Neuroserpin: A serpin to think about

Cellular and Molecular Life Sciences

Volumn 63, Issue 6, 2006, Pages 709-722

  Miranda, E ^a   Lomas, D A ^a  

^a UNIVERSITY OF CAMBRIDGE   (United Kingdom)

Author keywords

Conformational disease; FENIB; Nervous system; Polymers; Proteinase inhibitors; Proteinases; Serpin; Serpinopathies; Tissue type plasminogen activator (TPA)

Indexed keywords

NEUROSERPIN; SERINE PROTEINASE INHIBITOR; TISSUE PLASMINOGEN ACTIVATOR; UNCLASSIFIED DRUG;

AMINO ACID SEQUENCE; AUTOSOMAL DOMINANT DISORDER; BEHAVIOR; BRAIN DEVELOPMENT; BRAIN DISEASE; BRAIN FUNCTION; BRAIN ISCHEMIA; CELL INCLUSION; CLINICAL FEATURE; DEMENTIA; ENDOPLASMIC RETICULUM; ENZYME ACTIVITY; ENZYME RELEASE; EPILEPSY; HOMEOSTASIS; HUMAN; IN VITRO STUDY; LEARNING; MEMORY; NERVE CELL DIFFERENTIATION; NERVE GROWTH; NEUROPROTECTION; NONHUMAN; POINT MUTATION; PROTEIN ANALYSIS; PROTEIN CONFORMATION; PROTEIN EXPRESSION; PROTEIN FUNCTION; PROTEIN LOCALIZATION; PROTEIN SECRETION; PROTEIN STRUCTURE; REVIEW; SEQUENCE HOMOLOGY; SYNAPTOGENESIS; TISSUE DISTRIBUTION;

ANIMALS; BRAIN; DEMENTIA; HUMANS; MUTATION; NERVOUS SYSTEM DISEASES; NEUROPEPTIDES; SERINE PROTEINASE INHIBITORS; SERPINS;

EID: 33645078896     PISSN: 1420682X     EISSN: 15691632     Source Type: Journal    
DOI: 10.1007/s00018-005-5077-4     Document Type: Review

References (168)

1. Ruegg M. A., Stoeckli E. T., Kuhn T. B., Heller M., Zuellig R. and Sonderegger P. (1989) Purification of axonin-1, a protein that is secreted from axons during neurogenesis. EMBO J. 8: 55-63
2. Stoeckli E. T., Lemkin P. F., Kuhn T. B., Ruegg M. A., Heller M. and Sonderegger P. (1989) Identification of proteins secreted from axons of embryonic dorsal-root-ganglia neurons. Eur. J. Biochem. 180: 249-258
3. Osterwalder T., Contartese J., Stoeckli E. T., Kuhn T. B. and Sonderegger P. (1996) Neuroserpin, an axonally secreted serine protease inhibitor. EMBO J. 15: 2944-2953
4. Silverman G. A., Bird P. I., Carrell R. W., Church F. C., Coughlin P. B., Gettins P. et al. (2001) The serpins are an expanding superfamily of structurally similar but functionally diverse proteins. Evolution, novel functions, mechanism of inhibition and a revised nomenclature. J. Biol. Chem. 276: 33293-33296
5. Gettins P. G. (2002) Serpin structure, mechanism and function. Chem. Rev. 102: 4751-4804
6. Schrimpf S. P., Bleiker A. J., Brecevic L., Kozlov S. V., Berger P., Osterwalder T. et al. (1997) Human neuroserpin (PI12): cDNA cloning and chromosomal localization to 3q26. Genomics 40: 55-62
7. Krueger S. R., Ghisu G.-P., Cinelli P., Gschwend T. P., Osterwalder T., Wolfer D. P. et al. (1997) Expression of neuroserpin, an inhibitor of tissue plasminogen activator, in the developing and adult nervous system of the mouse. J. Neuroscience. 17: 8984-8996
8. Hastings G. A., Coleman T. A., Haudenschild C. C., Stefansson S., Smith E. P., Barthlow R. et al. (1997) Neuroserpin, a brain-associated inhibitor of tissue plasminogen activator is localized primarily in neurones. J. Biol. Chem. 272: 33062-33067
9. Hill R. M., Parmar P. K., Coates L. C., Mezey E., Pearson J. F. and Birch N. P. (2000) Neuroserpin is expressed in the pituitary and adrenal glands and induces the extension of neurite-like processes in AtT-20 cells. Biochem. J. 345: 595-601
10. Huntington J. A., Read R. J. and Carrell R. W. (2000) Structure of a serpin-protease complex shows inhibition by deformation. Nature 407: 923-926
11. Briand C., Kozlov S. V., Sonderegger P. and Grütter M. G. (2001) Crystal structure of neuroserpin: a neuronal serpin involved in a conformational disease. FEBS Lett. 25171: 1-5
12. Osterwalder T., Cinelli P., Baici A., Pennella A., Krueger S. R., Schrimpf S. P. et al. (1998) The axonally secreted serine proteinase inhibitor, neuroserpin, inhibits plasminogen activators and plasmin but not thrombin. J. Biol. Chem. 237: 2312-2321
13. Lawrence D. A., Ginsburg D., Day D. E., Berkenpas M. B., Verhamme I. M., Kvassman J.-O. et al. (1995) Serpin-protease complexes are trapped as stable acyl-enzyme intermediates. J. Biol. Chem. 270: 25309-25312
14. 2- macroglobulin receptor mediates cellular degradation of urokinase receptor-bound complexes. J. Biol. Chem. 267: 14543-14546
