Dysregulated ciliogenesis in scleroderma: novel pathogenic mechanism?

This RRF application builds on our groundbreaking recent discoveries showing that a novel cilia-associated gene called SPAG17 is markedly reduced in SSc skin biopsies, and loss of this gene causes aberrant ciliogenesis and multiple organ fibrosis. This RRF project seeks to understand the function of SPAG17 in ciliogenesis and fibrosis in SSc, with the ultimate goal of developing targeted therapies to correct altered ciliary signaling in the treatment of patients suffering from SSc