955 Altered expression of SPAG17, a cilia-related gene, in scleroderma and spontaneous skin fibrosis in Spag17 knockout mice suggests that scleroderma is a unique ciliopathy

Scleroderma is characterized by synchronous fibrosis in the skin and other organs with the myofibroblast as the main pathogenic cell driver. The mechanisms underlying mesenchymal progenitor-to-myofibroblast transition and sustained activation remain unknown. By unbiased RNAseq transcriptome analysis, we identified SPAG17, a gene encoding a cilia-related protein, as the most differentially-expressed gene in scleroderma skin biopsies (reduced 4.18-fold; p

Source: Journal of Investigative Dermatology - April 19, 2019 Category: Dermatology Authors: P.A. Sapao, B. Shi, E. Roberson, J. Atkinson, J.F. Strauss, M.E. Teves, J. Varga Tags: Tissue Regeneration and Wound Healing Source Type: research