15. Bu G., Maksymovitch E. A. and Schwartz A. L. (1993) Receptor-mediated endocytosis of tissue-type plasminogen activator by low density lipoprotein receptor-related protein on human hepatoma HepG2 cells. J. Biol. Chem. 268: 13002-13009
16. Makarova A., Mikhailenko I., Bugge T. H., List K., Lawrence D. A. and Strickland D. K. (2003) The LDL receptor-related protein modulates protease activity in the brain by mediating the cellular internalization of both neuroserpin and neuroserpin:tPA complexes. J. Biol. Chem. 278: 50250-50258
17. Belorgey D., Crowther D. C., Mahadeva R. and Lomas D. A. (2002) Mutant neuroserpin (Ser49Pro) that causes the familial dementia FENIB is a poor proteinase inhibitor and readily forms polymers in vitro. J. Biol. Chem. 277: 17367-17373
18. Barker-Carlson K., Lawrence D. A. and Schwartz B. S. (2002) Acyl-enzyme complexes between tissue type plasminogen activator and neuroserpin are short lived in vitro. J. Biol. Chem. 277: 46852-46857
19. Scott R. W., Bergman B. L., Bajpai A., Hersh R. T., Rodriguez H., Jones B. N. et al. (1985) Protease nexin. Properties and a modified purification procedure. J. Biol. Chem. 260: 7029-7034
20. Binder B. R., Christ G., Gruber F., Grubic N., Hufnagl P., Krebs M. et al. (2002) Plasminogen activator inhibitor 1: physiological and pathophysiological roles. News Physiol. Sci. 17: 56-61
21. Sawdey M. S. and Loskutoff D. J. (1991) Regulation of murine type 1 plasminogen activator inhibitor gene expression in vivo. Tissue specificity and induction by lipopolysaccharide, tumor necrosis factor-alpha and transforming growth factor-beta. J. Clin. Invest. 88: 1346-1353
22. Mansuy I. M., van der Putten H., Schmid P., Meins M., Botteri F. M. and Monard D. (1993) Variable and multiple expression of Protease Nexin-1 during mouse organogenesis and nervous system development. Development 119: 1119-1134
23. Yepes M. and Lawrence D. A. (2004) New functions for an old enzyme: nonhemostatic roles for tissue-type plasminogen activator in the central nervous system. Exp. Biol. Med. (Maywood) 229: 1097-1104
24. Yepes M. and Lawrence D. A. (2004) Tissue-type plasminogen activator and neuroserpin: a well-balanced act in the nervous system? Trends Cardiovasc. Med. 14: 173-180
25. Yepes M., Sandkvist M., Coleman T. A., Moore E., Wu J.-Y., Mitola D. et al. (2002) Regulation of seizure spreading by neuroserpin and tissue-type plasminogen activator is plasminogen independent. J. Clin. Invest. 109: 1571-1578
26. Zhang Z., Zhang L., Yepes M., Jiang Q., Li Q., Arniego P. et al. (2002) Adjuvant treatment with neuroserpin increases the therapeutic window for tissue-type plasminogen activator administration in a rat model of embolic stroke. Circulation 106: 740-745
27. Sumi Y., Dent M. A., Owen D. E., Seeley P. J. and Morris R. J. (1992) The expression of tissue and urokinase-type plasminogen activators in neural development suggests different modes of proteolytic involvement in neuronal growth. Development 116: 625-637
28. Dent M. A., Sumi Y., Morris R. J. and Seeley P. J. (1993) Urokinase-type plasminogen activator expression by neurons and oligodendrocytes during process outgrowth in developing rat brain. Eur. J. Neurosci. 5: 633-647
29. Krystosek A. and Seeds N. W. (1981) Plasminogen activator release at the neuronal growth cone. Science 213: 1532-1534
30. Parmar P. K., Coates L. C., Pearson J. F., Hill R. M. and Birch N. P. (2002) Neuroserpin regulates neurite outgrowth in nerve growth factor treated PC12 cells. J. Neurochemistry. 82: 1406-1415
31. Jacovina A. T., Zhong F., Khazanova E., Lev E., Deora A. B. and Hajjar K. A. (2001) Neuritogenesis and the nerve growth factor-induced differentiation of PC-12 cells requires annexin II-mediated plasmin generation. J. Biol. Chem. 276: 49350-49358
32. Teesalu T., Kulla A., Simisker A., Siren V., Lawrence D. A., Asser T. et al. (2004) Tissue plasminogen activator and neuroserpin are widely expressed in the human central nervous system. Thromb. Haemost. 92: 358-368
33. Berger P., Kozlov S. V., Cinelli P., Kruger S. R., Vogt L. and Sonderegger P. (1999) Neuronal depolarization enhances the transcription of the neuronal serine protease inhibitor neuroserpin. Mol. Cell. Neurosci. 14: 455-467
34. Meiri N., Masos T., Rosenblum K., Miskin R. and Dudai Y. (1994) Overexpression of urokinase-type plasminogen activator in transgenic mice is correlated with impaired learning. Proc. Natl. Acad. Sci. USA 91: 3196-3200
