Merops Switch to
Peptidases

Inhibitor

Family Clan Small-molecule
inhibitor SEARCHES BLAST
MEROPS

Other
information

Literature for inhibitor I04.025: neuroserpin

Summary Gene structure Alignment Tree Sequences Sequence features Distribution Structure Literature Inhibits

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

    2026
  1. Liao,Y., Zhang,Q., Shi,Q., Liu,P., Zhong,P., Guo,L., Huang,Z., Peng,Y., Liu,W., Zhang,S., Adorjan,I., Fukuzaki,Y., Kawashita,E., Zhang,X.Q., Ma,N., Zhang,X., Molnar,Z. and Shi,L.
    Neuroserpin alleviates cerebral ischemia-reperfusion injury by suppressing ischemia-induced endoplasmic reticulum stress
    Neural Regen Res (2026) 21, 333-345. PubMed  Europe PubMed DOI  PMC  EPMC
    2. 2023
  2. Chitranshi,N., Rajput,R., Godinez,A., Pushpitha,K., Mirzaei,M., Basavarajappa,D., Gupta,V., Sharma,S., You,Y., Galliciotti,G., Salekdeh,G.H., Baker,M.S., Graham,S.L. and Gupta,V.K.
    Neuroserpin gene therapy inhibits retinal ganglion cell apoptosis and promotes functional preservation in glaucoma
    Mol Ther (2023) 31, 2056-2076. PubMed  Europe PubMed DOI
    3. 2021
  3. D'Acunto,E., Fra,A., Visentin,C., Manno,M., Ricagno,S., Galliciotti,G. and Miranda,E.
    Neuroserpin: structure, function, physiology and pathology
    Cell Mol Life Sci (2021) PubMed  Europe PubMed DOI  V
  4. Fatima,S., Ansari,S., Bano,S., Ahamad,S., Ishqi,H.M., Tabish,M., Gupta,D., Rehman,S.U. and Jairajpuri,M.A.
    Detection of truncated isoforms of human neuroserpin lacking the reactive center loop: Implications in noninhibitory role
    IUBMB Life (2021) PubMed  Europe PubMed DOI
  5. Kement,D., Reumann,R., Schostak,K., Voss,H., Douceau,S., Dottermusch,M., Schweizer,M., Schluter,H., Vivien,D., Glatzel,M. and Galliciotti,G.
    Neuroserpin Is Strongly Expressed in the Developing and Adult Mouse Neocortex but Its Absence Does Not Perturb Cortical Lamination and Synaptic Proteome
    Front Neuroanat (2021) 15, 627896-627896. PubMed  Europe PubMed DOI  PMC  EPMC
    6. 2020
  6. Hermann,M., Reumann,R., Schostak,K., Kement,D., Gelderblom,M., Bernreuther,C., Frischknecht,R., Schipanski,A., Marik,S., Krasemann,S., Sepulveda-Falla,D., Schweizer,M., Magnus,T., Glatzel,M. and Galliciotti,G.
    Deficits in developmental neurogenesis and dendritic spine maturation in mice lacking the serine protease inhibitor neuroserpin
    Mol Cell Neurosci (2020) 102, 103420-103420. PubMed  Europe PubMed DOI  K
  7. Visentin,C., Broggini,L., Sala,B.M., Russo,R., Barbiroli,A., Santambrogio,C., Nonnis,S., Dubnovitsky,A., Bolognesi,M., Miranda,E., Achour,A. and Ricagno,S.
    Glycosylation tunes neuroserpin physiological and pathological properties
    Int J Mol Sci (2020) 21 PubMed  Europe PubMed DOI
    8. 2017
  8. Reumann,R., Vierk,R., Zhou,L., Gries,F., Kraus,V., Mienert,J., Romswinkel,E., Morellini,F., Ferrer,I., Nicolini,C., Fahnestock,M., Rune,G., Glatzel,M. and Galliciotti,G.
    The serine protease inhibitor neuroserpin is required for normal synaptic plasticity and regulates learning and social behavior
    Learn Mem (2017) 24, 650-659. PubMed  Europe PubMed DOI  PMC  EPMC
    9. 2016
  9. Ambadapadi,S., Munuswamy-Ramanujam,G., Zheng,D., Sullivan,C., Dai,E., Morshed,S., McFadden,B., Feldman,E., Pinard,M., McKenna,R., Tibbetts,S. and Lucas,A.
