Loss of cathepsin C enhances keratinocyte proliferation and inhibits apoptosis

Papillon-Lefevre Syndrome is a rare autosomal recessive disorder characterized by rapidly progressive periodontitis and confined palrnoplantar hyperkeratosis resulting from genetic mutations in cathepsin C （CTSC）. The present study investigated the effect of CTSC on keratinocyte proliferation and ap...

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Bibliographic Details
Published in:Science bulletin (Beijing) 2016-07, Vol.61 (14), p.1107-1114
Main Authors: Li, Xin, Jia, Ling-Fei, Zheng, Yunfei, Huang, Yiping, Qin, Man, Yang, Yuan
Format: Article
Language:English
Subjects:
blot分析
Cathepsin C
Chemistry/Food Science
Cytokeratin 1
Cytokeratin 9
Earth Sciences
Engineering
HaCaT细胞
Humanities and Social Sciences
Life Sciences
Loricrin
multidisciplinary
Papillon–Lefèvre syndrome
Physics
Science
Science (multidisciplinary)
实时荧光定量PCR
损失
组织蛋白酶
细胞凋亡
细胞增殖
角质形成细胞
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Summary:Papillon-Lefevre Syndrome is a rare autosomal recessive disorder characterized by rapidly progressive periodontitis and confined palrnoplantar hyperkeratosis resulting from genetic mutations in cathepsin C （CTSC）. The present study investigated the effect of CTSC on keratinocyte proliferation and apoptosis. HaCaT keratinocytes were transfected with wild-type CTSC and CTSC-targeted siRNAs to investigate the effects of CTSC expression on cell keratosis. Real-time PCR and Western blot analyses showed that the levels of loricrin and keratin （KRT）-I, but not KRT9, was correlated with CTSC expression. Loricrin was increased in the CTSC-overex- pression group and downregulated in the CTSC-silenced group. A positive association between loricrin expression and cell apoptosis was detected in HaCaT keratinocytes. KRT1 was decreased in the CTSC-overexpression group and increased in the CTSC-silenced group. Prominent, punctuate KRT1 aggregates were present in CTSC-knockdown HaCaT cells. This study suggested that loss of CTSC contributes to keratinocyte hyperkeratosis via downregulation of loricrin and enhanced cell proliferation.
ISSN:2095-9273
2095-9281
DOI:10.1007/s11434-016-1085-z