35. Huang Y. Y., Bach M. E., Lipp H. P., Zhuo M., Wolfer D. P., Hawkins R. D. et al. (1996) Mice lacking the gene encoding tissue-type plasminogen activator show a selective interference with late-phase long-term potentiation in both Schaffer collateral and mossy fiber pathways. Proc. Natl. Acad. Sci. USA 93: 8699-8704
36. Pang P. T., Teng H. K., Zaitsev E., Woo N. T., Sakata K., Zhen S. et al. (2004) Cleavage of proBDNF by tPA/plasmin is essential for long-term hippocampal plasticity. Science 306: 487-491
37. Madani R., Kozlov S., Akhmedov A., Cinelli P., Kinter J., Lipp H.-P. et al. (2003) Impaired explorative behavior and neophobia in genetically modified mice lacking or overexpressing the extracellular serine proteinase inhibitor neuroserpin. Mol. Cell Neurosci. 23: 473-494
38. Yepes M., Sandkvist M., Moore E. G., Bugge T. H., Strickland D. K. and Lawrence D. A. (2003) Tissue-type plasminogen activator induces opening of the blood-brain barrier via the LDL receptor-related protein. J. Clin. Invest. 112: 1533-1540
39. Wang Y. F., Tsirka S. E., Strickland S., Stieg P. E., Soriano S. G. and Lipton S. A. (1998) Tissue plasminogen activator (tPA) increases neuronal damage after focal cerebral ischemia in wild-type and tPA-deficient mice. Nat. Med. 4: 228-231
40. Docagne F., Nicole O., Marti H. H., MacKenzie E. T., Buisson A. and Vivien D. (1999) Transforming growth factor-beta1 as a regulator of the serpins/t-PA axis in cerebral ischemia. FASEB J. 13: 1315-1324
41. Yepes M., Sandkvist M., Wong M. K. K., Coleman T. A., Smith E., Cohan S. L. et al. (2000) Neuroserpin reduces cerebral infarct volume and protects neurones from ischaemia-induced apoptosis. Blood 96: 569-576
42. Cinelli P., Madani R., Tsuzuki N., Vallet P., Arras M., Zhao C. N. et al. (2001) Neuroserpin, a neuroprotective factor in focal ischaemic stroke. Mol. Cell. Neurosci. 18: 443-457
43. Cuadrado A., Navarro-Yubero C., Furneaux H., Kinter J., Sonderegger P. and Munoz A. (2002) HuD binds to three AU-rich sequences in the 3′-UTR of neuroserpin mRNA and promotes the accumulation of neuroserpin mRNA and protein. Nucleic Acids Res. 30: 2202-2211
44. Navarro-Yubero C., Cuadrado A., Sonderegger P. and Munoz A. (2004) Neuroserpin is post-transcriptionally regulated by thyroid hormone. Brain Res. Mol. Brain Res. 123: 56-65
45. de Groot D. M. and Martens G. J. (2005) Expression of neuroserpin is linked to neuroendocrine cell activation. Endocrinology 146: 3791-3799
46. Krystosek A. and Seeds N. W. (1984) Peripheral neurons and Schwann cells secrete plasminogen activator. J. Cell Biol. 98: 773-776
47. Pittman R. N. (1985) Release of plasminogen activator and a calcium-dependent metalloprotease from cultured sympathetic and sensory neurons. Dev. Biol. 110: 91-101
48. Friedman G. C. and Seeds N. W. (1994) Tissue plasminogen activator expression in the embryonic nervous system. Brain. Res. Dev. Brain Res. 81: 41-49
49. Sappino A. P., Madani R., Huarte J., Belin D., Kiss J. Z., Wohlwend A. et al. (1993) Extracellular proteolysis in the adult murine brain. J. Clin. Invest. 92: 679-685
50. Ware J. H., Dibenedetto A. J. and Pittman R. N. (1995) Localization of tissue plasminogen activator mRNA in adult rat brain. Brain Res. Bull. 37: 275-281
51. Basham M. E. and Seeds N. W. (2001) Plasminogen expression in the neonatal and adult mouse brain. J. Neurochem. 77: 318-325
52. Buisson A., Nicole O., Docagne F., Sartelet H., Mackenzie E. T. and Vivien D. (1998) Up-regulation of a serine protease inhibitor in astrocytes mediates the neuroprotective activity of transforming growth factor betal. FASEB J. 12: 1683-1691
53. Liu Y., Honda S., Kohsaka S. and Nakajima K. (2000) Plasminogen enhances the secretion of plasminogen activator inhibitor-1 from cultured rat astrocytes. Neurosci. Lett. 282: 137-140
54. Guenther J., Nick H. and Monard D. (1985) A glia-derived neurite-promoting factor with protease inhibitory activity. EMBO J. 4: 1963-1966
55. Gloor S., Odink K., Guenther J., Nick H. and Monard D. (1986) A glia-derived neurite promoting factor with protease inhibitory activity belongs to the protease nexins. Cell 47: 687-693
