Entry - *162815 - NEUTROPHIL AZUROCIDIN; NAZC - OMIM
 
* 162815

NEUTROPHIL AZUROCIDIN; NAZC


Alternative titles; symbols

AZUROCIDIN; AZU1
CATIONIC ANTIMICROBIAL PROTEIN-37; CAP37


HGNC Approved Gene Symbol: AZU1

Cytogenetic location: 19p13.3     Genomic coordinates (GRCh38): 19:827,837-832,018 (from NCBI)


TEXT

Cloning and Expression

Azurophil granules, specialized lysosomes of the neutrophil, contain at least 10 proteins implicated in the killing of microorganisms. Among these are 3 serine proteases that are additionally involved in degrading connective tissues: cathepsin G (116830), neutrophil elastase (130130), and proteinase-3 (PRTN3; 177020). These 3 proteins have collectively been termed serprocidins. Campanelli et al. (1990) isolated a 29-kD azurophil granule antibiotic protein, azurocidin, whose N-terminal sequence was highly homologous to the serprocidins but which lacked proteolytic activity. Azurocidin is the same as the 37-kD cationic antimicrobial protein of human neutrophil granules called CAP37. CAP37 is a specific chemoattractant for monocytes. It lacks the chemotactic activity for neutrophils and lymphocytes. It is probably responsible for the wave of monocytes that follows the initial wave of PMNs typical of the inflammatory response (Pereira et al., 1990).

Almeida et al. (1991) used a monospecific antibody to isolate from human bone marrow a cDNA encoding the entire azurocidin protein in its mature form, along with an N-terminal 24-residue hydrophobic peptide. The primary sequence was highly homologous to elastase, proteinase 3, cathepsin G, T-cell granzymes, and other serine proteases. However, azurocidin has gly for ser and ser for his substitutions in the catalytic triad. Southern blot analysis of human genomic DNA suggested the existence of a single azurocidin coding sequence.


Mapping

Zimmer et al. (1992) showed that the genes encoding azurocidin, neutrophil elastase (130130), and proteinase-3 (PRTN3; 177020) are in a cluster located at the tip of the short arm of chromosome 19. Each gene has 5 exons. All 3 genes are expressed coordinately and their protein products are packaged together into azurophil granules during neutrophil differentiation.


REFERENCES

  1. Almeida, R. P., Melchior, M., Campanelli, D., Nathan, C., Gabay, J. E. Complementary DNA sequence of human neutrophil azurocidin, an antibiotic with extensive homology to serine proteases. Biochem. Biophys. Res. Commun. 177: 688-695, 1991. [PubMed: 2049091, related citations] [Full Text]

  2. Campanelli, D., Detmers, P. A., Nathan, C. F., Gabay, J. E. Azurocidin and a homologous serine protease from neutrophils: differential antimicrobial and proteolytic properties. J. Clin. Invest. 85: 904-915, 1990. [PubMed: 2312733, related citations] [Full Text]

  3. Pereira, H. A., Shafer, W. M., Pohl, J., Martin, L. E., Spitznagel, J. K. CAP37: a human neutrophil-derived chemotactic factor with monocyte specific activity. J. Clin. Invest. 85: 1468-1476, 1990. [PubMed: 2332502, related citations] [Full Text]

  4. Zimmer, M., Medcalf, R. L., Fink, T. M., Mattmann, C., Lichter, P., Jenne, D. E. Three human elastase-like genes coordinately expressed in the myelomonocyte lineage are organized as a single genetic locus on 19pter. Proc. Nat. Acad. Sci. 89: 8215-8219, 1992. [PubMed: 1518849, related citations] [Full Text]


Contributors:
Alan F. Scott - updated : 8/15/1996
Creation Date:
Victor A. McKusick : 8/19/1991
alopez : 02/21/2014
mark : 8/19/1996
terry : 8/15/1996
carol : 11/23/1992
carol : 11/3/1992
carol : 9/29/1992
supermim : 3/16/1992
carol : 10/23/1991
carol : 10/15/1991

