Molecular pathways involved in injury-repair and ADPKD progression.

摘要: The major hallmark of Autosomal Dominant Polycystic Kidney Disease (ADPKD) is the formation many fluid-filled cysts in kidneys, which ultimately impairs normal renal structure and function, leading to end-stage disease (ESRD). A large body evidence suggests that injury-repair mechanisms are part ADPKD progression. Once have been formed, proliferation fluid secretion contribute cyst size increase, eventually causes stress on surrounding tissue resulting local injury fibrosis. In addition, can cause or accelerate formation. this review, we will describe various activated during repair show how they largely overlap with molecular PKD particular, discuss such as proliferation, inflammation, cell differentiation, cytokines growth factors secretion, following allow remodelling a proper organ repair. We also underline how, context PKD-related gene mutations, aberrant chronic activation these developmental pathways repair/remodelling results exacerbation disease.