Abstract |
For directly addressing the issue of gene therapy of adult patients with mitochondrial diseases, we carried out bone marrow transplantation to adult mito-mice with mutated mtDNA and expressing respiration defects for improvement of disease phenotypes. We supposed that bone marrow cells transdifferentiated into various tissues, so that their transplantation would suppress disease phenotypes. The results showed improvement of survival and delayed expression of renal failure. As most mito-mice without a transplant died due to renal failure, we examined whether transplanted bone marrow cells transdifferentiated into renal tissues carrying improved renal function. Histochemical analyses showed that the suppression of disease phenotypes was not due to transdifferentiation, but due to suppression of apoptosis of renal cells. Thus, bone marrow cells possess a novel function of supporting tissues by suppressing apoptosis induced by respiration defects.
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Authors | Shin-Ichi Inoue, Kaori Ishikawa, Kazuto Nakada, Akitsugu Sato, Hiroyuki Miyoshi, Jun-Ichi Hayashi |
Journal | Human molecular genetics
(Hum Mol Genet)
Vol. 15
Issue 11
Pg. 1801-7
(Jun 01 2006)
ISSN: 0964-6906 [Print] England |
PMID | 16613898
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
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Chemical References |
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Topics |
- Animals
- Apoptosis
- Bone Marrow Transplantation
(methods)
- Cell Differentiation
- DNA, Mitochondrial
(metabolism)
- Genetic Therapy
(methods)
- Immunohistochemistry
- In Situ Nick-End Labeling
- Male
- Mice
- Mice, Transgenic
- Mutation
- Phenotype
- Renal Insufficiency
(metabolism)
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