Murdoch University Research Repository

Welcome to the Murdoch University Research Repository

The Murdoch University Research Repository is an open access digital collection of research
created by Murdoch University staff, researchers and postgraduate students.

Learn more

Changes in intracellular energy transfer enzymes in muscles of mice with deleted wolframin (wfs1) gene

Eimre, M., Peet, N., Kadaja, L., Tarrend, M., Kasvandik, S., Orlova, E., Ivask, M. and Kõks, S. (2017) Changes in intracellular energy transfer enzymes in muscles of mice with deleted wolframin (wfs1) gene. Acta Physiologica, 219 . pp. 17-18.

Link to Published Version: https://doi.org/10.1111/apha.12841
*Subscription may be required

Abstract

Introduction
To study the mechanisms of Wolfram syndrome, we assessed the changes in activities, amounts and functional coupling between mitochondria and enzymes involved in the transport of energy in muscles of wfs1‐deficient mice, models of this syndrome.

Materials and methods
Samples of heart, m. soleus and m. rectus femoris of wfs1‐deficient and wild‐type mice. Real‐time PCR method, spectrophotometry and nano‐LC‐MS/MS analysis of homogenates. Coupling between mitochondria and enzymes was assayed by oxygraphy of permeabilized muscle fibres.

Results
Compared with wild type, in m. rectus femoris of wfs1‐deficient mice mRNA level of muscle‐type creatine kinase isoform was two times (P<0.05) lower, total activities of creatine and adenylate kinase decreased by 34% (P<0.01) and 48% (P<0.02) respectively. In wfs1‐deficient mice functional coupling of adenylate kinase and mitochondria in heart decreased by 39% (P<0.05), but in m. soleus and m. rectus femoris did not change. Coupling of mitochondria and creatine kinase did not alter in any muscle of wfs1‐deficient mice. The amounts of mitochondrial sarcomeric creatine kinase in wfs1‐deficient m. rectus femoris increased 3.16‐fold (P<0.001) and mitochondrial adenylate kinase 2 2.09‐fold (P<0.01), cytoplasmatic adenylate kinase 1 but 2.12‐fold (P<0.01) decreased compared with wild‐type muscle.

Conclusion
In hearts of wfs1‐deficient mice, functional coupling of adenylate kinase and mitochondria decreased. Despite the drop in total activities of creatine and adenylate kinase in m. rectus femoris, the functional coupling of mitochondria did not change, because the amounts of mitochondrial isoforms of these enzymes even increased.

Item Type: Journal Article
Publisher: Blackwell Publishing Inc.
Copyright: © 2017 The Authors
Other Information: Poster Abstracts from The 2016 Annual Meeting of the Scandinavian Physiological Society, University of Oslo, Oslo, Norway 26th – 28th August 2016
URI: http://researchrepository.murdoch.edu.au/id/eprint/51344
Item Control Page Item Control Page