Radiologic differences between bone marrow stromal and hematopoietic progenitor cell lines from Fanconi Anemia (Fancd2(-/-)) mice

Radiat Res. 2014 Jan;181(1):76-89. doi: 10.1667/RR13405.1. Epub 2014 Jan 7.

Abstract

FancD2 plays a central role in the human Fanconi anemia DNA damage response (DDR) pathway. Fancd2(-/-) mice exhibit many features of human Fanconi anemia including cellular DNA repair defects. Whether the DNA repair defect in Fancd2(-/-) mice results in radiologic changes in all cell lineages is unknown. We measured stress of hematopoiesis in long-term marrow cultures and radiosensitivity in clonogenic survival curves, as well as comet tail intensity, total antioxidant stores and radiation-induced gene expression in hematopoietic progenitor compared to bone marrow stromal cell lines. We further evaluated radioprotection by a mitochondrial-targeted antioxidant GS-nitroxide, JP4-039. Hematopoiesis longevity in Fancd2(-/-) mouse long-term marrow cultures was diminished and bone marrow stromal cell lines were radiosensitive compared to Fancd2(+/+) stromal cells (Fancd2(-/-) D0 = 1.4 ± 0.1 Gy, ñ = 5.0 ± 0.6 vs. Fancd2(+/+) D0 = 1.6 ± 0.1 Gy, ñ = 6.7 ± 1.6), P = 0.0124 for D0 and P = 0.0023 for ñ, respectively). In contrast, Fancd2(-/-) IL-3-dependent hematopoietic progenitor cells were radioresistant (D0 = 1.71 ± 0.04 Gy and ñ = 5.07 ± 0.52) compared to Fancd2(+/+) (D0 = 1.39 ± 0.09 Gy and ñ = 2.31 ± 0.85, P = 0.001 for D0). CFU-GM from freshly explanted Fancd2(-/-) marrow was also radioresistant. Consistent with radiosensitivity, irradiated Fancd2(-/-) stromal cells had higher DNA damage by comet tail intensity assay compared to Fancd2(+/+) cells (P < 0.0001), slower DNA damage recovery, lower baseline total antioxidant capacity, enhanced radiation-induced depletion of antioxidants, and increased CDKN1A-p21 gene transcripts and protein. Consistent with radioresistance, Fancd2(-/-) IL-3-dependent hematopoietic cells had higher baseline and post irradiation total antioxidant capacity. While, there was no detectable alteration of radiation-induced cell cycle arrest with Fancd2(-/-) stromal cells, hematopoietic progenitor cells showed reduced G2/M cell cycle arrest. The absence of the mouse Fancd2 gene product confers radiosensitivity to bone marrow stromal but not hematopoietic progenitor cells.

Publication types

  • Comparative Study
  • Research Support, N.I.H., Extramural

MeSH terms

  • Animals
  • Antioxidants / metabolism
  • Cell Cycle / drug effects
  • Cell Cycle / radiation effects
  • Cell Line
  • Fanconi Anemia / metabolism
  • Fanconi Anemia / pathology*
  • Fanconi Anemia Complementation Group D2 Protein / deficiency*
  • Free Radical Scavengers / pharmacology
  • Granulocyte-Macrophage Progenitor Cells / metabolism
  • Hematopoiesis / drug effects
  • Hematopoiesis / radiation effects
  • Hematopoietic Stem Cells / drug effects
  • Hematopoietic Stem Cells / metabolism
  • Hematopoietic Stem Cells / pathology*
  • Hematopoietic Stem Cells / radiation effects*
  • Humans
  • Interleukin-3 / pharmacology
  • Mesenchymal Stem Cells / drug effects
  • Mesenchymal Stem Cells / metabolism
  • Mesenchymal Stem Cells / pathology*
  • Mesenchymal Stem Cells / radiation effects*
  • Mice
  • Mitochondria / drug effects
  • Mitochondria / metabolism
  • Mitochondria / radiation effects
  • Nitrogen Oxides / pharmacology
  • Oxidative Stress / drug effects
  • Oxidative Stress / radiation effects
  • Radiation Tolerance
  • Reactive Oxygen Species / metabolism
  • Time Factors

Substances

  • Antioxidants
  • Fanconi Anemia Complementation Group D2 Protein
  • Free Radical Scavengers
  • Interleukin-3
  • JP4-039
  • Nitrogen Oxides
  • Reactive Oxygen Species