TDP-43 regulates the microprocessor complex activity during in vitro neuronal differentiation

Mol Neurobiol. 2013 Dec;48(3):952-63. doi: 10.1007/s12035-013-8564-x. Epub 2013 Oct 11.

Abstract

TDP-43 (TAR DNA-binding protein 43) is an RNA-binding protein implicated in RNA metabolism at several levels. Even if ubiquitously expressed, it is considered as a neuronal activity-responsive factor and a major signature for neurological pathologies, making the comprehension of its activity in the nervous system a very challenging issue. TDP-43 has also been described as an accessory component of the Drosha-DGCR8 (DiGeorge syndrome critical region gene 8) microprocessor complex, which is crucially involved in basal and tissue-specific RNA processing events. In the present study, we exploited in vitro neuronal differentiation systems to investigate the TDP-43 demand for the microprocessor function, focusing on both its canonical microRNA biosynthetic activity and its alternative role as a post-transcriptional regulator of gene expression. Our findings reveal a novel role for TDP-43 as an essential factor that controls the stability of Drosha protein during neuronal differentiation, thus globally affecting the production of microRNAs. We also demonstrate that TDP-43 is required for the Drosha-mediated regulation of Neurogenin 2, a master gene orchestrating neurogenesis, whereas post-transcriptional control of Dgcr8, another Drosha target, resulted to be TDP-43-independent. These results implicate a previously uncovered contribution of TDP-43 in regulating the abundance and the substrate specificity of the microprocessor complex and provide new insights into TDP-43 as a key player in neuronal differentiation.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Basic Helix-Loop-Helix Transcription Factors / metabolism
  • Cell Differentiation / drug effects
  • Cell Differentiation / genetics*
  • Cell Line, Tumor
  • DNA-Binding Proteins / metabolism*
  • Gene Expression Profiling
  • Gene Expression Regulation, Neoplastic / drug effects
  • Gene Knockdown Techniques
  • Humans
  • MicroRNAs / genetics
  • MicroRNAs / metabolism*
  • Models, Biological
  • Nerve Tissue Proteins / metabolism
  • Neuroblastoma / genetics
  • Neuroblastoma / pathology
  • Neurons / cytology*
  • Proteasome Inhibitors / pharmacology
  • Protein Binding / drug effects
  • Protein Binding / genetics
  • Proteins / metabolism
  • RNA, Messenger / genetics
  • RNA, Messenger / metabolism
  • RNA-Binding Proteins
  • Ribonuclease III / genetics
  • Ribonuclease III / metabolism

Substances

  • Basic Helix-Loop-Helix Transcription Factors
  • DGCR8 protein, human
  • DNA-Binding Proteins
  • MicroRNAs
  • NEUROG2 protein, human
  • Nerve Tissue Proteins
  • Proteasome Inhibitors
  • Proteins
  • RNA, Messenger
  • RNA-Binding Proteins
  • DROSHA protein, human
  • Ribonuclease III