* 162815

NEUTROPHIL AZUROCIDIN; NAZC


Alternative titles; symbols

AZUROCIDIN; AZU1
CATIONIC ANTIMICROBIAL PROTEIN-37; CAP37


HGNC Approved Gene Symbol: AZU1

Cytogenetic location: 19p13.3     Genomic coordinates (GRCh38): 19:827,837-832,018 (from NCBI)


TEXT

Cloning and Expression

Azurophil granules, specialized lysosomes of the neutrophil, contain at least 10 proteins implicated in the killing of microorganisms. Among these are 3 serine proteases that are additionally involved in degrading connective tissues: cathepsin G (116830), neutrophil elastase (130130), and proteinase-3 (PRTN3; 177020). These 3 proteins have collectively been termed serprocidins. Campanelli et al. (1990) isolated a 29-kD azurophil granule antibiotic protein, azurocidin, whose N-terminal sequence was highly homologous to the serprocidins but which lacked proteolytic activity. Azurocidin is the same as the 37-kD cationic antimicrobial protein of human neutrophil granules called CAP37. CAP37 is a specific chemoattractant for monocytes. It lacks the chemotactic activity for neutrophils and lymphocytes. It is probably responsible for the wave of monocytes that follows the initial wave of PMNs typical of the inflammatory response (Pereira et al., 1990).

Almeida et al. (1991) used a monospecific antibody to isolate from human bone marrow a cDNA encoding the entire azurocidin protein in its mature form, along with an N-terminal 24-residue hydrophobic peptide. The primary sequence was highly homologous to elastase, proteinase 3, cathepsin G, T-cell granzymes, and other serine proteases. However, azurocidin has gly for ser and ser for his substitutions in the catalytic triad. Southern blot analysis of human genomic DNA suggested the existence of a single azurocidin coding sequence.


Mapping

Zimmer et al. (1992) showed that the genes encoding azurocidin, neutrophil elastase (130130), and proteinase-3 (PRTN3; 177020) are in a cluster located at the tip of the short arm of chromosome 19. Each gene has 5 exons. All 3 genes are expressed coordinately and their protein products are packaged together into azurophil granules during neutrophil differentiation.


REFERENCES

  1. Almeida, R. P., Melchior, M., Campanelli, D., Nathan, C., Gabay, J. E. Complementary DNA sequence of human neutrophil azurocidin, an antibiotic with extensive homology to serine proteases. Biochem. Biophys. Res. Commun. 177: 688-695, 1991. [PubMed: 2049091] [Full Text: https://doi.org/10.1016/0006-291x(91)91843-2]

  2. Campanelli, D., Detmers, P. A., Nathan, C. F., Gabay, J. E. Azurocidin and a homologous serine protease from neutrophils: differential antimicrobial and proteolytic properties. J. Clin. Invest. 85: 904-915, 1990. [PubMed: 2312733] [Full Text: https://doi.org/10.1172/JCI114518]

  3. Pereira, H. A., Shafer, W. M., Pohl, J., Martin, L. E., Spitznagel, J. K. CAP37: a human neutrophil-derived chemotactic factor with monocyte specific activity. J. Clin. Invest. 85: 1468-1476, 1990. [PubMed: 2332502] [Full Text: https://doi.org/10.1172/JCI114593]

  4. Zimmer, M., Medcalf, R. L., Fink, T. M., Mattmann, C., Lichter, P., Jenne, D. E. Three human elastase-like genes coordinately expressed in the myelomonocyte lineage are organized as a single genetic locus on 19pter. Proc. Nat. Acad. Sci. 89: 8215-8219, 1992. [PubMed: 1518849] [Full Text: https://doi.org/10.1073/pnas.89.17.8215]


Contributors:
Alan F. Scott - updated : 8/15/1996

Creation Date:
Victor A. McKusick : 8/19/1991

Edit History:
alopez : 02/21/2014
mark : 8/19/1996
terry : 8/15/1996
carol : 11/23/1992
carol : 11/3/1992
carol : 9/29/1992
supermim : 3/16/1992
carol : 10/23/1991
carol : 10/15/1991