Gene Symbol: Prss12
Description: protease, serine 12 neurotrypsin (motopsin)
Alias: Bssp-3, motopsin, neurotrypsin, brain-specific serine protease 3, serine protease 12
Species: mouse
Products:     Prss12

Top Publications

  1. Iijima N, Tanaka M, Mitsui S, Yamamura Y, Yamaguchi N, Ibata Y. Expression of a serine protease (motopsin PRSS12) mRNA in the mouse brain: in situ hybridization histochemical study. Brain Res Mol Brain Res. 1999;66:141-9 pubmed
    ..In an attempt to find novel brain-specific serine proteases (BSSPs), motopsin (PRSS-12) was cloned from a mouse brain cDNA library by polymerase chain reaction (PCR)...
  2. Hoerder Suabedissen A, Oeschger F, Krishnan M, Belgard T, Wang W, Lee S, et al. Expression profiling of mouse subplate reveals a dynamic gene network and disease association with autism and schizophrenia. Proc Natl Acad Sci U S A. 2013;110:3555-60 pubmed publisher
    ..Our report emphasizes the importance of the study of transient features of the developing brain to better understand neurodevelopmental disorders. ..
  3. Mitsui S, Yamaguchi N, Osako Y, Yuri K. Enzymatic properties and localization of motopsin (PRSS12), a protease whose absence causes mental retardation. Brain Res. 2007;1136:1-12 pubmed
    b>Motopsin (PRSS12) is a mosaic protease expressed in the central nervous system. Truncation of the human motopsin gene causes nonsyndromic mental retardation...
  4. Mitsui S, Osako Y, Yokoi F, Dang M, Yuri K, Li Y, et al. A mental retardation gene, motopsin/neurotrypsin/prss12, modulates hippocampal function and social interaction. Eur J Neurosci. 2009;30:2368-78 pubmed publisher
    b>Motopsin is a mosaic serine protease secreted from neuronal cells in various brain regions, including the hippocampus...
  5. Stephan A, Mateos J, Kozlov S, Cinelli P, Kistler A, Hettwer S, et al. Neurotrypsin cleaves agrin locally at the synapse. FASEB J. 2008;22:1861-73 pubmed publisher
    The synaptic serine protease neurotrypsin is considered to be essential for the establishment and maintenance of cognitive brain functions, because humans lacking functional neurotrypsin suffer from severe mental retardation...
  6. Matsumoto Miyai K, Sokolowska E, Zurlinden A, Gee C, Lüscher D, Hettwer S, et al. Coincident pre- and postsynaptic activation induces dendritic filopodia via neurotrypsin-dependent agrin cleavage. Cell. 2009;136:1161-71 pubmed publisher
    The synaptic serine protease neurotrypsin is essential for cognitive function, as its deficiency in humans results in severe mental retardation...
  7. Almenar Queralt A, Kim S, Benner C, Herrera C, Kang D, Garcia Bassets I, et al. Presenilins regulate neurotrypsin gene expression and neurotrypsin-dependent agrin cleavage via cyclic AMP response element-binding protein (CREB) modulation. J Biol Chem. 2013;288:35222-36 pubmed publisher
    ..Prss12 encodes the serine protease neurotrypsin, which cleaves the heparan sulfate proteoglycan agrin...
  8. Frischknecht R, Fejtova A, Viesti M, Stephan A, Sonderegger P. Activity-induced synaptic capture and exocytosis of the neuronal serine protease neurotrypsin. J Neurosci. 2008;28:1568-79 pubmed publisher
    ..The extracellular serine protease neurotrypsin was implicated in cognitive function, because humans lacking a functional form of neurotrypsin suffer from ..
  9. Gschwend T, Krueger S, Kozlov S, Wolfer D, Sonderegger P. Neurotrypsin, a novel multidomain serine protease expressed in the nervous system. Mol Cell Neurosci. 1997;9:207-19 pubmed
    We have cloned a novel murine cDNA encoding a multidomain serine protease, termed neurotrypsin, which exhibits an unprecedented domain composition...

More Information


  1. Szabo R, Hobson J, Christoph K, Kosa P, List K, Bugge T. Regulation of cell surface protease matriptase by HAI2 is essential for placental development, neural tube closure and embryonic survival in mice. Development. 2009;136:2653-63 pubmed publisher
    ..This study indicates that unregulated matriptase-dependent cell surface proteolysis can cause a diverse array of abnormalities in mammalian development. ..
  2. Reif R, Sales S, Hettwer S, Dreier B, Gisler C, Wölfel J, et al. Specific cleavage of agrin by neurotrypsin, a synaptic protease linked to mental retardation. FASEB J. 2007;21:3468-78 pubmed
    The synaptic serine protease neurotrypsin is thought to be important for adaptive synaptic processes required for cognitive functions, because humans deficient in neurotrypsin suffer from severe mental retardation...
  3. Bolliger M, Zurlinden A, Lüscher D, Bütikofer L, Shakhova O, Francolini M, et al. Specific proteolytic cleavage of agrin regulates maturation of the neuromuscular junction. J Cell Sci. 2010;123:3944-55 pubmed publisher
    ..Excessive cleavage of agrin via transgenic overexpression of an agrin-cleaving protease, neurotrypsin, in motoneurons resulted in excessive reorganizational activity of the NMJs, leading to rapid dispersal of the ..
  4. Bütikofer L, Zurlinden A, Bolliger M, Kunz B, Sonderegger P. Destabilization of the neuromuscular junction by proteolytic cleavage of agrin results in precocious sarcopenia. FASEB J. 2011;25:4378-93 pubmed publisher
    ..We recently found that overexpression of the neural serine protease neurotrypsin in motoneurons resulted in the degeneration of their neuromuscular junctions (NMJ) within days...
  5. Simon R, Lufkin T. Postnatal lethality in mice lacking the Sax2 homeobox gene homologous to Drosophila S59/slouch: evidence for positive and negative autoregulation. Mol Cell Biol. 2003;23:9046-60 pubmed
    ..Intriguingly, our studies also demonstrated a striking autoregulation of the Sax2 gene in both positive- and negative-feedback mechanisms depending on the specific cell type expressing Sax2. ..
  6. Gisler C, Lüscher D, Schätzle P, Dürr S, Baici A, Galliciotti G, et al. Zymogen activation of neurotrypsin and neurotrypsin-dependent agrin cleavage on the cell surface are enhanced by glycosaminoglycans. Biochem J. 2013;453:83-100 pubmed publisher
    The serine peptidase neurotrypsin is stored in presynaptic nerve endings and secreted in an inactive zymogenic form by synaptic activity...
  7. Mitsui S, Hidaka C, Furihata M, Osako Y, Yuri K. A mental retardation gene, motopsin/prss12, modulates cell morphology by interaction with seizure-related gene 6. Biochem Biophys Res Commun. 2013;436:638-44 pubmed publisher
    A serine protease, motopsin (prss12), plays a significant role in cognitive function and the development of the brain, since the loss of motopsin function causes severe mental retardation in humans and enhances social behavior in mice...
  8. Yamamura Y, Yamashiro K, Tsuruoka N, Nakazato H, Tsujimura A, Yamaguchi N. Molecular cloning of a novel brain-specific serine protease with a kringle-like structure and three scavenger receptor cysteine-rich motifs. Biochem Biophys Res Commun. 1997;239:386-92 pubmed
    ..Northern blot analysis demonstrated that mBSSP-3 mRNA was specifically expressed in the mouse brain, lung and kidney. We concluded that a novel brain serine protease, BSSP-3, is a new member of kringle and SRCR superfamilies. ..