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Alpha-defensins
Stable Identifier
R-HSA-1462054
Type
Pathway
Species
Homo sapiens
ReviewStatus
5/5
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Immune System (Homo sapiens)
Innate Immune System (Homo sapiens)
Antimicrobial peptides (Homo sapiens)
Defensins (Homo sapiens)
Alpha-defensins (Homo sapiens)
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Humans have 7 alpha defensin genes plus 5 pseudogenes (see HGNC at http://www.genenames.org/genefamilies/DEFA). Alpha-defensins have six cysteines linked 1-6, 2-4, 3-5. The canonical sequence of alpha-defensins in humans is x1-2CXCRx2-3Cx3Ex3GxCx3Gx5CCx1-4, where x represents any amino acid residue.
Human alpha-defensins 1-4 are often called human neutrophil peptides (HNP1-4) as they were initially identified in neutrophil primary (azurophilic) granules. Alpha-defensins 5 and 6 (HD5, HD6) are products of Paneth cells. HNP-1 and -3 peptides are 30 residues long, differing only in the first amino acid. They are encoded by the genes DEFA1 and DEFA3 respectively. These exhibit copy number polymorphism, with some individuals having 4-14 copies per diploid genome, while 10-37% of individuals have no copies of DEFA3 (Aldred et al. 2005, Linzmier & Ganz 2005, Ballana et al. 2007). HNP-4, encoded by DEFA4, is 33 amino acids long of which 22 differ from the other HNPs (Wilde et al. 1989). It is a minor component of neutrophil granules compared to HNP1-3. In contrast to DEFA1 and DEFA3, the genes for HNP-4, HD-5 and HD-6 are only found as two copies per diploid genome (Linzmeier & Ganz 2005). HNP-2 is 29 amino acids in length and is the proteolytic product of cleavage of the N-terminal amino acid from either HNP-1 and/or HNP-3 (Selsted et al. 1985).
Literature References
PubMed ID
Title
Journal
Year
12949495
Defensins: antimicrobial peptides of innate immunity
Ganz, T
Nat Rev Immunol
2003
Participants
Events
Pro-HNP1-4 are cleaved to biologically active defensin
(Homo sapiens)
HNP1-4 are stored in primary neutrophil granules
(Homo sapiens)
HNP1-4 are released into phagocytic vacuoles
(Homo sapiens)
pro-defensin alpha 5 is stored in Paneth cell granules
(Homo sapiens)
pro-defensin alpha 5 is secreted
(Homo sapiens)
pro-HD5 is cleaved by trypsin
(Homo sapiens)
Alpha-defensins form biologically active dimers
(Homo sapiens)
Alpha-defensin dimers adsorb onto microbial membrane anionic phospholipids
(Homo sapiens)
Alpha-defensin dimers multimerize to form a pore complex
(Homo sapiens)
HNP1-3 bind gp120
(Homo sapiens)
HNP1-3 bind CD4
(Homo sapiens)
ADP-Ribosylation of HNP-1
(Homo sapiens)
Participates
as an event of
Defensins (Homo sapiens)
Orthologous Events
Alpha-defensins (Mus musculus)
Alpha-defensins (Rattus norvegicus)
Authored
Jupe, S (2011-04-28)
Reviewed
McDermott, AM (2011-11-03)
Created
Jupe, S (2011-07-27)
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