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Unfolded protein response in keratinocytes: impact on normal and abnormal keratinization.

Abstract：

:The unfolded protein response (UPR) is a signaling pathway from the endoplasmic reticulum (ER) to the nucleus that protects cells from stress caused by misfolded or unfolded proteins. As such, ER stress is an ongoing challenge for all cells, given the central biologic importance of secretion as part of normal physiologic functions. Mild UPR is activated by mild ER stress, which occurs under normal conditions. Abnormal UPR is activated by severe ER stress, which occurs under pathological conditions. Abnormal UPR activation is associated with a number of diseases, including diabetes mellitus and Alzheimer's disease. Within skin tissues, keratinocytes in the epidermis are especially dependent upon a mild UPR for normal differentiation in the course of their differentiation into secretory cells in the uppermost granular layers. Association between abnormal UPR activation and hereditary keratoses, including Darier's disease, keratosis linearis with ichthyosis congenita and keratoderma syndrome, erythrokeratoderma variabilis, and ichthyosis follicularis with atrichia and photophobia syndrome, have been elucidated recently. This review describes the UPR in normal and abnormal keratinization and discusses the regulation of abnormal UPR activation by chemical chaperones as a potential treatment for one of the hereditary keratoses.

journal_name

J Dermatol Sci

journal_title

Journal of dermatological science

authors

Sugiura K

doi

10.1016/j.jdermsci.2012.12.002

subject

Has Abstract

pub_date

2013-03-01 00:00:00

pages

181-6

issue

3

eissn

0923-1811

issn

1873-569X

pii

S0923-1811(12)00943-7

journal_volume

69

pub_type

杂志文章,评审

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