Mitochondrial biogenesis through activation of nuclear signaling proteins

Cold Spring Harb Perspect Biol. 2013 Jul 1;5(7):a015008. doi: 10.1101/cshperspect.a015008.

Abstract

The dynamics of mitochondrial biogenesis and function is a complex interplay of cellular and molecular processes that ultimately shape bioenergetics capacity. Mitochondrial mass, by itself, represents the net balance between rates of biogenesis and degradation. Mitochondrial biogenesis is dependent on different signaling cascades and transcriptional complexes that promote the formation and assembly of mitochondria--a process that is heavily dependent on timely and coordinated transcriptional control of genes encoding for mitochondrial proteins. In this article, we discuss the major signals and transcriptional complexes, programming mitochondrial biogenesis, and bioenergetic activity. This regulatory network represents a new therapeutic window into the treatment of the wide spectrum of mitochondrial and neurodegenerative diseases characterized by dysregulation of mitochondrial dynamics and bioenergetic deficiencies.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Energy Metabolism
  • Gene Expression Regulation
  • Humans
  • Mitochondria / metabolism*
  • Mitochondria / physiology
  • Mitochondrial Diseases / genetics
  • Mitochondrial Diseases / therapy
  • Mitochondrial Proteins / metabolism
  • Mitochondrial Turnover*
  • Models, Biological
  • Nuclear Proteins / metabolism*
  • Signal Transduction*

Substances

  • Mitochondrial Proteins
  • Nuclear Proteins