56. Reinhard E., Meier R., Halfter W., Rovelli G. and Monard D. (1988) Detection of glia-derived nexin in the olfactory system of the rat. Neuron 1: 387-394
57. Choi B. H., Suzuki M., Kim T., Wagner S. L. and Cunningham D. D. (1990) Protease nexin-1. Localization in the human brain suggests a protective role against extravasated serine proteases. Am. J. Pathol. 137: 741-747
58. Reinhard E., Suidan H. S., Pavlik A. and Monard D. (1994) Glia-derived nexin/protease nexin-1 is expressed by a subset of neurons in the rat brain. J. Neurosci. Res. 37: 256-270
59. Krystosek A. and Seeds N. W. (1981) Plasminogen activator secretion by granule neurons in cultures of developing cerebellum. Proc. Natl. Acad. Sci. USA 78: 7810-7814
60. Verrall S. and Seeds N. W. (1988) Tissue plasminogen activator binding to mouse cerebellar granule neurons. J. Neurosci. Res. 21: 420-425
61. Ware J. H., DiBenedetto A. J. and Pittman R. N. (1995) Localization of tissue plasminogen activator mRNA in the developing rat cerebellum and effects of inhibiting tissue plasminogen activator on granule cell migration. J. Neurobiol. 28: 9-22
62. Murtomaki S., Trenkner E., Wright J. M., Saksela O. and Liesi P. (1995) Increased proteolytic activity of the granule neurons may contribute to neuronal death in the weaver mouse cerebellum. Dev. Biol. 168: 635-648
63. Pittman R. N., Ivins J. K. and Buettner H. M. (1989) Neuronal plasminogen activators: cell surface binding sites and involvement in neurite outgrowth. J. Neurosci. 9: 4269-4286
64. McGuire P. G. and Seeds N. W. (1990) Degradation of underlying extracellular matrix by sensory neurons during neurite outgrowth. Neuron 4: 633-642
65. Pittman R. N. and DiBenedetto A. J. (1995) PC12 cells overexpressing tissue plasminogen activator regenerate neurites to a greater extent and migrate faster than control cells in complex extracellular matrix. J. Neurochem. 64: 566-575
66. Herbert C. B., Bittner G. D. and Hubbell J. A. (1996) Effects of fibinolysis on neurite growth from dorsal root ganglia cultured in two- and three-dimensional fibrin gels. J. Comp. Neurol. 365: 380-391
67. Hantai D., Rao J. S., Kahler C. and Festoff B. W. (1989) Decrease in plasminogen activator correlates with synapse elimination during neonatal development of mouse skeletal muscle. Proc. Natl. Acad. Sci. USA 86: 362-366
68. Qian Z., Gilbert M. E., Colicos M. A., Kandel E. R. and Kuhl D. (1993) Tissue-plasminogen activator is induced as an immediate-early gene during seizure, kindling and long-term potentiation. Nature 361: 453-457
69. Rogove A. D., Siao C., Keyt B., Strickland S. and Tsirka S. E. (1999) Activation of microglia reveals a non-proteolytic cytokine function for tissue plasminogen activator in the central nervous system. J Cell Sci. 112 (Pt 22): 4007-4016
70. Cuzner M. L. and Opdenakker G. (1999) Plasminogen activators and matrix metalloproteases, mediators of extracellular proteolysis in inflammatory demyelination of the central nervous system. J. Neuroimmunol. 94: 1-14
71. Akenami F. O., Koskiniemi M., Farkkila M. and Vaheri A. (1997) Cerebrospinal fluid plasminogen activator inhibitor-1 in patients with neurological disease. J. Clin. Pathol. 50: 157-160
72. Turgeon V. L. and Houenou L. J. (1997) The role of thrombin-like (serine) proteases in the development, plasticity and pathology of the nervous system. Brain Res. Brain Res. Rev. 25: 85-95
73. Turgeon V. L., Salman N. and Houenou L. J. (2000) Thrombin: a neuronal cell modulator. Thromb. Res. 99: 417-427
74. Dihanich M., Kaser M., Reinhard E., Cunningham D. and Monard D. (1991) Prothrombin mRNA is expressed by cells of the nervous system. Neuron 6: 575-581
75. Deschepper C. F., Bigornia V., Berens M. E. and Lapointe M. C. (1991) Production of thrombin and antithrombin III by brain and astroglial cell cultures. Brain Res. Mol. Brain Res. 11: 355-358
76. Rohatgi T., Sedehizade F., Reymarm K. G. and Reiser G. (2004) Protease-activated receptors in neuronal development, neurodegeneration and neuroprotection: thrombin as signaling molecule in the brain. Neuroscientist 10: 501-512
77. Festoff B. W., Rao J. S. and Chen M. (1992) Protease nexin I, thrombin- and urokinase-inhibiting serpin, concentrated in normal human cerebrospinal fluid. Neurology 42: 1361-1366
