{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"omics_type":["Unknown"],"volume":["31(10)"],"submitter":["Oortveld MAW"],"pubmed_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 <i>ichq</i> (Cst6-deficient; <i>Cst6</i><sup>-/-</sup>) mice by transgenic, epidermis-specific, reexpression of Cst6 under control of the human involucrin (INV) promoter. Rescued Tg(INV-<i>Cst6</i>)<i>Cst6<sup>ichq/ichq</sup></i> 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 <i>Cst6<sup>-/-</sup>Ctsl<sup>+/-</sup></i> mice, as we have reported in other studies. We found the INV promoter to be active in the hair follicle infundibulum; however,"],"journal":["FASEB journal : official publication of the Federation of American Societies for Experimental Biology"],"pagination":["4286-4294"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC5602906"],"repository":["biostudies-literature"],"pubmed_title":["Cathepsin B as a potential cystatin M/E target in the mouse hair follicle."],"pmcid":["PMC5602906"],"pubmed_authors":["Cheng T","Oortveld MAW","Verdoes M","Reinheckel T","Schalkwijk J","van Erp PEJ","Hendriks WJAJ","Zeeuwen PLJM","Kersten FFJ","Verbeek S","van Vlijmen-Willems IMJJ"],"additional_accession":[]},"is_claimable":false,"name":"Cathepsin B as a potential cystatin M/E target in the mouse hair follicle.","description":"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 <i>ichq</i> (Cst6-deficient; <i>Cst6</i><sup>-/-</sup>) mice by transgenic, epidermis-specific, reexpression of Cst6 under control of the human involucrin (INV) promoter. Rescued Tg(INV-<i>Cst6</i>)<i>Cst6<sup>ichq/ichq</sup></i> 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 <i>Cst6<sup>-/-</sup>Ctsl<sup>+/-</sup></i> mice, as we have reported in other studies. We found the INV promoter to be active in the hair follicle infundibulum; however,","dates":{"release":"2017-01-01T00:00:00Z","publication":"2017 Oct","modification":"2025-04-05T14:31:21.925Z","creation":"2019-03-27T02:56:39Z"},"accession":"S-EPMC5602906","cross_references":{"pubmed":["28596234"],"doi":["10.1096/fj.201700267R"]}}