serine protease inhibitor Kazal-type 5; lympho-epithelial Kazal-type-related inhibitor; LEKTI; contains: hemofiltrate peptide HF6478; hemofiltrate peptide HF7665 (SPINK5)

Function

• serine protease inhibitor
• probably important for the anti-inflammatory &/or antimicrobial protection of mucous epithelia
• contributes to the integrity & protective barrier function of the skin by regulating the activity of defense- activating & desquamation-involved proteases
• inhibits KLK5, it's major target, in a pH-dependent manner
• inhibits KLK7, KLK14 CASP14, & trypsin
• proteolytically processed by furin in individual domains (D1, D5, D6, D8 through D11, & D9 through D15) exhibiting various inhibitory potentials for multiple proteases

Structure

• contains at least one active inhibitory domain for trypsin (domain 6)
• contains 15 Kazal-like domains

Compartment

secreted

Alternative splicing

named isoforms=3

Expression

• highly expressed in the thymus & stratum corneum
• also found in the oral mucosa, parathyroid gland, Bartholin's glands, tonsils, & vaginal epithelium
• very low levels are detected in lung, kidney, & prostate

Pathology

• defects in SPINK5 are the cause of Netherton syndrome

More general terms

• Kazal type serine protease inhibitor
• secreted protein

Additional terms

• Netherton syndrome; trichorrhexis invaginata; bamboo hair

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=11005
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:11005
• OMIM: https://mirror.omim.org/entry/256500
• OMIM: https://mirror.omim.org/entry/605010
• UniProt: http://www.uniprot.org/uniprot/Q9NQ38.html