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Gene Review

SERPINB1  -  serpin peptidase inhibitor, clade B...

Homo sapiens

Synonyms: EI, ELANH2, HEL57, LEI, Leukocyte elastase inhibitor, ...
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Disease relevance of SERPINB1


Psychiatry related information on SERPINB1

  • The LEI group showed significantly slower one- and two-finger reaction time, had an impaired ability to balance, and were compromised in their ability to maintain body temperature, when compared with the HEI group [6].

High impact information on SERPINB1


Chemical compound and disease context of SERPINB1

  • HP1 and MNEI levels inversely correlate in a number of normal and leukemia myeloid cells and show strikingly opposite coordinated changes during differentiation of U937 cell line induced by retinoic acid [11].
  • These data indicate that alanyl alanyl prolyl alanine chloromethylketone in the doses administered (1) had significant anti-elastase activity in vivo; (2) markedly decreased the extent of elastase-induced emphysema; and (3) produced no adverse toxic effect during the period covered by this study protocol [12].

Biological context of SERPINB1


Anatomical context of SERPINB1


Associations of SERPINB1 with chemical compounds

  • Sequence alignment indicates that the reactive center P1 residue is Cys-344, consistent with abrogation of elastase inhibitory activity by iodoacetamide and making EI a naturally occurring Cys-serpin [10].
  • The serpin MNEI inhibits elastase-like and chymotrypsin-like serine proteases through efficient reactions at two active sites [16].
  • One was methionine 358, whose oxidation was known to cause loss of anti-elastase activity [21].
  • Thus, heparin increases both the rate of inhibition by promoting the formation of a high affinity EI* intermediate and the rate of EI dissociation [22].
  • Using synthetic peptides corresponding to full-length elafin (H2N-1AVT.....95Q-OH), the NH2-terminal domain (H2N-1AVT.....50K-OH) and the COOH-terminal domain (H2N-51PGS.....95Q-OH), we demonstrate that elafin's anti-elastase activity resides exclusively in the COOH-terminus [23].

