Serpini1

Summary

Gene Symbol: Serpini1
Description: serine (or cysteine) peptidase inhibitor, clade I, member 1
Alias: AI837402, PI-12, PI12, Spi17, neuroserpin, peptidase inhibitor 12, protease inhibitor 17, serine protease inhibitor 17, serpin I1
Species: mouse
Products:     Serpini1

Top Publications

  1. Ishigami S, Sandkvist M, Tsui F, Moore E, Coleman T, Lawrence D. Identification of a novel targeting sequence for regulated secretion in the serine protease inhibitor neuroserpin. Biochem J. 2007;402:25-34 pubmed
    Ns (neuroserpin) is a member of the serpin (serine protease inhibitor) gene family that is primarily expressed within the central nervous system...
  2. Cinelli P, Madani R, Tsuzuki N, Vallet P, Arras M, Zhao C, et al. Neuroserpin, a neuroprotective factor in focal ischemic stroke. Mol Cell Neurosci. 2001;18:443-57 pubmed
    ..neurodegenerative processes in many neural pathologies, we studied whether the endogenous tPA antagonist neuroserpin has a neuroprotective effect in an animal model of focal ischemic stroke...
  3. Lebeurrier N, Launay S, Macrez R, Maubert E, Legros H, Leclerc A, et al. Anti-Mullerian-hormone-dependent regulation of the brain serine-protease inhibitor neuroserpin. J Cell Sci. 2008;121:3357-65 pubmed publisher
    The balance between tissue-type plasminogen activator (tPA) and one of its inhibitors, neuroserpin, has crucial roles in the central nervous system, including the control of neuronal migration, neuronal plasticity and neuronal death...
  4. Wu J, Echeverry R, Guzman J, Yepes M. Neuroserpin protects neurons from ischemia-induced plasmin-mediated cell death independently of tissue-type plasminogen activator inhibition. Am J Pathol. 2010;177:2576-84 pubmed publisher
    The serine proteinase tissue-type plasminogen activator (tPA) and the serine proteinase inhibitor neuroserpin are both expressed in areas of the brain with the highest vulnerability to hypoxia/ischemia...
  5. Osterwalder T, Cinelli P, Baici A, Pennella A, Krueger S, Schrimpf S, et al. The axonally secreted serine proteinase inhibitor, neuroserpin, inhibits plasminogen activators and plasmin but not thrombin. J Biol Chem. 1998;273:2312-21 pubmed
    b>Neuroserpin is an axonally secreted serine proteinase inhibitor that is expressed in neurons during embryogenesis and in the adult nervous system...
  6. Briand C, Kozlov S, Sonderegger P, Grutter M. Crystal structure of neuroserpin: a neuronal serpin involved in a conformational disease. FEBS Lett. 2001;505:18-22 pubmed
    The protease inhibitor neuroserpin regulates the development of the nervous system and its plasticity in the adult. Neuroserpins carrying the Ser53Pro or Ser56Arg mutation form polymers in neuronal cells...
  7. Wannier Morino P, Rager G, Sonderegger P, Grabs D. Expression of neuroserpin in the visual cortex of the mouse during the developmental critical period. Eur J Neurosci. 2003;17:1853-60 pubmed
    The neuronal serine protease inhibitor neuroserpin is widely expressed in the developing and adult brain...
  8. Lebeurrier N, Liot G, Lopez Atalaya J, Orset C, Fernandez Monreal M, Sonderegger P, et al. The brain-specific tissue-type plasminogen activator inhibitor, neuroserpin, protects neurons against excitotoxicity both in vitro and in vivo. Mol Cell Neurosci. 2005;30:552-8 pubmed
    Considering its brain-specific expression, neuroserpin (NS), a potent inhibitor of tissue-type plasminogen activator (tPA), might be a good therapeutic target to limit the pro-excitotoxic effects of tPA within the cerebral parenchyma, ..
  9. Krueger S, Ghisu G, Cinelli P, Gschwend T, Osterwalder T, Wolfer D, et al. Expression of neuroserpin, an inhibitor of tissue plasminogen activator, in the developing and adult nervous system of the mouse. J Neurosci. 1997;17:8984-96 pubmed
    b>Neuroserpin is a serine protease inhibitor of the serpin family that has been identified as an axonally secreted glycoprotein in neuronal cultures of chicken dorsal root ganglia...