78. Smirnova I. V., Salazar A., Arnold P. M., Glatt S., Handler M. and Festoff B. W. (1997) Thrombin and its precursor in human cerebrospinal fluid. Thromb. Haemost. 78: 1473-1479
79. Gurwitz D. and Cunningham D. D. (1988) Thrombin modulates and reverses neuroblastoma neurite outgrowth. Proc. Natl. Acad. Sci. USA 85: 3440-3444
80. Gurwitz D. and Cunningham D. D. (1990) Neurite outgrowth activity of protease nexin-1 on neuroblastoma cells requires thrombin inhibition. J. Cell Physiol. 142: 155-162
81. Suidan H. S., Stone S. R., Hemmings B. A. and Monard D. (1992) Thrombin causes neurite retraction in neuronal cells through activation of cell surface receptors. Neuron 8: 363-375
82. Turgeon V. L., Milligan C. E. and Houenou L. J. (1999) Activation of the protease-activated thrombin receptor (PAR)-1 induces motoncuron degeneration in the developing avian embryo. J. Neuropathol. Exp. Neurol. 58: 499-504
83. Festoff B. W., Suo Z. and Citron B. A. (2001) Plasticity and stabilization of neuromuscular and CNS synapses: interactions between thrombin protease signaling pathways and tissue transglutaminase. Int. Rev. Cytol. 211: 153-177
84. Cavanaugh K. P., Gurwitz D., Cunningham D. D. and Bradshaw R. A. (1990) Reciprocal modulation of astrocyte stellation by thrombin and protease nexin-1. J. Neurochem. 54: 1735-1743
85. Pindon A., Festoff B. W. and Hantai D. (1998) Thrombin-induced reversal of astrocyte stellation is mediated by activation of protein kinase C beta-1. Eur. J. Biochem. 255: 766-774
86. Perraud F., Besnard F., Sensenbrenner M. and Labourdette G. (1987) Thrombin is a potent mitogen for rat astroblasts but not for oligodendroblasts and neuroblasts in primary culture. Int. J. Dev. Neurosci. 5: 181-188
87. Smirnova I. V., Ma J. Y., Citron B. A., Ratzlaff K. T., Gregory E. J., Akaaboune M. et al. (1996) Neural thrombin and protease nexin I kinetics after murine peripheral nerve injury. J. Neurochem. 67: 2188-2199
88. Festoff B. W., Nelson P. G. and Brenneman D. E. (1996) Prevention of activity-dependent neuronal death: vasoactive intestinal polypeptide stimulates astrocytes to secrete the thrombin-inhibiting neurotrophic serpin, protease nexin I. J. Neurobiol. 30: 255-266
89. Smirnova I. V., Zhang S. X., Citron B. A., Arnold P. M. and Festoff B. W. (1998) Thrombin is an extracellular signal that activates intracellular death protease pathways inducing apoptosis in model motor neurons. J. Neurobiol. 36: 64-80
90. Turgeon V. L., Lloyd E. D., Wang S., Festoff B. W. and Houenou L. J. (1998) Thrombin perturbs neurite outgrowth and induces apoptotic cell death in enriched chick spinal motoneuron cultures through caspase activation. J. Neurosci. 18: 6882-6891
91. Wagner S. L., Geddes J. W., Cotman C. W., Lau A. L., Gurwitz D., Isackson P. J. et al. (1989) Protease nexin-1, an antithrombin with neurite outgrowth activity, is reduced in Alzheimer disease. Proc. Natl. Acad. Sci. USA 86: 8284-8288
92. Akiyama H., Ikeda K., Kondo H. and McGeer P. L. (1992) Thrombin accumulation in brains of patients with Alzheimer's disease. Neurosci. Lett. 146: 152-154
93. Pike C. J., Vaughan P. J., Cunningham D. D. and Cotman C. W. (1996) Thrombin attenuates neuronal cell death and modulates astrocyte reactivity induced by beta-amyloid in vitro. J. Neurochem. 66: 1374-1382
94. Smith-Swintosky V. L., Zimmer S., Fenton J. W. 2nd and Mattson M. P. (1995) Opposing actions of thrombin and protease nexin-1 on amyloid beta-peptide toxicity and on accumulation of peroxides and calcium in hippocampal neurons. J. Neurochem. 65: 1415-1418
95. Justice D. L., Rhodes R. H. and Tokes Z. A. (1987) Immunohistochemical demonstration of proteinase inhibitor alpha-1-antichymotrypsin in normal human central nervous system. J. Cell. Biochem. 34: 227-238
96. Kordula T., Rydel R. E., Brigham E. F., Horn F., Heinrich P. C. and Travis J. (1998) Oncostatin M and the interleukin-6 and soluble interleukin-6 receptor complex regulate alpha1-antichymotrypsin expression in human cortical astrocytes. J. Biol. Chem. 273: 4112-4118
97. Lieb K., Fiebich B. L., Schaller H., Berger M. and Bauer J. (1996) Interleukin-1 beta and tumor necrosis factor-alpha induce expression of alpha 1-antichymotrypsin in human astrocytoma cells by activation of nuclear factor-kappa B. J. Neurochem. 67: 2039-2044
