Accumulating mitochondrial DNA mutations drive premature hematopoietic aging phenotypes distinct from physiological stem cell aging

Cell Stem Cell. 2011 May 6;8(5):499-510. doi: 10.1016/j.stem.2011.03.009.

Abstract

Somatic stem cells mediate tissue maintenance for the lifetime of an organism. Despite the well-established longevity that is a prerequisite for such function, accumulating data argue for compromised stem cell function with age. Identifying the mechanisms underlying age-dependent stem cell dysfunction is therefore key to understanding the aging process. Here, using a model carrying a proofreading-defective mitochondrial DNA polymerase, we demonstrate hematopoietic defects reminiscent of premature HSC aging, including anemia, lymphopenia, and myeloid lineage skewing. However, in contrast to physiological stem cell aging, rapidly accumulating mitochondrial DNA mutations had little functional effect on the hematopoietic stem cell pool, and instead caused distinct differentiation blocks and/or disappearance of downstream progenitors. These results show that intact mitochondrial function is required for appropriate multilineage stem cell differentiation, but argue against mitochondrial DNA mutations per se being a primary driver of somatic stem cell aging.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult Stem Cells / physiology
  • Aging, Premature / genetics*
  • Aging, Premature / physiopathology
  • Anemia
  • Animals
  • Cells, Cultured
  • Cellular Senescence / physiology*
  • Chimera
  • DNA Polymerase gamma
  • DNA, Mitochondrial / genetics
  • DNA-Directed DNA Polymerase / genetics
  • DNA-Directed DNA Polymerase / metabolism*
  • Gene Expression Profiling
  • Hematopoiesis / genetics
  • Hematopoietic Stem Cells / immunology
  • Hematopoietic Stem Cells / metabolism*
  • Hematopoietic Stem Cells / pathology
  • Lymphopenia
  • Membrane Potential, Mitochondrial
  • Mice
  • Mice, Inbred C57BL
  • Mice, Mutant Strains
  • Mitochondria / metabolism*
  • Mutation / genetics
  • Regeneration

Substances

  • DNA, Mitochondrial
  • DNA Polymerase gamma
  • DNA-Directed DNA Polymerase

Associated data

  • GEO/GSE14833
  • GEO/GSE18734
  • GEO/GSE27686
  • GEO/GSE6503
  • GEO/GSE8407