Cathepsin B as a potential cystatin M/E target in the mouse hair follicle.

ABSTRACT: Deficiency of the cysteine protease inhibitor cystatin M/E (Cst6) in mice leads to disturbed epidermal cornification, impaired barrier function, and neonatal lethality. We report the rescue of the lethal skin phenotype of ichq (Cst6-deficient; Cst6^-/-) mice by transgenic, epidermis-specific, reexpression of Cst6 under control of the human involucrin (INV) promoter. Rescued Tg(INV-Cst6)Cst6^ichq/ichq mice survive the neonatal phase, but display severe eye pathology and alopecia after 4 mo. We observed keratitis and squamous metaplasia of the corneal epithelium, comparable to Cst6^-/-Ctsl^+/- mice, as we have reported in other studies. We found the INV promoter to be active in the hair follicle infundibulum; however,

SUBMITTER: Oortveld MAW 

PROVIDER: S-EPMC5602906 | biostudies-literature | 2017 Oct

REPOSITORIES: biostudies-literature