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Gene Review

Pcolce  -  procollagen C-endopeptidase enhancer protein

Mus musculus

Synonyms: Astt-2, Astt2, P14, PCPE-1, Pcpe1, ...
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Disease relevance of Pcolce


Psychiatry related information on Pcolce

  • We investigated the development of spontaneous miniature excitatory postsynaptic currents (mEPSCs) in AVCN neurons using whole cell patch-clamp techniques during [postnatal day 7 (P7)] and after (P14, P21) this critical period [5].

High impact information on Pcolce


Biological context of Pcolce

  • A significant upregulation of Egr1 at P11 and several microglia/glia-related transcripts starting at P11 with a peak at P14 were identified in the diseased retina [10].
  • RESULTS: VEGF transgene mRNA was first detected in the retina by RT-PCR on postnatal day 6 (P6) and increased over the next several days to reach a constant steady-state level between P14 and P21 [11].
  • Initially, low cell numbers rise to a density of 0.1 2A4(+)ORNs/mm OE length by P14, plateau at 0.9 2A4(+)ORNs/mm by P49, then fall to adult values of 0.4 cells/mm [12].
  • In developing mice, down-regulation of nestin expression was observed between P7 and P14 [13].
  • Hepatic enlargement was observed as early as gestational day 12.5 (G12.5) and thereafter became more prominent, whereas expression of CSE in the livers was found after postpartum day 1 (P1) and reached a peak at P14 [14].

Anatomical context of Pcolce

  • They showed reduced neovascularization in the liver sinusoids and kidney outer medulla vasa recta at P7, which most likely caused the ischemic necrosis observed by P14 in hepatocytes and renal tubular epithelia [15].
  • Abnormal cells were seen in the outer nuclear layer on P10 and among photoreceptors on P14; by P18 there were cell aggregates in the subretinal space with evidence of lumen formation [11].
  • However, upon infection with LCMV, reactive cytotoxic T lymphocytes (CTL) from P14 beta-transgenic mice were predominantly V alpha 2+ whereas CTL from P14 alpha-transgenic mice preferentially expressed V beta 8.1 and unexpectedly also V beta 8.3 (but not V beta 8.2) [16].
  • Whole mounts of retinas perfused with fluorescein-labeled dextran showed a similar sequence of events, with sprouts from retinal vessels in the deep capillary bed seen on P14 and vessels reaching the subretinal space by P18 [11].
  • Seven days after cochlea ablation at P7 or P14, mEPSCs in surviving bushy cells were similar to controls, except that rise and decay times were positively correlated (R = 0.31 and 0.14 for surgery at P7 and P14, respectively) [5].

Associations of Pcolce with chemical compounds


Regulatory relationships of Pcolce

  • Here we show that the 36 kDa, active fragment of PCPE enhances the activity of both the short (mouse) and long (chick) forms of PCP/BMP-1 [20].

Other interactions of Pcolce

  • Finally, when the amount of PCP is adjusted so that the rate of C-terminal processing remains constant, PCPE (36 kDa) has no effect on the assembly of collagen or pN-collagen in vitro following C-terminal processing of the corresponding precursors [20].
  • In addition, PCPE (36 kDa) has no effect in vitro on N-terminal procollagen processing by highly purified procollagen N-proteinase [20].

Analytical, diagnostic and therapeutic context of Pcolce

  • By contrast, Northern and Western blotting studies revealed that procollagen C-proteinases bone morphogenic protein-1 and mammalians Tolloid and procollagen C-proteinase enhancer were expressed in MC3T3-E1 cells and not down-regulated [9].
  • Fetal thymic organ culture of P14/CNAalpha(-/-) lobes showed no defect in positive or negative selection of thymocytes [21].
  • Sequence analysis of a large panel of LCMV-reactive "half-transgenic" TcR from P14 single receptor chain-transgenic mice revealed a highly conserved VJ alpha and a more diverse VDJ beta junctional region [16].
  • MNTB immunohistochemistry demonstrates that Nav1.1 subunits are expressed postsynaptically in both P14 normal and deaf mice, while postsynaptic Nav1.6 staining was only observed in deaf mice [22].
  • Western blot analysis demonstrated that, in the control group, the embryonic form of N-CAM (E-N-CAM) was expressed in the 7-day-old mouse (P7) cerebellum, but not in the P14 cerebellum which exhibited only the adult form of N-CAM (A-N-CAM) [23].