Other interactions of SERPINB1


Analytical, diagnostic and therapeutic context of SERPINB1


  1. Human monocyte/neutrophil elastase inhibitor (MNEI) is regulated by PU.1/Spi-1, Sp1, and NF-kappaB. Zeng, W., Remold-O'Donnell, E. J. Cell. Biochem. (2000) [Pubmed]
  2. Effect of a synthetic leukocyte elastase inhibitor on thrombin-induced pulmonary edema in the rat. Ahn, C.M., Sandler, H., Glass, M., Saldeen, T. Exp. Lung Res. (1993) [Pubmed]
  3. Aerosol treatment with MNEI suppresses bacterial proliferation in a model of chronic Pseudomonas aeruginosa lung infection. Woods, D.E., Cantin, A., Cooley, J., Kenney, D.M., Remold-O'Donnell, E. Pediatr. Pulmonol. (2005) [Pubmed]
  4. Decreased specific anti-elastase activity in the uninvolved skin of patients with psoriasis. Glinski, W., Pierozynska-Dubowska, M., Glinska-Ferenz, M., Jablońska, S. Arch. Dermatol. Res. (1991) [Pubmed]
  5. Comparison of the predictive power of different prognostic indices for overall survival in patients with advanced ovarian carcinoma. Lund, B., Williamson, P., van Houwelingen, H.C., Neijt, J.P. Cancer Res. (1990) [Pubmed]
  6. Physiological, metabolic, and performance implications of a prolonged hill walk: influence of energy intake. Ainslie, P.N., Campbell, I.T., Frayn, K.N., Humphreys, S.M., MacLaren, D.P., Reilly, T. J. Appl. Physiol. (2003) [Pubmed]
  7. Biochemical efficacy and safety of monthly augmentation therapy for alpha 1-antitrypsin deficiency. Hubbard, R.C., Sellers, S., Czerski, D., Stephens, L., Crystal, R.G. JAMA (1988) [Pubmed]
  8. Constitutive and inducible expression of SKALP/elafin provides anti-elastase defense in human epithelia. Pfundt, R., van Ruissen, F., van Vlijmen-Willems, I.M., Alkemade, H.A., Zeeuwen, P.L., Jap, P.H., Dijkman, H., Fransen, J., Croes, H., van Erp, P.E., Schalkwijk, J. J. Clin. Invest. (1996) [Pubmed]
  9. Circulating anti-elastase in systemic lupus erythematosus. Nässberger, L., Jonsson, H., Sjöholm, A.G., Sturfelt, G., Heubner, A. Lancet (1989) [Pubmed]
  10. Sequence and molecular characterization of human monocyte/neutrophil elastase inhibitor. Remold-O'Donnell, E., Chin, J., Alberts, M. Proc. Natl. Acad. Sci. U.S.A. (1992) [Pubmed]
  11. Epigenetic heterochromatin markers distinguish terminally differentiated leukocytes from incompletely differentiated leukemia cells in human blood. Popova, E.Y., Claxton, D.F., Lukasova, E., Bird, P.I., Grigoryev, S.A. Exp. Hematol. (2006) [Pubmed]
  12. The effect of the specific elastase inhibitor, alanyl alanyl prolyl alanine chloromethylketone, on elastase-induced emphysema. Kleinerman, J., Ranga, V., Rynbrandt, D., Sorensen, J., Powers, J.C. Am. Rev. Respir. Dis. (1980) [Pubmed]
  13. Targeted disruption of SPI3/Serpinb6 does not result in developmental or growth defects, leukocyte dysfunction, or susceptibility to stroke. Scarff, K.L., Ung, K.S., Nandurkar, H., Crack, P.J., Bird, C.H., Bird, P.I. Mol. Cell. Biol. (2004) [Pubmed]
  14. Discriminating between the activities of human neutrophil elastase and proteinase 3 using serpin-derived fluorogenic substrates. Korkmaz, B., Attucci, S., Hazouard, E., Ferrandiere, M., Jourdan, M.L., Brillard-Bourdet, M., Juliano, L., Gauthier, F. J. Biol. Chem. (2002) [Pubmed]
  15. Characterization and chromosomal localization of ELANH2, the gene encoding human monocyte/neutrophil elastase inhibitor. Evans, E., Cooley, J., Remold-O'Donnell, E. Genomics (1995) [Pubmed]
  16. The serpin MNEI inhibits elastase-like and chymotrypsin-like serine proteases through efficient reactions at two active sites. Cooley, J., Takayama, T.K., Shapiro, S.D., Schechter, N.M., Remold-O'Donnell, E. Biochemistry (2001) [Pubmed]
  17. Secretory leukocyte proteinase inhibitor is a major leukocyte elastase inhibitor in human neutrophils. Sallenave, J.M., Si Tahar, M., Cox, G., Chignard, M., Gauldie, J. J. Leukoc. Biol. (1997) [Pubmed]
  18. Mutations on the hinge region of leukocyte elastase inhibitor determine the loss of inhibitory function. Perani, P., Zeggai, S., Torriglia, A., Courtois, Y. Biochem. Biophys. Res. Commun. (2000) [Pubmed]
  19. Stage specific gene expression of serpins and their cognate proteases during myeloid differentiation. Missen, M.A., Haylock, D., Whitty, G., Medcalf, R.L., Coughlin, P.B. Br. J. Haematol. (2006) [Pubmed]
  20. Strategies for aerosol therapy of alpha 1-antitrypsin deficiency by the aerosol route. Hubbard, R.C., Crystal, R.G. Lung (1990) [Pubmed]
  21. Oxidation of either methionine 351 or methionine 358 in alpha 1-antitrypsin causes loss of anti-neutrophil elastase activity. Taggart, C., Cervantes-Laurean, D., Kim, G., McElvaney, N.G., Wehr, N., Moss, J., Levine, R.L. J. Biol. Chem. (2000) [Pubmed]
  22. Influence of low molecular mass heparin on the kinetics of neutrophil elastase inhibition by mucus proteinase inhibitor. Cadène, M., Boudier, C., de Marcillac, G.D., Bieth, J.G. J. Biol. Chem. (1995) [Pubmed]
  23. Elafin (elastase-specific inhibitor) has anti-microbial activity against gram-positive and gram-negative respiratory pathogens. Simpson, A.J., Maxwell, A.I., Govan, J.R., Haslett, C., Sallenave, J.M. FEBS Lett. (1999) [Pubmed]
  24. The fusion of IGF I with stromal cell-derived factor I or alpha1 proteinase inhibitor alters their mitogenic or chemotactic activities while keeping their ability to inhibit HIV-1-gp120 binding. Sandoval, C., Stojanova, A., DiFalco, M.R., Congote, L.F. Biochem. Pharmacol. (2003) [Pubmed]
  25. Docosahexanoic acid (22:6, n-3) but not eicosapentaenoic acid (20:5, n-3) can induce neutrophil-mediated injury of cultured endothelial cells: involvement of neutrophil elastase. Bates, E.J., Ferrante, A., Harvey, D.P., Nandoskar, M., Poulos, A. J. Leukoc. Biol. (1993) [Pubmed]
  26. Neutrophil oxidants inactivate alpha-1-protease inhibitor and promote PMN-mediated detachment of cultured endothelium. Protection by free methionine. Stroncek, D.F., Vercellotti, G.M., Huh, P.W., Jacob, H.S. Arteriosclerosis (1986) [Pubmed]
  27. Lung lavage fluid from patients with alpha 1-proteinase inhibitor deficiency or chronic obstructive bronchitis: anti-elastase function and cell profile. Morrison, H.M., Kramps, J.A., Burnett, D., Stockley, R.A. Clin. Sci. (1987) [Pubmed]
  28. Mitogenic stimulation of human lymphocytes mediated by a cell surface elastase. Packard, B.Z., Mostowski, H.S., Komoriya, A. Biochim. Biophys. Acta (1995) [Pubmed]
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