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KLK11

Function

Possible multifunctional protease. Efficiently cleaves 'bz-Phe-Arg-4-methylcoumaryl-7-amide', a kallikrein substrate, and weakly cleaves other substrates for kallikrein and trypsin. Cleaves synthetic peptides after arginine but not lysine residues.

Involvement in disease

Ichthyosis with erythrokeratoderma

IEKD

An autosomal dominant genodermatosis characterized by early-onset ichthyosiform erythroderma with excessive skin scaling and peeling, and erythematous hyperkeratotic plaques. Lesions are present at birth or appear soon after.

None

The disease is caused by variants affecting the gene represented in this entry.

Post-translational modifications

About 40% of KLK11 is inactivated by internal cleavage after Arg-188. This proteolytic inactivation may be effected by plasminogen.

Sequence Similarities

Belongs to the peptidase S1 family. Kallikrein subfamily.

Tissue Specificity

Expressed in brain, skin and prostate. Isoform 1 is expressed preferentially in brain. Isoform 2 is expressed in prostate. Present in seminal plasma at concentrations ranging from 2 to 37 microg/mL (at protein level).

Cellular localization

Alternative names

PRSS20, TLSP, UNQ649/PRO1279, KLK11, Kallikrein-11, hK11, Hippostasin, Serine protease 20, Trypsin-like protease

swissprot:Q9UBX7 entrezGene:11012 omim:604434