Elastase

Forms and classification

There exist two human genes for elastase:

• Pancreatic elastase (ELA-1)
• Neutrophil elastase (ELA-2)

Bacterial forms: Organisms such as P. aeruginosa also produce elastase, and is considered a virulence factor.

Function

Elastase breaks down elastin, an serine protease.

The role of human elastase in disease

A1AT

Elastase is inhibited by the α₁-antitryspin deficiency (A1AD) leads to uninhibited destruction of elastic fiber by elastase; the main result is pulmonary emphysema.

Cyclic hematopoeiesis

The rare disease cyclic hematopoeiesis (also called "cyclic neutropenia") is an autosomal dominant genetic disorder characterised by fluctuating neutrophil granulocyte counts over 21-day periods. During neutropenia, patients are at risk for infections. In 1999, this disease was linked to disorders in the ELA-2 gene.^[1] Other forms of congenital neutropenia also appear to be linked to ELA-2 mutations.

Other diseases

Neutrophil elastase is responsible for the blistering in bullous pemphigoid, a skin condition, in the presence of antibodies.

The role of bacterial elastase in disease

Elastase has been shown to disrupt tight junctions, cause proteolytic damage to tissue, break down cytokines and alpha proteinase inhibitor, cleave pathology.

References

1. ^ Horwitz M, Benson KF, Person RE, Aprikyan AG, Dale DC (1999). "Mutations in ELA2, encoding neutrophil elastase, define a 21-day biological clock in cyclic haematopoiesis". Nat. Genet. 23 (4): 433-6. doi:10.1038/70544. PMID 10581030.