li,S., Hume,S., Chiang,S.C., Ruggiano,A., Narayan Singh,A., Freire,R., Hassanieh,S., Domingo,E., Vendrell,I., Fischer,R., Kessler,B.M., Maughan,T.S., El-Khamisy,S.F. and Ramadan,K.
    TEX264 coordinates p97- and SPRTN-mediated resolution of topoisomerase 1-DNA adducts
    Nat Commun (2020) 11, 1274-1274. PubMed  Europe PubMed DOI  PMC  EPMC
  27. Homchan,A., Sukted,J., Matangkasombut,O. and Pakotiprapha,D.
    Emerging roles of Wss1 in the survival of Candida albicans under genotoxic stresses
    Curr Genet (2020) PubMed  Europe PubMed DOI
  28. Huang,J., Zhou,Q., Gao,M., Nowsheen,S., Zhao,F., Kim,W., Zhu,Q., Kojima,Y., Yin,P., Zhang,Y., Guo,G., Tu,X., Deng,M., Luo,K., Qin,B., Machida,Y. and Lou,Z.
    Tandem Deubiquitination and Acetylation of SPRTN Promotes DNA-Protein Crosslink Repair and Protects against Aging
    Mol Cell (2020) PubMed  Europe PubMed DOI
  29. Reinking,H.K., Hofmann,K. and Stingele,J.
    Function and evolution of the DNA-protein crosslink proteases Wss1 and SPRTN
    DNA Repair (Amst) (2020) 88, 102822-102822. PubMed  Europe PubMed DOI
  30. Reinking,H.K., Kang,H.S., Gotz,M.J., Li,H.Y., Kieser,A., Zhao,S., Acampora,A.C., Weickert,P., Fessler,E., Jae,L.T., Sattler,M. and Stingele,J.
    DNA Structure-Specific Cleavage of DNA-Protein Crosslinks by the SPRTN Protease
    Mol Cell (2020) PubMed  Europe PubMed DOI
  31. Ruggiano,A. and Ramadan,K.
    The Trinity of SPRTN Protease Regulation
    Trends Biochem Sci (2020) PubMed  Europe PubMed DOI
  32. Sun,Y., Saha,L.K., Saha,S., Jo,U. and Pommier,Y.
    Debulking of topoisomerase DNA-protein crosslinks (TOP-DPC) by the proteasome, non-proteasomal and non-proteolytic pathways
    DNA Repair (Amst) (2020) 94, 102926-102926. PubMed  Europe PubMed DOI
  33. Zhang,H., Xiong,Y. and Chen,J.
    DNA-protein cross-link repair: what do we know now?
    Cell Biosci (2020) 10, 3-3. PubMed  Europe PubMed DOI  PMC  EPMC
  34. 2019
  35. Borgermann,N., Ackermann,L., Schwertman,P., Hendriks,I.A., Thijssen,K., Liu,J.C., Lans,H., Nielsen,M.L. and Mailand,N.
    SUMOylation promotes protective responses to DNA-protein crosslinks
    EMBO J (2019) 38, e101496-e101496. PubMed  Europe PubMed DOI
  36. Bruhn,C. and Foiani,M.
    A model of DNA damage response activation at stalled replication forks by SPRTN
    Nat Commun (2019) 10, 5671-5671. PubMed  Europe PubMed DOI  PMC  EPMC
  37. Halder,S., Torrecilla,I., Burkhalter,M.D., Popovic,M., Fielden,J., Vaz,B., Oehler,J., Pilger,D., Lessel,D., Wiseman,K., Singh,A.N., Vendrell,I., Fischer,R., Philipp,M. and Ramadan,K.
