Succinate links mitochondria to deadly bacteria in cystic fibrosis
ANNALS OF TRANSLATIONAL MEDICINE(2019)
摘要
Cystic fibrosis (CF) is a life shortening–autosomal recessive disease caused by loss–of–function mutations in the gene encoding the CF transmembrane conductance regulator (CFTR) that transports essential electrolytes in and out of specialized cells (1). The loss of CFTR causes anomalies in the epithelial ion transport, leading to dehydration of cell surfaces and resulting in the accumulation of sticky obstructions that eventually destroy the integrity and physiology of different organ systems (2).
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