More Information

Publications22

  1. Takasawa A, Kato I, Takasawa K, Ishii Y, Yoshida T, Shehata M, et al. Mutation-, aging-, and gene dosage-dependent accumulation of neuroserpin (G392E) in endoplasmic reticula and lysosomes of neurons in transgenic mice. J Biol Chem. 2008;283:35606-13 pubmed publisher
    Mutations in human neuroserpin gene cause an autosomal dementia, familial encephalopathy with neuroserpin inclusion bodies (FENIB)...
  2. Berger P, Kozlov S, Krueger S, Sonderegger P. Structure of the mouse gene for the serine protease inhibitor neuroserpin (PI12). Gene. 1998;214:25-33 pubmed
    b>Neuroserpin (PI12), initially identified as an axonally secreted protein in cultured chicken dorsal root ganglion neurons, belongs to the serpin family of the serine protease inhibitors and is mainly expressed by neurons of both the ..
  3. Chang W, Chang N, Lin S, Wu C, Wu F. Tissue-specific cancer-related serpin gene cluster at human chromosome band 3q26. Genes Chromosomes Cancer. 2000;29:240-55 pubmed
    ..members: the pancreas-specific protease inhibitor, pancpin (PI14), and the brain-associated protease inhibitor, neuroserpin (PI12)...
  4. Gelderblom M, Neumann M, Ludewig P, Bernreuther C, Krasemann S, Arunachalam P, et al. Deficiency in serine protease inhibitor neuroserpin exacerbates ischemic brain injury by increased postischemic inflammation. PLoS ONE. 2013;8:e63118 pubmed publisher
    ..b>Neuroserpin, an endogenous inhibitor of tPA, is up-regulated following cerebral ischemia...
  5. Simonin Y, Charron Y, Sonderegger P, Vassalli J, Kato A. An inhibitor of serine proteases, neuroserpin, acts as a neuroprotective agent in a mouse model of neurodegenerative disease. J Neurosci. 2006;26:10614-9 pubmed
    ..Transgenic mice overexpressing an axonally secreted inhibitor of serine proteases (neuroserpin) were crossed with mice characterized by a "dying-back" motor neuron disease [progressive motor ..
  6. Ragg H. The role of serpins in the surveillance of the secretory pathway. Cell Mol Life Sci. 2007;64:2763-70 pubmed
    ..The molecular targets of these serpins are often unknown, opening new avenues for future research. ..
  7. Schipanski A, Lange S, Segref A, Gutschmidt A, Lomas D, Miranda E, et al. A novel interaction between aging and ER overload in a protein conformational dementia. Genetics. 2013;193:865-76 pubmed publisher
    ..deposition of aggregated proteins in tauopathies, Parkinson disease, or familial encephalopathy with neuroserpin inclusion bodies (FENIB) leads to impaired protein homeostasis (proteostasis)...
  8. Gu R, Fu L, Jiang C, Xu Y, Wang X, Yu J. Retina Is Protected by Neuroserpin from Ischemic/Reperfusion-Induced Injury Independent of Tissue-Type Plasminogen Activator. PLoS ONE. 2015;10:e0130440 pubmed publisher
    The purpose of the present study was to investigate the potential neuroprotective effect of neuroserpin (NSP) on acute retinal ischemic/reperfusion-induced (IR) injury...
  9. Makarova A, Mikhailenko I, Bugge T, List K, Lawrence D, Strickland D. 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. 2003;278:50250-8 pubmed
    ..of processes in the brain; consequently, their activity is carefully regulated by protease inhibitors, such as neuroserpin. This inhibitor is thought to be secreted by axons at synaptic regions where it controls tissue-type ..
  10. Valiente M, Obenauf A, Jin X, Chen Q, Zhang X, Lee D, et al. Serpins promote cancer cell survival and vascular co-option in brain metastasis. Cell. 2014;156:1002-16 pubmed publisher
    ..Brain metastatic cells from lung cancer and breast cancer express high levels of anti-PA serpins, including neuroserpin and serpin B2, to prevent plasmin generation and its metastasis-suppressive effects...
  11. Madani R, Kozlov S, Akhmedov A, Cinelli P, Kinter J, Lipp H, et al. Impaired explorative behavior and neophobia in genetically modified mice lacking or overexpressing the extracellular serine protease inhibitor neuroserpin. Mol Cell Neurosci. 2003;23:473-94 pubmed
    b>Neuroserpin is a neural serpin that inhibits the extracellular protease tissue-type plasminogen activator (tPA). We have generated neuroserpin-deficient mice which are viable and healthy...
  12. Lee T, Tsang V, Loef E, Birch N. Physiological and pathological functions of neuroserpin: Regulation of cellular responses through multiple mechanisms. Semin Cell Dev Biol. 2017;62:152-159 pubmed publisher
    It is 27 years since neuroserpin was first discovered in the nervous system and identified as a member of the serpin superfamily...
  13. Belorgey D, Hägglöf P, Karlsson Li S, Lomas D. Protein misfolding and the serpinopathies. Prion. 2007;1:15-20 pubmed
    ..An identical process in the neuron specific serpin, neuroserpin, results in the accumulation of polymers in neurons and the dementia FENIB...