98. Abraham C. R., Selkoe D. J. and Potter H. (1988) Immunochemical identification of the serine protease inhibitor alpha 1-antichymotrypsin in the brain amyloid deposits of Alzheimer's disease. Cell 52: 487-501
99. Abraham C. R. and Potter H. (1989b) The protease inhibitor, alpha 1-antichymotrypsin, is a component of the brain amyloid deposits in normal aging and Alzheimer's disease. Ann. Med. 21: 77-81
100. Crowther D. C., Kinghorn K. J., Page R. and Lomas D. A. (2004) Therapeutic targets from a Drosophila model of Alzheimer's disease. Curr. Opin. Pharmacol. 4: 513-516
101. Licastro F., Davis L. J., Morini M. C., Cucinotta D. and Savorani G. (1994) Cerebrospinal fluid of patients with senile dementia of Alzheimer's type shows an increased inhibition of alpha-chymotrypsin. Alzheimer Dis. Assoc. Disord. 8: 241-249
102. Ma J., Yee A., Brewer H. B. Jr, Das S. and Potter H. (1994) Amyloid-associated proteins alpha 1-antichymotrypsin and apolipoprotein E promote assembly of Alzheimer beta-protein into filaments. Nature 372: 92-94
103. Abraham C. R., McGraw W. T., Slot F. and Yamin R. (2000) Alpha 1-antichymotrypsin inhibits A beta degradation in vitro and in vivo. Ann. N. Y. Acad. Sci. 920: 245-248
104. Mucke L., Yu G. Q., McConlogue L., Rockenstein E. M., Abraham C. R. and Masliah E. (2000) Astroglial expression of human alpha(1)-antichyinotrypsin enhances alzheimer-like pathology in amyloid protein precursor transgenic mice. Am. J. Pathol. 157: 2003-2010
105. Nilsson L. N., Bales K. R., DiCarlo G., Gordon M. N., Morgan D., Paul S. M. et al. (2001) Alpha-1-antichymotrypsin promotes beta-sheet amyloid plaque deposition in a transgenic mouse model of Alzheimer's disease. J. Neurosci. 21: 1444-1451
106. Lomas D. A., Stone S. R., Llewelyn-Jones C., Keogan M.-T., Wang Z. M., Rubin H. et al. (1995) The control of neutrophil chemotaxis by inhibitors of cathepsin G and chymotrypsin. J. Biol. Chem. 270: 23437-23443
107. Abraham C. R., Kanemaru K. and Mucke L. (1993) Expression of cathepsin G-like and alpha 1-antichymotrypsin-like proteins in reactive astrocytes. Brain Res. 621: 222-232
108. Nelson R. B. and Siman R. (1990) Clipsin, a chymotrypsin-like protease in rat brain which is irreversibly inhibited by alpha-1-antichymotrypsin. J. Biol. Chem. 265: 3836-3843
109. Klegeris A. and McGeer P. L. (2005) Chymotrypsin-like proteases contribute to human monocytic THP-1 cell as well as human microglial neurotoxicity. Glia 51: 56-64
110. Walker D. G., Yasuhara O., Patston P. A., McGeer E. G. and McGeer P. L. (1995) Complement C1 inhibitor is produced by brain tissue and is cleaved in Alzheimer disease. Brain Res. 675: 75-82
111. Thomas A., Gasque P., Vaudry D., Gonzalez B. and Fontaine M. (2000) Expression of a complete and functional complement system by human neuronal cells in vitro. Int. Immunol. 12: 1015-1023
112. Yousef G. M., Kishi T. and Diamandis E. P. (2003) Role of kallikrein enzymes in the central nervous system. Clin. Chim. Acta. 329: 1-8
113. Chen Z. L., Yoshida S., Kato K., Momota Y., Suzuki J., Tanaka T. et al. (1995) Expression and activity-dependent changes of a novel limbic-serine protease gene in the hippocampus. J. Neurosci. 15: 5088-5097
114. Suzuki J., Yoshida S., Chen Z. L., Momota Y., Kato K., Hirata A. et al. (1995) Ontogeny of neuropsin mRNA expression in the mouse brain. Neurosci. Res. 23: 345-351
115. Yoshida S., Taniguchi M., Hirata A. and Shiosaka S. (1998) Sequence analysis and expression of human neuropsin cDNA and gene. Gene 213: 9-16
116. Mitsui S., Tsuruoka N., Yamashiro K., Nakazato H. and Yamaguchi N. (1999) A novel form of human neuropsin, a brain-related serine protease, is generated by alternative splicing and is expressed preferentially in human adult brain. Eur. J. Biochem. 260: 627-634
117. Shimizu C., Yoshida S., Shibata M., Kato K., Momota Y., Matsumoto K. et al. (1998) Characterization of recombinant and brain neuropsin, a plasticity-related serine protease. J. Biol. Chem. 273: 11189-11196
118. Matsumoto-Miyai K., Ninomiya A., Yamasaki H., Tamura H., Nakamura Y. and Shiosaka S. (2003) NMDA-dependent proteolysis of presynaptic adhesion molecule L1 in the hippocampus by neuropsin. J. Neurosci. 23: 7727-7736
119. Komai S., Matsuyama T., Matsumoto K., Kato K., Kobayashi M., Imamura K. et al. (2000) Neuropsin regulates an early phase of schaffer-collateral long-term potentiation in the murine hippocampus. Eur. J. Neurosci. 12: 1479-1486