    Reactive center loop (RCL) peptides derived from serpins display independent coagulation and immune modulating activities
    J Biol Chem (2016) 291, 2874-2887. PubMed  Europe PubMed DOI
  10. Carlson,K.S., Nguyen,L., Schwartz,K., Lawrence,D.A. and Schwartz,B.S.
    Neuroserpin differentiates between forms of tissue type plasminogen activator via pH dependent deacylation
    Front Cell Neurosci (2016) 10, 154-154. PubMed  Europe PubMed DOI  PMC  EPMC
    11. 2015
  11. Lee,T.W., Yang,A.S., Brittain,T. and Birch,N.P.
    An analysis approach to identify specific functional sites in orthologous proteins using sequence and structural information: application to neuroserpin reveals regions that differentially regulate inhibitory activity
    Proteins (2015) 83, 135-152. PubMed  Europe PubMed DOI
  12. Noto,R., Randazzo,L., Raccosta,S., Caccia,S., Moriconi,C., Miranda,E., Martorana,V. and Manno,M.
    The stability and activity of human neuroserpin are modulated by a salt bridge that stabilises the reactive centre loop
    Sci Rep (2015) 5, 13666-13666. PubMed  Europe PubMed DOI
    13. 2014
  13. Tsang,V.W., Young,D., During,M.J. and Birch,N.P.
    AAV-Mediated overexpression of neuroserpin in the hippocampus decreases PSD-95 expression but does not affect hippocampal-dependent learning and memory
    PLoS ONE (2014) 9, e91050-e91050. PubMed  Europe PubMed DOI  PMC  EPMC
    14. 2013
  14. Gelderblom,M., Neumann,M., Ludewig,P., Bernreuther,C., Krasemann,S., Arunachalam,P., Gerloff,C., Glatzel,M. and Magnus,T.
    Deficiency in serine protease inhibitor neuroserpin exacerbates ischemic brain injury by increased postischemic inflammation
    PLoS ONE (2013) 8, e63118-e63118. PubMed  Europe PubMed DOI  PMC  EPMC  K
    15. 2011
  15. Hagen,M.C., Murrell,J.R., Delisle,M.B., Andermann,E., Andermann,F., Guiot,M.C. and Ghetti,B.
    Encephalopathy with neuroserpin inclusion bodies presenting as progressive myoclonus epilepsy and associated with a novel mutation in the proteinase inhibitor 12 gene
    Brain Pathol (2011) 21, 575-582. PubMed  Europe PubMed DOI  M
    16. 2010
  16. Munuswamy-Ramanujam,G., Dai,E., Liu,L., Shnabel,M., Sun,Y.M., Bartee,M., Lomas,D.A. and Lucas,A.R.
    Neuroserpin, a thrombolytic serine protease inhibitor (serpin), blocks transplant vasculopathy with associated modification of T-helper cell subsets
    Thromb Haemost (2010) 103, 545-555. PubMed  Europe PubMed DOI
  17. Ricagno,S., Pezzullo,M., Barbiroli,A., Manno,M., Levantino,M., Santangelo,M.G., Bonomi,F. and Bolognesi,M.
    Two latent and two hyperstable polymeric forms of human neuroserpin
    Biophys J (2010) 99, 3402-3411. PubMed  Europe PubMed DOI  PMC  EPMC
  18. Srikeerthana,K. and De Causmaecker,P.
    Comparative sequence and structural analyses of neuroserpin: the serine protease inhibitor family
    [ISSN:ISBN:978-1-60558-722-6] (2010) DOI
    19. 2009
  19. Ricagno,S., Caccia,S., Sorrentino,G., Antonini,G. and Bolognesi,M.
    Human neuroserpin: structure and time-dependent inhibition
    J Mol Biol (2009) 388, 109-121. PubMed  Europe PubMed DOI  S
  20. Takehara,S., Onda,M., Zhang,J., Nishiyama,M., Yang,X., Mikami,B. and Lomas,D.A.
    The 2.1-A crystal structure of native neuroserpin reveals unique structural elements that contribute to conformational instability
