VCP and chronic obstructive pulmonary disease: Moreover, deficiency of membrane CFTR also causes autophagy-impairment via induction of reactive oxygen species (ROS) [12], leading to peri-nuclear accumulation of damaged/misfolded proteins in aggresome-bodies, as seen by the accumulation of ubiquitinated proteins, p62 and VCP in the insoluble fraction, which may mediate COPD-like symptoms or BPD in pediatric subjects.