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Papillon–Lefe`vre Syndrome is a rare autosomal recessive disorder characterized by rapidly progressive periodontitis and confined palmoplantar hyperkeratosis resulting from genetic mutations in cathepsin C(CTSC).The present study investigated the effect of CTSC on keratinocyte proliferation and apoptosis. Ha Ca T keratinocytes were transfected with wild-type CTSC and CTSC-targeted si RNAs 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)-1, but not KRT9, was correlated with CTSC expression. Loricrin was increased in the CTSC-overexpression group and downregulated in the CTSC-silenced group. A positive association between loricrin expression and cell apoptosis was detected in Ha Ca T 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 Ha Ca T cells. This study suggested that loss of CTSC contributes to keratinocyte hyperkeratosis via downregulation of loricrin and enhanced cell proliferation.
Papillon-Lefe`vre Syndrome is a rare autosomal recessive disorder characterized by rapidly progressive periodontitis and confined palmoplantar hyperkeratosis resulting from genetic mutations in cathepsin C (CTSC). The present study investigating the effect of CTSC on keratinocyte proliferation and apoptosis. Ha Ca T keratinocytes were transfected with wild-type CTSC and CTSC-targeted si RNAs to investigate the effects of CTSC expression on cell keratosis. Real-time PCR and Western blot analysis showed that the levels of loricrin and keratin (KRT) -1, but not KRT9, was correlated with CTSC expression. Loricrin was increased in the CTSC-overexpression group and downregulated in the CTSC-silenced group. A positive association between loricrin expression and cell apoptosis was detected in HaCa T 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 Ha Ca T cells. This stud y suggested that loss of CTSC contributes to keratinocyte hyperkeratosis via downregulation of loricrin and enhanced cell proliferation.