120. Hirata A., Yoshida S., Inoue N., Matsumoto-Miyai K., Ninomiya A., Taniguchi M. et al. (2001) Abnormalities of synapses and neurons in the hippocampus of neuropsin-def icient mice. Mol. Cell. Neurosci. 17: 600-610
121. Oka T., Akisada M., Okabe A., Sakurai K., Shiosaka S. and Kato K. (2002) Extracellular serine protease neuropsin (KLK8) modulates neurite outgrowth and fasciculation of mouse hippocampal neurons in culture. Neurosci. Lett. 321: 141-144
122. Okabe A., Momota Y., Yoshida S., Hirata A., Ito J., Nishino H. et al. (1996) Kindling induces neuropsin mRNA in the mouse brain. Brain Res. 728: 116-120
123. Momota Y., Yoshida S., Ito J., Shibata M., Kato K., Sakurai K. et al. (1998) Blockade of neuropsin, a serine protease, ameliorates kindling epilepsy. Eur. J. Neurosci. 10: 760-764
124. Davies B., Kearns I. R., Ure J., Davies C. H. and Lathe R. (2001) Loss of hippocampal serine protease BSP1/neuropsin predisposes to global seizure activity. J. Neurosci. 21: 6993-7000
125. He X. P., Shiosaka S. and Yoshida S. (2001) Expression of neuropsin in oligodendrocytes after injury to the CNS. Neurosci. Res. 39: 455-462
126. Terayama R., Bando Y., Takahashi T. and Yoshida S. (2004) Differential expression of neuropsin and protease M/neurosin in oligodendrocytes after injury to the spinal cord. Glia 48: 91-101
127. Shimizu-Okabe C., Yousef G. M., Diamandis E. P., Yoshida S., Shiosaka S. and Fahnestock M. (2001) Expression of the kallikrein gene family in normal and Alzheimer's disease brain. Neuroreport 12: 2747-2751
128. Kato K., Kishi T., Kamachi T., Akisada M., Oka T., Midorikawa R. et al. (2001) Serine proteinase inhibitor 3 and murinoglobulin I are potent inhibitors of neuropsin in adult mouse brain. J. Biol. Chem. 276: 14562-14571
129. Gschwend T. P., Krueger S. R., Kozlov S. V., Wolfer D. P. and Sonderegger P. (1997) Neurotrypsin, a novel multidomain serine protease expressed in the nervous system. Mol. Cell. Neurosci. 9: 207-219
130. Proba K., Gschwend T. P. and Sonderegger P. (1998) Cloning and sequencing of the cDNA encoding human neurotrypsin. Biochim. Biophys. Acta 1396: 143-147
131. Wolfer D. P., Lang R., Cinelli P., Madani R. and Sonderegger P. (2001) Multiple roles of neurotrypsin in tissue morphogenesis and nervous system development suggested by the mRNA expression pattern. Mol. Cell. Neurosci. 18: 407-433
132. Molinari F., Rio M., Meskenaite V., Encha-Razavi F., Auge J., Bacq D. et al. (2002) Truncating neurotrypsin mutation in autosomal recessive nonsyndromic mental retardation. Science 298: 1779-1781
133. Hengst U., Albrecht H., Hess D. and Monard D. (2001) The phosphatidylethanolamine-binding protein is the prototype of a novel family of serine protease inhibitors. J. Biol. Chem. 276: 535-540
134. Ohshima S., Murata M., Sakamoto W., Ogura Y. and Motoyoshi F. (1997) Cloning and molecular analysis of the Arabidopsis gene Terminal Flower 1. Mol. Gen. Genet. 254: 186-194
135. Robinson L. C. and Tatchell K. (1991) TFS1: a suppressor of cdc25 mutations in Saccharomyces cerevisiae. Mol. Gen. Genet. 230: 241-250
136. Seddiqi N., Bollengier F., Alliel P. M., Perin J. P., Bonnet F., Bucquoy S. et al. (1994) Amino acid sequence of the Homo sapiens brain 21-23- kDa protein (neuropolypeptide h3), comparison with its counterparts from Rattus norvegicus and Bos taurus species and expression of its mRNA in different tissues. J. Mol. Evol. 39: 655-660
137. Frayne J., Ingram C., Love S. and Hall L. (1999) Localisation of phosphatidylethanolamine-binding protein in the brain and other tissues of the rat. Cell Tissue Res. 298: 415-423
138. Ojika K., Mitake S., Tohdoh N., Appel S. H., Otsuka Y., Katada E. et al. (2000) Hippocampal cholinergic neurostimulating peptides (HCNP). Prog. Neurobiol. 60: 37-83
139. Yeung K., Seitz T., Li S., Janosch P., McFerran B., Kaiser C. et al. (1999) Suppression of Raf-1 kinase activity and MAP kinase signalling by RKIP. Nature 401: 173-177
140. 1- antichymotrypsin indicates two stage insertion of the reactive loop; implications for inhibitory function and conformational disease. Proc. Natl. Acad. Sci. USA 97: 67-72
141. 1- antitrypsin related cirrhosis. J. Biol. Chem. 277: 6771-6774
