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Serpin peptidase inhibitor, clade B (ovalbumin), member 1
Crystal structure of the cleaved equine leukocyte inhibitor.[1]
Available structures
PDB Ortholog search: PDBe, RCSB
External IDs OMIM130135 MGI1913472 HomoloGene133768 GeneCards: SERPINB1 Gene
RNA expression pattern
PBB GE SERPINB1 213572 s at tn.png
PBB GE SERPINB1 212268 at tn.png
More reference expression data
Species Human Mouse
Entrez 1992 66222
Ensembl ENSG00000021355 ENSMUSG00000044734
UniProt P30740 Q9D154
RefSeq (mRNA) NM_030666 NM_025429
RefSeq (protein) NP_109591 NP_079705
Location (UCSC) Chr 6:
2.83 – 2.84 Mb
Chr 13:
32.84 – 32.85 Mb
PubMed search [1] [2]

Leukocyte elastase inhibitor (LEI) also known as serpin B1 is a protein that in humans is encoded by the SERPINB1 gene. It is a member of the clade B serpins or ov-serpins (ovalbumin related serpins) founded by ovalbumin.[2][3][4]

MNEI (murine neutrophil elastase inhibitor) is the mouse orthologue of human SerpinB1.[4]


SerpinB1 is a cytoplasmic serine protease inhibitor of polymorphonuclear neutrophils. Among other serine proteases, it specifically inhibits neutrophil elastase, PR3 and cathepsin G, all found in neutrophil granules, by a suicide inhibition mechanism. SerpinB1 was found to reduce tissue damage caused by the mentioned proteases during inflammation and has a role in neutrophil homeostasis in mice. In various infection models (e.g. pneumonia) correlation of SerpinB1 absence and lack of microbial clearance have been shown. Different knockout strains serve as model to investigate the role of SerpinB1 in vivo.[5][6][7]

See also[edit]


  1. ^ PDB 1HLE; Baumann U, Bode W, Huber R, Travis J, Potempa J (August 1992). "Crystal structure of cleaved equine leucocyte elastase inhibitor determined at 1.95 A resolution". J. Mol. Biol. 226 (4): 1207–18. doi:10.1016/0022-2836(92)91062-T. PMID 1518052. 
  2. ^ Remold-O'Donnell E, Chin J, Alberts M (Jul 1992). "Sequence and molecular characterization of human monocyte/neutrophil elastase inhibitor". Proc Natl Acad Sci U S A 89 (12): 5635–9. doi:10.1073/pnas.89.12.5635. PMC 49347. PMID 1376927. 
  3. ^ "Entrez Gene: SERPINB1 serpin peptidase inhibitor, clade B (ovalbumin), member 1". 
  4. ^ a b Benarafa C, Remold-O'Donnell E (August 2005). "The ovalbumin serpins revisited: perspective from the chicken genome of clade B serpin evolution in vertebrates". Proc. Natl. Acad. Sci. U.S.A. 102 (32): 11367–72. doi:10.1073/pnas.0502934102. PMC 1183561. PMID 16055559. 
  5. ^ Gong D, Farley K, White M, Hartshorn KL, Benarafa C, Remold-O'Donnell E (August 2011). "Critical role of serpinB1 in regulating inflammatory responses in pulmonary influenza infection". J. Infect. Dis. 204 (4): 592–600. doi:10.1093/infdis/jir352. PMC 3144176. PMID 21791661. 
  6. ^ Benarafa C, LeCuyer TE, Baumann M, Stolley JM, Cremona TP, Remold-O'Donnell E (July 2011). "SerpinB1 protects the mature neutrophil reserve in the bone marrow". J. Leukoc. Biol. 90 (1): 21–9. doi:10.1189/jlb.0810461. PMC 3114599. PMID 21248149. 
  7. ^ Benarafa C, Priebe GP, Remold-O'Donnell E (August 2007). "The neutrophil serine protease inhibitor serpinb1 preserves lung defense functions in Pseudomonas aeruginosa infection". J. Exp. Med. 204 (8): 1901–9. doi:10.1084/jem.20070494. PMC 2118684. PMID 17664292. 

