1. Academic Validation
  2. Low dose TGF-β1 can improve vohwinkel syndrome by promoting the proliferation of keratinocytes

Low dose TGF-β1 can improve vohwinkel syndrome by promoting the proliferation of keratinocytes

  • Acta Histochem. 2023 Feb;125(2):152010. doi: 10.1016/j.acthis.2023.152010.
Xia Ling 1 Shujing Dong 2 Li Zhang 3
Affiliations

Affiliations

  • 1 Department of Dermatology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan 250021, Shandong, China; Shandong First Medical University, Jinan 250117, Shandong, China.
  • 2 Department of Dermatology, Affiliated Hospital of Shandong University of traditional Chinese Medicine, Jinan 250012, Shandong, China.
  • 3 Department of Dermatology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan 250021, Shandong, China. Electronic address: zhangliwenzhe@Medmail.com.cn.
Abstract

Vohwinkel syndrome (VS) is a very rare autosomal dominant disorder that can cause disability and deformity in severe cases. Mutations of the LOR (loricrin) and GJB2 (Cx26) genes have been found in VS so far. Many studies have indicated that the differentiation and growth of epidermal keratinocytes are regulated by mutant Cx26, and it may explain the pathogenesis of VS. It has been found that transforming growth factor β1 (TGF-β1) expression was lower in G130V (OE1) and D66H (OE2) mutant keratinocytes in the VS model with GJB2 mutation as compared to normal keratinocytes (NC). TGF-β is a cytokine involved in the regulation of processes like cell proliferation and differentiation in different types of cells. At present, many in vitro studies focus on TGF- β 1 inhibition of keratinocyte growth.However, the relationship between TGF-β1 and VS remains unknown. This study aimed at elucidating the role and potential pathogenic mechanism of TGF-β in VS. The results indicated that the down-regulation expression of TGF-β1 in VS was linked to cell proliferation inhibition through p-Smad3/c-Myc. In contrast, low-dose TGF-β1 treatment of VS keratinocytes can improve their proliferation inhibition and up-regulate the expression Cyclin D1. This suggests that low doses of TGF-β1 can improve the proliferation of VS and provide new insights into its treatment.

Keywords

GJB2; Transforming growth factor β1; Vohwinkel syndrome.

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