    SPRTN protease and checkpoint kinase 1 cross-activation loop safeguards DNA replication
    Nat Commun (2019) 10, 3142-3142. PubMed  Europe PubMed DOI  PMC  EPMC
  38. Larsen,N.B., Gao,A.O., Sparks,J.L., Gallina,I., Wu,R.A., Mann,M., Raschle,M., Walter,J.C. and Duxin,J.P.
    Replication-coupled DNA-protein crosslink repair by SPRTN and the proteasome in Xenopus egg extracts
    Mol Cell (2019) 73, 574-588. PubMed  Europe PubMed DOI
  39. Li,F., Raczynska,J.E., Chen,Z. and Yu,H.
    Structural insight into DNA-dependent activation of human metalloprotease Spartan
    Cell Rep (2019) 26, 3336-3346. PubMed  Europe PubMed DOI  S
  40. 2018
  41. Fielden,J., Ruggiano,A., Popovic,M. and Ramadan,K.
    DNA protein crosslink proteolysis repair: from yeast to premature ageing and cancer in humans
    DNA Repair (Amst) (2018) 71, 198-204. PubMed  Europe PubMed DOI
  42. Ide,H., Nakano,T., Salem,A.M.H. and Shoulkamy,M.I.
    DNA-protein cross-links: formidable challenges to maintaining genome integrity
    DNA Repair (Amst) (2018) 71, 190-197. PubMed  Europe PubMed DOI
  43. 2017
  44. Morocz,M., Zsigmond,E., Toth,R., Enyedi,M.Z., Pinter,L. and Haracska,L.
    DNA-dependent protease activity of human Spartan facilitates replication of DNA-protein crosslink-containing DNA
    Nucleic Acids Res (2017) 45, 3172-3188. PubMed  Europe PubMed DOI
  45. Nakano,T., Xu,X., Salem,A.M.H., Shoulkamy,M.I. and Ide,H.
    Radiation-induced DNA-protein cross-links: Mechanisms and biological significance
    Free Radic Biol Med (2017) 107, 136-145. PubMed  Europe PubMed DOI
  46. Vaz,B., Popovic,M. and Ramadan,K.
    DNA-Protein crosslink proteolysis repair
    Trends Biochem Sci (2017) 42, 483-495. PubMed  Europe PubMed DOI
  47. 2016
  48. Lopez-Mosqueda,J., Maddi,K., Prgomet,S., Kalayil,S., Marinovic-Terzic,I., Terzic,J. and Dikic,I.
    SPRTN is a mammalian DNA-binding metalloprotease that resolves DNA-protein crosslinks
    elife (2016) 5, e21491-e21491. PubMed  Europe PubMed DOI
  49. Stingele,J., Bellelli,R., Alte,F., Hewitt,G., Sarek,G., Maslen,S.L., Tsutakawa,S.E., Borg,A., Kjaer,S., Tainer,J.A., Skehel,J.M., Groll,M. and Boulton,S.J.
    Mechanism and regulation of DNA-protein crosslink repair by the DNA-dependent metalloprotease SPRTN
    Mol Cell (2016) 64, 688-703. PubMed  Europe PubMed DOI  M
  50. Vaz,B., Popovic,M., Newman,J.A., Fielden,J., Aitkenhead,H., Halder,S., Singh,A.N., Vendrell,I., Fischer,R., Torrecilla,I., Drobnitzky,N., Freire,R., Amor,D.J., Lockhart,P.J., Kessler,B.M., McKenna,G.W., Gileadi,O. and Ramadan,K.
    Metalloprotease SPRTN/DVC1 orchestrates replication-coupled DNA-protein crosslink repair
    Mol Cell (2016) 64, 704-719. PubMed  Europe PubMed DOI
  51. 2015
  52. Stingele,J., Habermann,B. and Jentsch,S.
    DNA-protein crosslink repair: proteases as DNA repair enzymes
    Trends Biochem Sci (2015) 40, 67-71. PubMed  Europe PubMed DOI
  53. Stingele,J. and Jentsch,S.
    DNA-protein crosslink repair
    Nat Rev Mol Cell Biol (2015) 16, 455-460. PubMed  Europe PubMed DOI
  54. 2014
  55. Lessel,D., Vaz,B., Halder,S., Lockhart,P.J., Marinovic-Terzic,I., Lopez-Mosqueda,J., Philipp,M., Sim,J.C., Smith,K.R., Oehler,J., Cabrera,E., Freire,R., Pope,K., Nahid,A., Norris,F., Leventer,R.J., Delatycki,M.B., Barbi,G., von Ameln,S., Hogel,J., Degoricija,M., Fertig,R., Burkhalter,M.D., Hofmann,K., Thiele,H., Altmuller,J., Nurnberg,G., Nurnberg,P., Bahlo,M., Martin,G.M., Aalfs,C.M., Oshima,J., Terzic,J., Amor,D.J., Dikic,I., Ramadan,K. and Kubisch,C.
