{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,1]],"date-time":"2026-01-01T05:27:59Z","timestamp":1767245279388,"version":"build-2065373602"},"reference-count":14,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2023,2,26]],"date-time":"2023-02-26T00:00:00Z","timestamp":1677369600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Experimental Dermatology"],"published-print":{"date-parts":[[2024,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Absence of a functional proteasome in the suprabasal layers of the epidermis is responsible for keratosis linearis with ichthyosis congenital and sclerosing keratoderma syndrome. Patient epidermis shows hypergranulosis associated with abnormally shaped keratohyalin granules and abnormal distribution of filaggrin in the\n                    <jats:italic>Stratum granulosum<\/jats:italic>\n                    and\n                    <jats:italic>Stratum corneum<\/jats:italic>\n                    . This suggests that the proteasome is involved in the degradation of filaggrin. To test this hypothesis, the proteasome proteolytic activity was inhibited in 3D reconstructed human epidermis (RHE) with the specific clasto\u2010lactacystin \u03b2\u2010lactone inhibitor. Confirming the efficacy of inhibition, ubiquitinated proteins accumulated in treated RHEs as compared to controls. Levels of urocanic acid (UCA) and pyrrolidone carboxylic acid (PCA), the end products of filaggrin degradation, were reduced. However, neither filaggrin accumulation nor appearance of filaggrin\u2010derived peptides were observed. On the contrary, the amount of filaggrin was shown to decrease, and a similar tendency was observed for profilaggrin, its precursor. Accumulation of small cytoplasmic vesicles associated with a significant increase in autophagy markers indicated activation of the autophagy process upon proteasome inhibition. Taken together, these results suggest that the perturbation of UCA and PCA production after proteasome inhibition was probably due to down\u2010regulation of filaggrin expression rather than to blocking of filaggrin proteolysis.\n                  <\/jats:p>","DOI":"10.1111\/exd.14772","type":"journal-article","created":{"date-parts":[[2023,2,20]],"date-time":"2023-02-20T03:04:27Z","timestamp":1676862267000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Human filaggrin monomer does not seem to be a proteasome target"],"prefix":"10.1111","volume":"33","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4154-6766","authenticated-orcid":false,"given":"Julie","family":"Briot","sequence":"first","affiliation":[{"name":"Toulouse Institute for Infectious and Inflammatory Diseases (Infinity) University of Toulouse, CNRS, INSERM, UPS  Toulouse France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eric","family":"Arbey","sequence":"additional","affiliation":[{"name":"L'Or\u00e9al Research and Innovation, Aulnay\u2010sous\u2010bois  Aulnay\u2010sous\u2010bois France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0338-5079","authenticated-orcid":false,"given":"Dominique","family":"Goudoun\u00e8che","sequence":"additional","affiliation":[{"name":"Centre de Microscopie Electronique Appliqu\u00e9e \u00e0 la Biologie Universit\u00e9 de Toulouse  Toulouse France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1034-0020","authenticated-orcid":false,"given":"Dominique","family":"Bernard","sequence":"additional","affiliation":[{"name":"L'Or\u00e9al Research and Innovation, Aulnay\u2010sous\u2010bois  Aulnay\u2010sous\u2010bois France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3655-6329","authenticated-orcid":false,"given":"Michel","family":"Simon","sequence":"additional","affiliation":[{"name":"Toulouse Institute for Infectious and Inflammatory Diseases (Infinity) University of Toulouse, CNRS, INSERM, UPS  Toulouse France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8915-8223","authenticated-orcid":false,"given":"Marie\u2010Claire","family":"M\u00e9chin","sequence":"additional","affiliation":[{"name":"Toulouse Institute for Infectious and Inflammatory Diseases (Infinity) University of Toulouse, CNRS, INSERM, UPS  Toulouse 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