Skip Navigation


Human Molecular Genetics Advance Access originally published online on April 13, 2006
Human Molecular Genetics 2006 15(11):1801-1807; doi:10.1093/hmg/ddl102
This Article
Right arrow Full Text Freely available
Right arrow FREE Full Text (PDF) Freely available
Right arrowOA All Versions of this Article:
15/11/1801    most recent
ddl102v1
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in ISI Web of Science
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My Personal Archive
Right arrow Download to citation manager
Right arrow Search for citing articles in:
ISI Web of Science (3)
Google Scholar
Right arrow Articles by Inoue, S.-I.
Right arrow Articles by Hayashi, J.-I.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Inoue, S.-I.
Right arrow Articles by Hayashi, J.-I.
Social Bookmarking
Add to CiteULike   Add to Connotea   Add to Del.icio.us  
What's this?

© 2006 The Author(s)
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/2.0/uk/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited

Suppression of disease phenotypes of adult mito-mice carrying pathogenic mtDNA by bone marrow transplantation

Shin-Ichi Inoue1,3, Kaori Ishikawa1,2,3, Kazuto Nakada1,2, Akitsugu Sato1,2, Hiroyuki Miyoshi3 and Jun-Ichi Hayashi1,*

1Graduate School of Life and Environmental Sciences and 2Center for Tsukuba Advanced Research Alliance (TARA), University of Tsukuba, Tsukuba, Ibaraki 305-8572, Japan and 3Subteam for Manipulation of Cell Fate, BioResource Center, RIKEN Tsukuba Institute, Ibaraki 305-0074, Japan

* To whom correspondence should be addressed at: Graduate School of Life and Environmental Sciences, Institute of Biological Sciences, University of Tsukuba, 1-1-1 Tennoudai, Tsukuba, Ibaraki 305-8572, Japan. Tel/Fax: +81 298536650; Email: jih45{at}sakura.cc.tsukuba.ac.jp

Received March 6, 2006; Accepted April 7, 2006

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.


Add to CiteULike CiteULike   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us    What's this?


This article has been cited by other articles:


Home page
 Am. J. Physiol. Cell Physiol.Home page
S. M. Khan, R. M. Smigrodzki, and R. H. Swerdlow
Cell and animal models of mtDNA biology: progress and prospects
Am J Physiol Cell Physiol, February 1, 2007; 292(2): C658 - C669.
[Abstract] [Full Text] [PDF]


Disclaimer:
Please note that abstracts for content published before 1996 were created through digital scanning and may therefore not exactly replicate the text of the original print issues. All efforts have been made to ensure accuracy, but the Publisher will not be held responsible for any remaining inaccuracies. If you require any further clarification, please contact our Customer Services Department.