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Abstract
Valosin-containing human protein (VCP) or p97 performs enzyme functions associated with the maintenance of protein homeostasis and control of protein quality. Disruption of its normal functioning might be associated with the development of Parkinson’s disease (PD). Tissues of mice with toxin-induced presymptomatic and early symptomatic stages of PD, as well as 52 treated and untreated patients with newly diagnosed PD and nine patients with a “predicted” form of PD, were investigated. Significant changes in Vcp gene expression were observed in almost all studied mouse tissues. A significant decrease in VCP expression specific for PD was also detected at both the late preclinical and the early clinical stages of PD in untreated patients. Thus, a decrease in VCP expression is important for changes in the function of the nervous system at early stages of PD. Analysis of changes in VCP expression in all patients with PD and in Vcp in the peripheral blood of mice used as models of PD revealed significant decreases in expression specific for PD. These data suggest that a decrease in the relative levels of VCP mRNA might serve as a biomarker for the development of pathology at the early clinical and preclinical stages of human PD.
Highlights
Valosin-containing human protein (VCP) or p97 performs enzyme functions associated with the maintenance of protein homeostasis and control of protein quality
It has been shown that impairment of VCP functioning is involved in the accumulation of inclusions in expanding inclusion body myopathy with early-onset Paget disease and frontotemporal dementia[10,11,12], and mutations in VCP can be causative in Charcot–Marie–Tooth disease[13] and amyotrophic lateral sclerosis[14]
Analysis of changes in the Vcp protein level revealed the largest changes in the substantia nigra at all stages studied, and in the striatum at advanced presymptomatic stage (AdvPSS) and early symptomatic stage of PD (ESS)
Summary
Valosin-containing human protein (VCP) or p97 performs enzyme functions associated with the maintenance of protein homeostasis and control of protein quality. These data suggest that a decrease in the relative levels of VCP mRNA might serve as a biomarker for the development of pathology at the early clinical and preclinical stages of human PD. It was shown that patients with neurodegenerative diseases and mutations in VCP17,18 demonstrated signs of parkinsonism including unilateral rigidity, tremor, and bradykinesia[14,19,20] These data suggest that disruption of normal VCP functioning plays an important role in Parkinson’s disease (PD), which is the one of the most common neurodegenerative pathologies[21,22]
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