Analysis of gene expression differences between utrophin/dystrophin-deficient vs mdx skeletal muscles reveals a specific upregulation of slow muscle genes in limb muscles

Neurogenetics
Patrick E BakerJill A Rafael-Fortney

Abstract

Dystrophin deficiency leads to the progressive muscle wasting disease Duchenne muscular dystrophy (DMD). Dystrophin-deficient mdx mice are characterized by skeletal muscle weakness and degeneration but they appear outwardly normal in contrast to DMD patients. Mice lacking both dystrophin and the dystrophin homolog utrophin [double knockout (dko)] have muscle degeneration similar to mdx mice, but they display clinical features similar to DMD patients. Dko limb muscles also lack postsynaptic membrane folding and display fiber-type abnormalities including an abundance of phenotypically oxidative muscle fibers. Extraocular muscles, which are spared in mdx mice, show a significant pathology in dko mice. In this study, microarray analysis was used to characterize gene expression differences between mdx and dko tibialis anterior and extraocular skeletal muscles in an effort to understand the phenotypic differences between these two dystrophic mouse models. Analysis of gene expression differences showed that upregulation of slow muscle genes specifically characterizes dko limb muscle and suggests that upregulation of these genes may directly account for the more severe phenotype of dko mice. To investigate whether any upregulation of s...Continue Reading

References

Dec 1, 1991·Journal of Neurocytology·P R Lyons, C R Slater
Aug 1, 1990·Muscle & Nerve·A NagelA G Engel
Dec 1, 1990·The Journal of Cell Biology·G E LyonsM Buckingham
Aug 1, 1972·Journal of Neurology, Neurosurgery, and Psychiatry·W G BradleyM Jenkison
Feb 1, 1970·Journal of the Neurological Sciences·C D Bell, P E Conen
May 15, 1965·Nature·R Close
Nov 10, 1995·The Journal of Biological Chemistry·D JungK P Campbell
Oct 1, 1994·Journal of Cellular Physiology·J P CanavanD F Goldspink
Apr 1, 1993·Nature Genetics·A H Ahn, L M Kunkel
Aug 30, 1994·Proceedings of the National Academy of Sciences of the United States of America·J M TinsleyK E Davies
Jul 1, 1993·Neuromuscular Disorders : NMD·A E Emery
Jun 14, 1996·Biochemical and Biophysical Research Communications·G RosaT C Petrucci
Jul 1, 1996·The Journal of Cell Biology·J A RafaelJ S Chamberlain
Nov 1, 1996·The Journal of Cell Biology·I N RybakovaJ M Ervasti
Feb 24, 1997·The Journal of Cell Biology·A E DeconinckK E Davies
Jun 21, 2001·The Journal of Laryngology and Otology·R P MillsE W Abel
Jul 14, 2001·Histochemistry and Cell Biology·D Pette, R S Staron
Jul 18, 2001·Journal of Applied Physiology·T J Hawke, D J Garry
Feb 13, 2003·Nucleic Acids Research·Rafael A IrizarryTerence P Speed
Oct 2, 2003·Journal of Molecular and Cellular Cardiology·W A LaFramboiseJ A Magovern
Oct 2, 2003·Annals of Neurology·Homa TajsharghiAnders Oldfors
Jul 22, 2004·Muscle & Nerve·Jamie L SanfordJill A Rafael-Fortney
Mar 17, 2005·European Heart Journal·Eric VillardMichel Komajda
Apr 28, 2005·Journal of Molecular Medicine : Official Organ of the Gesellschaft Deutscher Naturforscher Und Ärzte·Andreas PerrotKarl Josef Osterziel
Oct 14, 2005·Progress in Brain Research·Robert F Spencer, John D Porter

❮ Previous
Next ❯

Citations

May 2, 2012·Molecular Therapy : the Journal of the American Society of Gene Therapy·Dawn A DelfínJill A Rafael-Fortney
Dec 29, 2010·European Journal of Orthodontics·Alexander SpassovChristiane Kunert-Keil
May 7, 2009·FASEB Journal : Official Publication of the Federation of American Societies for Experimental Biology·Peter KloverLothar Hennighausen
Feb 5, 2010·American Journal of Physiology. Cell Physiology·Joana CapoteJulio L Vergara
Mar 6, 2012·BMC Bioinformatics·Donny SohLimsoon Wong
Jan 16, 2009·The FEBS Journal·Lydie LaureNathalie Danièle
Sep 1, 2007·Proteomics. Clinical Applications·Philip DoranKay Ohlendieck
Apr 14, 2016·The Journal of General Physiology·Marijana Sekulic-JablanovicSusan Treves
Jun 13, 2014·PLoS Genetics·Glen B BanksJeffrey S Chamberlain
Jan 19, 2010·Journal of Muscle Research and Cell Motility·Caroline LewisKay Ohlendieck
May 21, 2014·Cranio : the Journal of Craniomandibular Practice·Nicolae ChipailaAnnalisa Monaco
Jul 17, 2015·FASEB Journal : Official Publication of the Federation of American Societies for Experimental Biology·Jessica A ChadwickJill A Rafael-Fortney
Jun 5, 2020·International Journal of Molecular Sciences·Kiisa NishikawaDhruv Mishra
Feb 19, 2021·Database : the Journal of Biological Databases and Curation·Nathaniel LimPaul Pavlidis
Jun 10, 2021·Communications Biology·James MooreErik C Böttger

❮ Previous
Next ❯

Related Concepts

Related Feeds

Cachexia & Brown Fat

Cachexia is a condition associated with progressive weight loss due to severe illness. In cancer patients, it is proposed to occur as a result of tumor-induced energy wasting. Several proteins have been implicated in browning and depletion of white adipose tissue. Here is the latest research on cachexia and brown fat.

Cardiac Cachexia

Cardiac cachexia is a syndrome associated with the progressive loss of muscle and fat mass. It most commonly affects patients with heart failure and can significantly decrease the quality of life and survival in these patients. Here is the latest research on cardiac cachexia.