142. 1-antitrypsin accumulation in the liver. Nature 357: 605-607
143. 1- antitrypsin. J. Biol. Chem. 274: 9548-9555
144. 1-antitrypsin polymers are formed by reactive loop-β-sheet A linkage. J. Biol. Chem. 275: 33663-33668
145. Lomas D. A., Belorgey D., Mallya M., Onda M., Kinghorn K. J., Sharp L. K. et al. (2004) Polymerisation underlies alpha1-antitrypsin deficiency, dementia and other serpinopathies. Front Biosci. 9: 2873-2891
146. Crowther D. C., Belorgey D., Miranda E., Kinghorn K. J., Sharp L. K. and Lomas D. A. (2004) Practical genetics: alpha1-antitrypsin deficiency and the serpinopathies. Eur. J. Hum. Genet. 12: 167-172
147. 1-antitrypsin deficiency: a model for conformational diseases. New. Engl. J. Med. 346: 45-53
148. Davis R. L., Shrimpton A. E., Holohan P. D., Bradshaw C., Feiglin D., Sonderegger P. et al. (1999) Familial dementia caused by polymerisation of mutant neuroserpin. Nature 401: 376-379
149. Davis R. L., Holohan P. D., Shrimpton A. E., Tatum A., Daucher J., Collins G. H. et al. (1999) Familial encephalopathy with neuroserpin inclusion bodies (FENIB). Am. J. Pathol. 155: 1901-1913
150. Takao M., Murrell J. R., Benson M. D., Yazaki M., Piccardo P., Davis R. et al. (2000) Neuroserpin S52R mutation causes progressive myoclonus epilepsy and a wide distribution of intra-neuronal neuroserpin deposits in the CNS. Brain Pathol. 4: 597
151. Davis R. L., Shrimpton A. E., Carrell R. W., Lomas D. A., Gerhard L., Baumann B. et al. (2002) Association between conformational mutations in neuroserpin and onset and severity of dementia. Lancet 359: 2242-2247
152. iiyama (serine 53 (TCC) to phenylalanine 53 (TTC)). A new α1-antitrypsin-deficient variant with mutation on a predicted conserved residue of the serpin backbone. J. Biol. Chem. 266: 12627-12632
153. 53ØPhe) ; further evidence for intracellular loop-sheet polymerisation. J. Biol. Chem. 268: 15333-15335
154. Belorgey D., Sharp L. K., Crowther D. C., Onda M., Johansson J. and Lomas D. A. (2004) Neuroserpin Portland (Ser52Arg) is trapped as an inactive intermediate that rapidly forms polymers: implications for the epilepsy seen in the dementia FENIB. Eur. J. Biochem. 271: 3360-3367
155. Onda M., Belorgey D., Sharp L. K. and Lomas D. A. (2005) Latent Ser49Pro neuroserpin forms polymers in the dementia familial encephalopathy with neuroserpin inclusion bodies. J. Biol. Chem. I280: 13735-13741
156. Miranda E., Romisch K. and Lomas D. A. (2004) Mutants of neuroserpin that cause dementia accumulate as polymers within the endoplasmic reticulum. J. Biol. Chem. 279: 28283-28291
157. 1-antitrypsin variant. Biochemical mapping of the degradative event among compartments in the secretory pathway. J. Biol. Chem. 265: 14001-14007
158. Tsirka S. E., Gualandris A., Amaral D. G. and Strickland S. (1995) Excitotoxin-induced neuronal degeneration and seizure are mediated by tissue plasminogen activator. Nature 377: 340-344
159. Tsirka S. E., Rogove A. D. and Strickland S. (1996) Neuronal cell death and tPA. Nature 384: 123-124
160. Schroder M. and Kaufman R. J. (2005) ER stress and the unfolded protein response. Mutat. Res. 569: 29-63
161. 1-antitrypsin within the hepatic endoplasmic reticulum does not elevate the steady-state level of grp78/BiP. J. Biol. Chem. 265: 20463-20468
162. Lawless M. W., Greene C. M., Mulgrew A., Taggart C. C., O'Neill S. J. and McElvaney N. G. (2004) Activation of endoplasmic reticulum-specific stress responses associated with the conformational disease Z alpha 1-antitrypsin deficiency. J. Immunol. 172: 5722-5726
163. Hidvegi T., Schmidt B. Z., Hale P. and Perlmutter D. H. (2005) Accumulation of mutant alpha 1 antitrypsin Z in the ER activates caspases-4 and -12, NFkappa B and BAP31 but not the unfolded protein response. J. Biol. Chem. 280: 39002-39015
164. Pahl H. L. (1999) Signal transduction from the endoplasmic reticulum to the cell nucleus. Physiol. Rev. 79: 683-701
165. 1-antitrypsin. Nat. Struct. Biol. 3: 676-681
166. Lomas D. A. and Carrell R. W. (2002) Serpinopathies and the conformational dementias. Nat. Rev. Genet. 3: 759-768
167. 1- antitrypsin. J. Biol. Chem. 270: 5282-5288
168. Im H., Woo M.-S., Hwang K. Y. and Yu M.-H. (2002) Interactions causing the kinetic trap in serpin protein folding. J. Biol. Chem. 277: 46347-46354