    Mutations in SPRTN cause early onset hepatocellular carcinoma, genomic instability and progeroid features
    Nat Genet (2014) 46, 1239-1244. PubMed  Europe PubMed DOI  M
  56. Maskey,R.S., Kim,M.S., Baker,D.J., Childs,B., Malureanu,L.A., Jeganathan,K.B., Machida,Y., van Deursen,J.M. and Machida,Y.J.
    Spartan deficiency causes genomic instability and progeroid phenotypes
    Nat Commun (2014) 5, 5744-5744. PubMed  Europe PubMed DOI  PMC  EPMC
  57. 2013
  58. Fang,Q.
    DNA-protein crosslinks processed by nucleotide excision repair and homologous recombination with base and strand preference in E. coli model system
    Mutat Res (2013) 741-742, 1-10. PubMed  Europe PubMed DOI  PMC  EPMC
  59. Kim,M.S., Machida,Y., Vashisht,A.A., Wohlschlegel,J.A., Pang,Y.P. and Machida,Y.J.
    Regulation of error-prone translesion synthesis by Spartan/C1orf124
    Nucleic Acids Res (2013) 41, 1661-1668. PubMed  Europe PubMed DOI  PMC  EPMC
  60. 2012
  61. Centore,R.C., Yazinski,S.A., Tse,A. and Zou,L.
    Spartan/C1orf124, a reader of PCNA ubiquitylation and a regulator of UV-induced DNA damage response
    Mol Cell (2012) 46, 625-635. PubMed  Europe PubMed DOI  PMC  EPMC
  62. Davis,E.J., Lachaud,C., Appleton,P., Macartney,T.J., Nathke,I. and Rouse,J.
    DVC1 (C1orf124) recruits the p97 protein segregase to sites of DNA damage
    Nat Struct Mol Biol (2012) 19, 1093-1100. PubMed  Europe PubMed DOI
  63. Ghosal,G., Leung,J.W., Nair,B.C., Fong,K.W. and Chen,J.
    Proliferating cell nuclear antigen (PCNA)-binding protein C1orf124 is a regulator of translesion synthesis
    J Biol Chem (2012) 287, 34225-34233. PubMed  Europe PubMed DOI  PMC  EPMC
  64. Juhasz,S., Balogh,D., Hajdu,I., Burkovics,P., Villamil,M.A., Zhuang,Z. and Haracska,L.
    Characterization of human Spartan/C1orf124, an ubiquitin-PCNA interacting regulator of DNA damage tolerance
    Nucleic Acids Res (2012) 40, 10795-10808. PubMed  Europe PubMed DOI  PMC  EPMC
  65. Machida,Y., Kim,M.S. and Machida,Y.J.
    Spartan/C1orf124 is important to prevent UV-induced mutagenesis
    Cell Cycle (2012) 11, 3395-3402. PubMed  Europe PubMed DOI  PMC  EPMC
  66. Mosbech,A., Gibbs-Seymour,I., Kagias,K., Thorslund,T., Beli,P., Povlsen,L., Nielsen,S.V., Smedegaard,S., Sedgwick,G., Lukas,C., Hartmann-Petersen,R., Lukas,J., Choudhary,C., Pocock,R., Bekker-Jensen,S. and Mailand,N.
    DVC1 (C1orf124) is a DNA damage-targeting p97 adaptor that promotes ubiquitin-dependent responses to replication blocks
    Nat Struct Mol Biol (2012) 19, 1084-1092. PubMed  Europe PubMed DOI