Cerebellar Long Noncoding RNA Expression Profile in a Niemann-Pick C Disease Mouse Model.

ABSTRACT: Niemann-Pick type C (NP-C) disease is a neurodegenerative lysosomal storage disorder primarily caused by mutations in NPC1. However, its pathogenesis remains poorly understood. While mounting evidence has demonstrated the involvement of long noncoding RNAs (lncRNAs) in the pathogenesis of neurodegenerative disorders, the lncRNA expression profile in NP-C has not been determined. Here, we used RNA-seq analysis to determine lncRNA and mRNA expression profiles of the cerebella of NPC1^-/- mice. We found that 272 lncRNAs and 856 mRNAs were significantly dysregulated in NPC1^-/- mice relative to controls (≥ 2.0-fold, p < 0.05). Quantitative real-time PCR (qRT-PCR) was utilized to validate the expression of selected lncRNAs and mRNAs. Next, a lncRNA-mRNA coexpression network

SUBMITTER: Han S 

PROVIDER: S-EPMC8599378 | biostudies-literature | 2021 Nov

REPOSITORIES: biostudies-literature