Serine Peptidase Inhibitor Kazal Type 5 (SPINK5)

SPINK5 encodes a multidomain serine protease inhibitor that contains 15 potential inhibitory domains. The inhibitor may play a role in skin and hair morphogenesis and anti-inflammatory and/or antimicrobial protection of mucous epithelia. Mutations may result in Netherton syndrome, a disorder characterized by ichthyosis, defective cornification, and atopy.The entire precursor protein, as deduced from the nucleotide sequence of the cloned cDNA, includes 15 potential inhibitory domains, 13 of which exhibit a Kazal-type-derived 4-cysteine-residue pattern that represents a novel protein module of serine protease inhibitor. Northern blot analysis and RT-PCR detected expression of LEKTI in thymus, vaginal epithelium, Bartholin gland, oral mucosa, tonsil, and parathyroid gland.