[Pathogenic and Compensatory Mechanisms in Epidermis of Sphingomyelin Synthase 2-Deficient Mice].

Autor:	Sakai S; Laboratory of Biomembrane and Biofunctional Chemistry, Faculty of Advanced Life Science, Hokkaido University, Sapporo, Japan.; Department of Biochemistry & Cell Biology, National Institute of Infectious Diseases, Tokyo, Japan., Makino A; Lipid Biology Laboratory, Advanced Science Institute, RIKEN Laboratory of Biochemistry, Asaka, Japan., Nishi A; Laboratory of Biochemistry, Azabu University School of Veterinary Medicine, Sagamihara, Japan., Ichikawa T; Department of Life Science, Medical Research Institute, Kanazawa Medical University, Kahoku, Japan., Yamashita T; Laboratory of Biochemistry, Azabu University School of Veterinary Medicine, Sagamihara, Japan., Taniguchi M; Department of Life Science, Medical Research Institute, Kanazawa Medical University, Kahoku, Japan., Tokudome Y; Laboratory of Dermatological Physiology, Faculty of Pharmaceutical Sciences, Josai University, Sakado, Japan., Hirabayashi Y; Department of Dermatology, Nagoya University Graduate School of Medicine, Nagoya, Japan., Akiyama M; Laboratory of Dermatological Physiology, Faculty of Pharmaceutical Sciences, Josai University, Sakado, Japan., Crumrine D; Department of Dermatology, School of Medicine, University of California, San Francisco, California, USA.; Northern California Institute for Research and Education, Veterans Affairs Medical Center, San Francisco, California, USA., Uchida Y; Department of Dermatology, School of Medicine, University of California, San Francisco, California, USA.; Northern California Institute for Research and Education, Veterans Affairs Medical Center, San Francisco, California, USA., Elias PM; Department of Dermatology, School of Medicine, University of California, San Francisco, California, USA.; Northern California Institute for Research and Education, Veterans Affairs Medical Center, San Francisco, California, USA., Tsuchida T; Department of Dermatology, Faculty of Medicine, Saitama Medical University, Moroyama, Japan., Hamanaka S; Department of Dermatology, Faculty of Medicine, Saitama Medical University, Moroyama, Japan.
Jazyk:	čínština
Zdroj:	Skin pharmacology and physiology [Skin Pharmacol Physiol] 2021; Vol. 34 (5), pp. 246-252. Date of Electronic Publication: 2021 Apr 29.
DOI:	10.1159/000515608
Abstrakt:	Sphingomyelin (SM) is a constituent of cellular membranes, while ceramides (Cer) produced from SM on plasma membranes serve as a lipid mediator that regulates cell proliferation, differentiation, and apoptosis. In the skin, SM also is a precursor of Cer, an important constituent of epidermal permeability barrier. We investigated the role of epidermal SM synthase (SMS)2, an isoform of SMS, which modulates SM and Cer levels on plasma membranes. Although SMS2-knockout (SMS2-KO) mice were not neonatal lethal, an ichthyotic phenotype with epidermal hyperplasia and hyperkeratosis was evident at birth, which persisted until 2 weeks of age. These mice showed abnormal lamellar body morphology and secretion, and abnormal extracellular lamellar membranes in the stratum corneum. These abnormalities were no longer evident by 4 weeks of age in SMS2-KO mice. Our study suggests that (1) exposure to a dry terrestrial environment initiates compensatory responses, thereby normalizing epidermal ichthyotic abnormalities and (2) that a nonlethal gene abnormality can cause an ichthyotic skin phenotype. (© 2021 S. Karger AG, Basel.)
Databáze:	MEDLINE
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