  1. Identification and expression of a novel type I procollagen C-proteinase enhancer protein gene from the glaucoma candidate region on 3q21-q24. Xu, H., Acott, T.S., Wirtz, M.K. Genomics (2000) [Pubmed]
  2. Negative regulation of T cell proliferation and interleukin 2 production by the serine threonine kinase GSK-3. Ohteki, T., Parsons, M., Zakarian, A., Jones, R.G., Nguyen, L.T., Woodgett, J.R., Ohashi, P.S. J. Exp. Med. (2000) [Pubmed]
  3. Cutting edge: CCR7+ and CCR7- memory T cells do not differ in immediate effector cell function. Unsoeld, H., Krautwald, S., Voehringer, D., Kunzendorf, U., Pircher, H. J. Immunol. (2002) [Pubmed]
  4. Tyrosinase inhibitory effects and inhibition mechanisms of nobiletin and hesperidin from citrus peel crude extracts. Zhang, C., Lu, Y., Tao, L., Tao, X., Su, X., Wei, D. J Enzyme Inhib Med Chem (2007) [Pubmed]
  5. Development of Spontaneous Miniature EPSCs in Mouse AVCN Neurons During a Critical Period of Afferent-Dependent Neuron Survival. Lu, Y., Harris, J.A., Rubel, E.W. J. Neurophysiol. (2007) [Pubmed]
  6. Crippling of CD3-zeta ITAMs does not impair T cell receptor signaling. Ardouin, L., Boyer, C., Gillet, A., Trucy, J., Bernard, A.M., Nunes, J., Delon, J., Trautmann, A., He, H.T., Malissen, B., Malissen, M. Immunity (1999) [Pubmed]
  7. Normal timing of oligodendrocyte development depends on thyroid hormone receptor alpha 1 (TRalpha1). Billon, N., Jolicoeur, C., Tokumoto, Y., Vennström, B., Raff, M. EMBO J. (2002) [Pubmed]
  8. Procollagen C proteinase enhancer 1 genes are important determinants of the mechanical properties and geometry of bone and the ultrastructure of connective tissues. Steiglitz, B.M., Kreider, J.M., Frankenburg, E.P., Pappano, W.N., Hoffman, G.G., Meganck, J.A., Liang, X., Höök, M., Birk, D.E., Goldstein, S.A., Greenspan, D.S. Mol. Cell. Biol. (2006) [Pubmed]
  9. Regulation of collagen deposition and lysyl oxidase by tumor necrosis factor-alpha in osteoblasts. Pischon, N., Darbois, L.M., Palamakumbura, A.H., Kessler, E., Trackman, P.C. J. Biol. Chem. (2004) [Pubmed]
  10. Genome-wide expression profiling of the retinoschisin-deficient retina in early postnatal mouse development. Gehrig, A., Langmann, T., Horling, F., Janssen, A., Bonin, M., Walter, M., Poths, S., Weber, B.H. Invest. Ophthalmol. Vis. Sci. (2007) [Pubmed]
  11. Evolution of neovascularization in mice with overexpression of vascular endothelial growth factor in photoreceptors. Tobe, T., Okamoto, N., Vinores, M.A., Derevjanik, N.L., Vinores, S.A., Zack, D.J., Campochiaro, P.A. Invest. Ophthalmol. Vis. Sci. (1998) [Pubmed]
  12. Development and further characterization of a small subclass of rat olfactory receptor neurons that shows immunoreactivity for the HSP70 heat shock protein. Carr, V.M., Morimoto, R.I., Farbman, A.I. J. Comp. Neurol. (1999) [Pubmed]
  13. Nestin-containing cells express glial fibrillary acidic protein in the proliferative regions of central nervous system of postnatal developing and adult mice. Wei, L.C., Shi, M., Chen, L.W., Cao, R., Zhang, P., Chan, Y.S. Brain Res. Dev. Brain Res. (2002) [Pubmed]
  14. Differential induction of cystathionine gamma-lyase in the livers and kidneys of mouse dams during gestation and lactation. Akahoshi, N., Izumi, T., Ishizaki, Y., Ishii, I. Biol. Pharm. Bull. (2006) [Pubmed]
  15. Redundant roles of Sox17 and Sox18 in postnatal angiogenesis in mice. Matsui, T., Kanai-Azuma, M., Hara, K., Matoba, S., Hiramatsu, R., Kawakami, H., Kurohmaru, M., Koopman, P., Kanai, Y. J. Cell. Sci. (2006) [Pubmed]
  16. Involvement of both T cell receptor V alpha and V beta variable region domains and alpha chain junctional region in viral antigen recognition. Brändle, D., Bürki, K., Wallace, V.A., Rohrer, U.H., Mak, T.W., Malissen, B., Hengartner, H., Pircher, H. Eur. J. Immunol. (1991) [Pubmed]
  17. Procollagen type I C-proteinase enhancer is a naturally occurring connective tissue glycoprotein. Kessler, E., Mould, A.P., Hulmes, D.J. Biochem. Biophys. Res. Commun. (1990) [Pubmed]
  18. Comparative effects of early postnatal ibuprofen and indomethacin on VEGF, IGF-I, and GH during rat ocular development. Beharry, K.D., Modanlou, H.D., Hasan, J., Gharraee, Z., Abad-Santos, P., Sills, J.H., Jan, A., Nageotte, S., Aranda, J.V. Invest. Ophthalmol. Vis. Sci. (2006) [Pubmed]
  19. Tyrosinase inhibitory effects and inhibition mechanisms of nobiletin and hesperidin from citrus peel crude extracts. Zhang, C., Lu, Y., Tao, L., Tao, X., Su, X., Wei, D. J Enzyme Inhib Med Chem (2007) [Pubmed]
  20. The CUB domains of procollagen C-proteinase enhancer control collagen assembly solely by their effect on procollagen C-proteinase/bone morphogenetic protein-1. Hulmes, D.J., Mould, A.P., Kessler, E. Matrix Biol. (1997) [Pubmed]
  21. Calcineurin Aalpha plays an exclusive role in TCR signaling in mature but not in immature T cells. Chan, V.S., Wong, C., Ohashi, P.S. Eur. J. Immunol. (2002) [Pubmed]
  22. Altered sodium currents in auditory neurons of congenitally deaf mice. Leão, R.N., Naves, M.M., Leão, K.E., Walmsley, B. Eur. J. Neurosci. (2006) [Pubmed]
  23. Abnormal expression of embryonic neural cell adhesion molecule (N-CAM) in the developing mouse cerebellum after neonatal administration of cytosine arabinoside. Ono, K., Tokunaga, A., Mizukawa, K., Kurose, K., Tanaka, H. Brain Res. Dev. Brain Res. (1992) [Pubmed]
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