Kelch-like protein 42 is a profibrotic ubiquitin E3 ligase involved in systemic sclerosis.

ABSTRACT: Systemic scleroderma (SSc) is an autoimmune disease that affects over 2.5 million people globally. SSc results in dysfunctional connective tissues with excessive profibrotic signaling, affecting skin, cardiovascular, and particularly lung tissue. Over three-quarters of individuals with SSc develop pulmonary fibrosis within 5 years, the main cause of SSc mortality. No approved medicines to manage lung SSc currently exist. Recent research suggests that profibrotic signaling by transforming growth factor β (TGF-β) is directly tied to SSc. Previous studies have also shown that ubiquitin E3 ligases potently control TGF-β signaling through targeted degradation of key regulatory proteins; however, the roles of these ligases in SSc-TGF-β signaling remain unclear. Here we utilized primary SSc patie

SUBMITTER: Lear TB 

PROVIDER: S-EPMC7105301 | biostudies-literature | 2020 Mar

REPOSITORIES: biostudies-literature