Distinct roles of stress-activated protein kinases in Fanconi anemia-type C-deficient hematopoiesis

Blood. 2009 Mar 19;113(12):2655-60. doi: 10.1182/blood-2008-09-181420. Epub 2009 Jan 23.

Abstract

The underlying molecular mechanisms that promote bone marrow failure in Fanconi anemia are incompletely understood. Evidence suggests that enhanced apoptosis of hematopoietic precursors is a major contributing factor. Previously, enhanced apoptosis of Fanconi anemia type C-deficient (Fancc(-/-)) progenitors was shown to involve aberrant p38 MAPK activation. Given the importance of c-Jun N-terminal kinase (JNK) in the stress response, we tested whether enhanced apoptosis of Fancc(-/-) cells also involved altered JNK activation. In Fancc(-/-) murine embryonic fibroblasts, tumor necrosis factor alpha (TNF-alpha) induced elevated JNK activity. In addition, JNK inhibition protected Fancc(-/-) murine embryonic fibroblasts and c-kit(+) bone marrow cells from TNF-alpha-induced apoptosis. Importantly, hematopoietic progenitor assays demonstrated that JNK inhibition enhanced Fancc(-/-) colony formation in the presence of TNF-alpha. Competitive repopulation assays showed that Fancc(-/-) donor cells cultured with the JNK inhibitor had equivalent levels of donor chimerism compared with Fancc(-/-) donor cells cultured with vehicle control. In contrast, culturing Fancc(-/-) cells with a p38 MAPK inhibitor significantly increased repopulating ability, supporting an integral role of p38 MAPK in maintaining Fancc(-/-) hematopoietic stem cell function. Taken together, these data suggest that p38 MAPK, but not JNK, has a critical role in maintaining the engraftment of Fancc(-/-)-reconstituting cells under conditions of stress.

Publication types

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

MeSH terms

  • Animals
  • Apoptosis / drug effects
  • Cells, Cultured / drug effects
  • Cells, Cultured / enzymology
  • Cells, Cultured / transplantation
  • Colony-Forming Units Assay
  • Enzyme Activation
  • Fanconi Anemia / enzymology*
  • Fanconi Anemia / pathology
  • Fanconi Anemia Complementation Group C Protein / deficiency*
  • Fibroblasts / drug effects
  • Fibroblasts / enzymology
  • Hematopoietic Stem Cell Transplantation
  • Hematopoietic Stem Cells / drug effects
  • Hematopoietic Stem Cells / enzymology
  • Hematopoietic Stem Cells / pathology
  • Imidazoles / pharmacology
  • Imidazoles / therapeutic use
  • Mice
  • Mice, Inbred C57BL
  • Mice, Knockout
  • Mitogen-Activated Protein Kinase 8 / antagonists & inhibitors
  • Mitogen-Activated Protein Kinase 8 / genetics
  • Mitogen-Activated Protein Kinase 8 / physiology*
  • Models, Animal
  • Protein Kinase Inhibitors / pharmacology
  • Protein Kinase Inhibitors / therapeutic use
  • Pyridines / pharmacology
  • Pyridines / therapeutic use
  • RNA Interference
  • RNA, Small Interfering / pharmacology
  • Radiation Chimera
  • Stress, Physiological
  • Tumor Necrosis Factor-alpha / pharmacology
  • p38 Mitogen-Activated Protein Kinases / antagonists & inhibitors
  • p38 Mitogen-Activated Protein Kinases / physiology*

Substances

  • Fancc protein, mouse
  • Fanconi Anemia Complementation Group C Protein
  • Imidazoles
  • Protein Kinase Inhibitors
  • Pyridines
  • RNA, Small Interfering
  • Tumor Necrosis Factor-alpha
  • Mitogen-Activated Protein Kinase 8
  • p38 Mitogen-Activated Protein Kinases
  • SB 203580