Pathway
Alpha-defensins
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.
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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. HNP-4, encoded by DEFA4, is 33 amino acids long of which 22 differ from the other HNPs. 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. 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.
What the reference databases state
Statements
8 statements
8 statements · 1 reference database · Subject: Alpha-defensins · Sources: Reactome
- ART1 participates in Alpha-defensinsProteinReactome R-HSA-1462054Inferred only
- CD4 participates in Alpha-defensinsProteinReactome R-HSA-1462054Inferred only
- DEFA1; DEFA1B participates in Alpha-defensinsProteinReactome R-HSA-1462054Inferred only
- DEFA3 participates in Alpha-defensinsProteinReactome R-HSA-1462054Inferred only
- DEFA5 participates in Alpha-defensinsProteinReactome R-HSA-1462054Inferred only
- DEFA6 participates in Alpha-defensinsProteinReactome R-HSA-1462054Inferred only
- PRSS2 participates in Alpha-defensinsProteinReactome R-HSA-1462054Inferred only
- PRSS3 participates in Alpha-defensinsProteinReactome R-HSA-1462054Inferred only