    J Mol Biol (2009) 388, 11-20. PubMed  Europe PubMed DOI  S
    21. 2008
  21. Kumar,A. and Ragg,H.
    Ancestry and evolution of a secretory pathway serpin
    BMC Evol Biol (2008) 8, 250-250. PubMed  Europe PubMed DOI  PMC  EPMC
    22. 2007
  22. [YEAR:9-1-2007]Chen,P.Y., Chang,W.S., Chou,R.H., Lai,Y.K., Lin,S.C., Chi,C.Y. and Wu,C.W.
    Two non-homologous brain diseases-related genes, SERPINI1 and PDCD10, are tightly linked by an asymmetric bidirectional promoter in an evolutionarily conserved manner
    BMC Mol Biol (9-1-2007) 8, 2-2. PubMed  Europe PubMed DOI  PMC  EPMC
  23. Ishigami,S., Sandkvist,M., Tsui,F., Moore,E., Coleman,T.A. and Lawrence,D.A.
    Identification of a novel targeting sequence for regulated secretion in the serine protease inhibitor neuroserpin
    Biochem J (2007) 402, 25-34. PubMed  Europe PubMed DOI  PMC  EPMC
    24. 2006
  24. [YEAR:1-1-2006]Galliciotti,G. and Sonderegger,P.
    Neuroserpin
    Front Biosci (1-1-2006) 11, 33-45. PubMed  Europe PubMed DOI  V
  25. [YEAR:7-2-2006]Miranda,E. and Lomas,D.A.
    Neuroserpin: a serpin to think about
    Cell Mol Life Sci (7-2-2006) 63, 709-722. PubMed  Europe PubMed DOI  V
  26. Simonin,Y., Charron,Y., Sonderegger,P., Vassalli,J.D. and Kato,A.C.
    An inhibitor of serine proteases, neuroserpin, acts as a neuroprotective agent in a mouse model of neurodegenerative disease
    J Neurosci (2006) 26, 10614-10619. PubMed  Europe PubMed DOI
    27. 2004
  27. [YEAR:16-4-2004]Miranda,E., Romisch,K. and Lomas,D.A.
    Mutants of neuroserpin that cause dementia accumulate as polymers within the endoplasmic reticulum
    J Biol Chem (16-4-2004) 279, 28283-28291. PubMed  Europe PubMed DOI
  28. [YEAR:7-4-2004]Navarro-Yubero,C., Cuadrado,A., Sonderegger,P. and Munoz,A.
    Neuroserpin is post-transcriptionally regulated by thyroid hormone
    Brain Res Mol Brain Res (7-4-2004) 123, 56-65. PubMed  Europe PubMed DOI
  29. Yepes,M. and Lawrence,D.A.
    Neuroserpin: a selective inhibitor of tissue-type plasminogen activator in the central nervous system
    Thromb Haemost (2004) 91, 457-464. PubMed  Europe PubMed DOI
  30. Yepes,M. and Lawrence,D.A.
    Tissue-type plasminogen activator and neuroserpin: a well-balanced act in the nervous system?
    Trends Cardiovasc Med (2004) 14, 173-180. PubMed  Europe PubMed DOI
  31. Yepes,M. and Lawrence,D.A.
    New functions for an old enzyme: nonhemostatic roles for tissue-type plasminogen activator in the central nervous system
    Exp Biol Med (Maywood) (2004) 229, 1097-1104. PubMed  Europe PubMed
    32. 2003
  32. [YEAR:12-12-2003]Makarova,A., Mikhailenko,I., Bugge,T.H., List,K., Lawrence,D.A. and Strickland,D.K.
    The low density lipoprotein receptor-related protein modulates protease activity in the brain by mediating the cellular internalization of both neuroserpin and neuroserpin-tissue-type plasminogen activator complexes
    J Biol Chem (12-12-2003) 278, 50250-50258. PubMed  Europe PubMed DOI
    33. 2002
  33. [YEAR:6-12-2002]Barker-Carlson,K., Lawrence,D.A. and Schwartz,B.S.
    Acyl-enzyme complexes between tissue-type plasminogen activator and neuroserpin are short-lived in vitro
    J Biol Chem (6-12-2002) 277, 46852-46857. PubMed  Europe PubMed DOI