Further reading[edit]

  • Rasmussen HH, van Damme J, Puype M et al. (1993). "Microsequences of 145 proteins recorded in the two-dimensional gel protein database of normal human epidermal keratinocytes.". Electrophoresis 13 (12): 960–9. doi:10.1002/elps.11501301199. PMID 1286667. 
  • Packard BZ, Lee SS, Remold-O'Donnell E, Komoriya A (1995). "A serpin from human tumor cells with direct lymphoid immunomodulatory activity: mitogenic stimulation of human tumor-infiltrating lymphocytes.". Biochim. Biophys. Acta 1269 (1): 41–50. doi:10.1016/0167-4889(95)00113-7. PMID 7578269. 
  • Sugimori T, Cooley J, Hoidal JR, Remold-O'Donnell E (1995). "Inhibitory properties of recombinant human monocyte/neutrophil elastase inhibitor.". Am. J. Respir. Cell Mol. Biol. 13 (3): 314–22. doi:10.1165/ajrcmb.13.3.7654387. PMID 7654387. 
  • Maruyama K, Sugano S (1994). "Oligo-capping: a simple method to replace the cap structure of eukaryotic mRNAs with oligoribonucleotides.". Gene 138 (1-2): 171–4. doi:10.1016/0378-1119(94)90802-8. PMID 8125298. 
  • Evans E, Cooley J, Remold-O'Donnell E (1996). "Characterization and chromosomal localization of ELANH2, the gene encoding human monocyte/neutrophil elastase inhibitor.". Genomics 28 (2): 235–40. doi:10.1006/geno.1995.1136. PMID 8530031. 
  • Suzuki Y, Yoshitomo-Nakagawa K, Maruyama K et al. (1997). "Construction and characterization of a full length-enriched and a 5'-end-enriched cDNA library.". Gene 200 (1-2): 149–56. doi:10.1016/S0378-1119(97)00411-3. PMID 9373149. 
  • Zeng W, Silverman GA, Remold-O'Donnell E (1998). "Structure and sequence of human M/NEI (monocyte/neutrophil elastase inhibitor), an Ov-serpin family gene.". Gene 213 (1-2): 179–87. doi:10.1016/S0378-1119(98)00189-9. PMID 9630619. 
  • Sun J, Stephens R, Mirza G et al. (1999). "A serpin gene cluster on human chromosome 6p25 contains PI6, PI9 and ELANH2 which have a common structure almost identical to the 18q21 ovalbumin serpin genes.". Cytogenet. Cell Genet. 82 (3-4): 273–7. doi:10.1159/000015118. PMID 9858835. 
  • Perani P, Zeggai S, Torriglia A, Courtois Y (2000). "Mutations on the hinge region of leukocyte elastase inhibitor determine the loss of inhibitory function.". Biochem. Biophys. Res. Commun. 274 (3): 841–4. doi:10.1006/bbrc.2000.3191. PMID 10924364. 
  • Cooley J, Takayama TK, Shapiro SD et al. (2002). "The serpin MNEI inhibits elastase-like and chymotrypsin-like serine proteases through efficient reactions at two active sites.". Biochemistry 40 (51): 15762–70. doi:10.1021/bi0113925. PMID 11747453. 
  • Korkmaz B, Attucci S, Hazouard E et al. (2002). "Discriminating between the activities of human neutrophil elastase and proteinase 3 using serpin-derived fluorogenic substrates.". J. Biol. Chem. 277 (42): 39074–81. doi:10.1074/jbc.M202918200. PMID 12114510. 
  • Benarafa C, Cooley J, Zeng W et al. (2003). "Characterization of four murine homologs of the human ov-serpin monocyte neutrophil elastase inhibitor MNEI (SERPINB1).". J. Biol. Chem. 277 (44): 42028–33. doi:10.1074/jbc.M207080200. PMID 12189154. 
  • Strausberg RL, Feingold EA, Grouse LH et al. (2003). "Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences.". Proc. Natl. Acad. Sci. U.S.A. 99 (26): 16899–903. doi:10.1073/pnas.242603899. PMC 139241. PMID 12477932. 
  • Gevaert K, Goethals M, Martens L et al. (2004). "Exploring proteomes and analyzing protein processing by mass spectrometric identification of sorted N-terminal peptides.". Nat. Biotechnol. 21 (5): 566–9. doi:10.1038/nbt810. PMID 12665801. 
  • Mungall AJ, Palmer SA, Sims SK et al. (2003). "The DNA sequence and analysis of human chromosome 6.". Nature 425 (6960): 805–11. doi:10.1038/nature02055. PMID 14574404. 
  • Gerhard DS, Wagner L, Feingold EA et al. (2004). "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).". Genome Res. 14 (10B): 2121–7. doi:10.1101/gr.2596504. PMC 528928. PMID 15489334. 
  • Ahmed M, Forsberg J, Bergsten P (2005). "Protein profiling of human pancreatic islets by two-dimensional gel electrophoresis and mass spectrometry.". J. Proteome Res. 4 (3): 931–40. doi:10.1021/pr050024a. PMID 15952740. 

External links[edit]

This article incorporates text from the United States National Library of Medicine, which is in the public domain.