Browse by author
Lookup NU author(s): Dr Matthias Elstner, Emeritus Professor Doug Turnbull
Full text for this publication is not currently held within this repository. Alternative links are provided below where available.
The spectrum of genetic disorders associated with primary mitochondrial dysfunction ranges from isolated hearing loss to lethal neonatal syndromes. Mitochondrial biogenesis and function relies on the enigmatic interplay of the mitochondrial and nuclear genome and allows for adjustment of energy consumption to substrate availability and adaption to genetic and toxic stressors. Whole transcriptome studies permit a global perspective on these events and promise deeper insight into mitochondrial physiology and dysfunction. Data coming from microarray studies has revealed the activation of an intricate signaling network that promotes bioenergetic adaption through autophagy and enhanced mitochondrial biogenesis. The effectors of this network are currently under much investigation for their therapeutic potential. Microarray data also implicate a profound impact of mitochondrial dysfunction on global nuclear transcription activity through alteration of genomic stability, cell cycle progression and epigenetic regulation. In this review, results of gene expression studies performed on human and animal tissue as well as cell culture models with mitochondrial dysfunction are summarized and discussed. (C) 2011 Elsevier Inc. All rights reserved.
Author(s): Elstner M, Turnbull DM
Publication type: Review
Publication status: Published
Journal: Brain Research Bulletin
Year: 2012
Volume: 88
Issue: 4
Pages: 285-293
Print publication date: 01/07/2012
ISSN (print): 0361-9230
ISSN (electronic): 1873-2747
Publisher: PERGAMON-ELSEVIER SCIENCE LTD
URL: http://dx.doi.org/10.1016/j.brainresbull.2011.07.018
DOI: 10.1016/j.brainresbull.2011.07.018