  34. [YEAR:10-5-2002]Belorgey,D., Crowther,D.C., Mahadeva,R. and Lomas,D.A.
    Mutant neuroserpin (S49P) that causes familial encephalopathy with neuroserpin inclusion bodies is a poor proteinase inhibitor and readily forms polymers in vitro
    J Biol Chem (10-5-2002) 277, 17367-17373. PubMed  Europe PubMed DOI
  35. [YEAR:29-6-2002]Davis,R.L., Shrimpton,A.E., Carrell,R.W., Lomas,D.A., Gerhard,L., Baumann,B., Lawrence,D.A., Yepes,M., Kim,T.S., Ghetti,B., Piccardo,P., Takao,M., Lacbawan,F., Muenke,M., Sifers,R.N., Bradshaw,C.B., Kent,P.F., Collins,G.H., Larocca,D. and Holohan,P.D.
    Association between conformational mutations in neuroserpin and onset and severity of dementia
    Lancet (29-6-2002) 359, 2242-2247. PubMed  Europe PubMed DOI
  36. Lomas,D.A. and Carrell,R.W.
    Serpinopathies and the conformational dementias
    Nat Rev Genet (2002) 3, 759-768. PubMed  Europe PubMed DOI
  37. Lomas,D.A., Lourbakos,A., Cumming,S.A. and Belorgey,D.
    Hypersensitive mousetraps, alpha1-antitrypsin deficiency and dementia
    Biochem Soc Trans (2002) 30, 89-92. PubMed  Europe PubMed
  38. Parmar,P.K., Coates,L.C., Pearson,J.F., Hill,R.M. and Birch,N.P.
    Neuroserpin regulates neurite outgrowth in nerve growth factor-treated PC12 cells
    J Neurochem (2002) 82, 1406-1415. PubMed  Europe PubMed DOI
  39. Yepes,M., Sandkvist,M., Coleman,T.A., Moore,E., Wu,J.Y., Mitola,D., Bugge,T.H. and Lawrence,D.A.
    Regulation of seizure spreading by neuroserpin and tissue-type plasminogen activator is plasminogen-independent
    J Clin Invest (2002) 109, 1571-1578. PubMed  Europe PubMed DOI  PMC  EPMC
  40. [YEAR:6-8-2002]Zhang,Z., Zhang,L., Yepes,M., Jiang,Q., Li,Q., Arniego,P., Coleman,T.A., Lawrence,D.A. and Chopp,M.
    Adjuvant treatment with neuroserpin increases the therapeutic window for tissue-type plasminogen activator administration in a rat model of embolic stroke
    Circulation (6-8-2002) 106, 740-745. PubMed  Europe PubMed DOI
    41. 2001
  41. [YEAR:7-9-2001]Briand,C., Kozlov,S.V., Sonderegger,P. and Grutter,M.G.
    Crystal structure of neuroserpin: a neuronal serpin involved in a conformational disease
    FEBS Lett (7-9-2001) 505, 18-22. PubMed  Europe PubMed DOI  S
    42. 1998
  42. [YEAR:23-1-1998]Osterwalder,T., Cinelli,P., Baici,A., Pennella,A., Krueger,S.R., Schrimpf,S.P., Meins,M. and Sonderegger,P.
    The axonally secreted serine proteinase inhibitor, neuroserpin, inhibits plasminogen activators and plasmin but not thrombin
    J Biol Chem (23-1-1998) 273, 2312-2321. PubMed  Europe PubMed DOI
    43. 1997
  43. [YEAR:26-12-1997]Hastings,G.A., Coleman,T.A., Haudenschild,C.C., Stefansson,S., Smith,E.P., Barthlow,R., Cherry,S., Sandkvist,M. and Lawrence,D.A.
    Neuroserpin, a brain-associated inhibitor of tissue plasminogen activator is localized primarily in neurons. Implications for the regulation of motor learning and neuronal survival
    J Biol Chem (26-12-1997) 272, 33062-33067. PubMed  Europe PubMed DOI
    44. 1996
  44. [YEAR:17-6-1996]Osterwalder,T., Contartese,J., Stoeckli,E.T., Kuhn,T.B. and Sonderegger,P.
    Neuroserpin, an axonally secreted serine protease inhibitor
    EMBO J (17-6-1996) 15, 2944-2953. PubMed  Europe PubMed  PMC  EPMC

feedback
Page created 8-September-2023

EMBL-EBI logo

© 2026 EBI
Terms of use