Difference between revisions of "Onopiuk 2009 Biochem Biophys Res Commun"
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{{Publication | {{Publication | ||
|title=Onopiuk M, Brutkowski W, Wierzbicka K, Wojciechowska S, Szczepanowska J, Fronk J, Lochmüller H, Górecki DC, Zablocki K (2009) Mutation in dystrophin-encoding gene affects energy metabolism in mouse myoblasts. Biochem | |title=Onopiuk M, Brutkowski W, Wierzbicka K, Wojciechowska S, Szczepanowska J, Fronk J, Lochmüller H, Górecki DC, Zablocki K (2009) Mutation in dystrophin-encoding gene affects energy metabolism in mouse myoblasts. Biochem Biophys Res Commun 386:463-6. | ||
|info=[http://www.ncbi.nlm.nih.gov/pubmed/19527684 PMID: 19527684] | |||
|authors=Onopiuk M, Brutkowski W, Wierzbicka K, Wojciechowska S, Szczepanowska J, Fronk J, Lochmueller H, Gorecki DC, Zablocki K | |authors=Onopiuk M, Brutkowski W, Wierzbicka K, Wojciechowska S, Szczepanowska J, Fronk J, Lochmueller H, Gorecki DC, Zablocki K | ||
|year=2009 | |year=2009 | ||
|journal=Biochem | |journal=Biochem Biophys Res Commun | ||
|abstract=Duchenne Muscular Dystrophy is characterized by severe defects in differentiated muscle fibers, including abnormal calcium homeostasis and impaired cellular energy metabolism. Here we demonstrate that myoblasts derived from dystrophic (mdx) mouse exhibit reduced oxygen consumption, increased mitochondrial membrane potential, enhanced reactive oxygen species formation, stimulated glycolysis but unaffected total cellular ATP content. Moreover, reduced amounts of specific subunits of the mitochondrial respiratory complexes and ATP-synthase as well as disorganized mitochondrial network were observed. Both the dystrophic and control myoblasts used were derived from a common inbred mouse strain and the only difference between them is a point mutation in the dystrophin-encoding gene, thus these data indicate that this mutation results in multiple phenotypic alterations demonstrating as early as in undifferentiated myoblasts. This finding sheds new light on the molecular mechanisms of Duchenne Muscular Dystrophy pathogenesis. | |abstract=Duchenne Muscular Dystrophy is characterized by severe defects in differentiated muscle fibers, including abnormal calcium homeostasis and impaired cellular energy metabolism. Here we demonstrate that myoblasts derived from dystrophic (''mdx'') mouse exhibit reduced oxygen consumption, increased mitochondrial membrane potential, enhanced reactive oxygen species formation, stimulated glycolysis but unaffected total cellular ATP content. Moreover, reduced amounts of specific subunits of the mitochondrial respiratory complexes and ATP-synthase as well as disorganized mitochondrial network were observed. Both the dystrophic and control myoblasts used were derived from a common inbred mouse strain and the only difference between them is a point mutation in the dystrophin-encoding gene, thus these data indicate that this mutation results in multiple phenotypic alterations demonstrating as early as in undifferentiated myoblasts. This finding sheds new light on the molecular mechanisms of Duchenne Muscular Dystrophy pathogenesis. | ||
|keywords=mdx myoblast | |keywords=''mdx'' myoblast, Mitochondrial potential, Reactive oxygen species, Respiration, Mitochondrial organization | ||
| | |mipnetlab=PL Warsaw Zablocki K | ||
|discipline=Biomedicine | |||
}} | }} | ||
{{Labeling | {{Labeling | ||
| | |area=Respiration, Genetic knockout;overexpression | ||
|organism=Mouse | |organism=Mouse | ||
|tissues=Skeletal | |tissues=Skeletal muscle | ||
|preparations=Permeabilized | |preparations=Intact cells, Permeabilized cells | ||
| | |injuries=Oxidative stress;RONS | ||
|topics= | |diseases=Myopathy | ||
|topics=ATP, Ion;substrate transport | |||
|couplingstates=OXPHOS | |||
|instruments=Oxygraph-2k | |instruments=Oxygraph-2k | ||
| | |discipline=Biomedicine | ||
}} | }} | ||
Latest revision as of 17:41, 25 November 2015
| Onopiuk M, Brutkowski W, Wierzbicka K, Wojciechowska S, Szczepanowska J, Fronk J, Lochmüller H, Górecki DC, Zablocki K (2009) Mutation in dystrophin-encoding gene affects energy metabolism in mouse myoblasts. Biochem Biophys Res Commun 386:463-6. |
Onopiuk M, Brutkowski W, Wierzbicka K, Wojciechowska S, Szczepanowska J, Fronk J, Lochmueller H, Gorecki DC, Zablocki K (2009) Biochem Biophys Res Commun
Abstract: Duchenne Muscular Dystrophy is characterized by severe defects in differentiated muscle fibers, including abnormal calcium homeostasis and impaired cellular energy metabolism. Here we demonstrate that myoblasts derived from dystrophic (mdx) mouse exhibit reduced oxygen consumption, increased mitochondrial membrane potential, enhanced reactive oxygen species formation, stimulated glycolysis but unaffected total cellular ATP content. Moreover, reduced amounts of specific subunits of the mitochondrial respiratory complexes and ATP-synthase as well as disorganized mitochondrial network were observed. Both the dystrophic and control myoblasts used were derived from a common inbred mouse strain and the only difference between them is a point mutation in the dystrophin-encoding gene, thus these data indicate that this mutation results in multiple phenotypic alterations demonstrating as early as in undifferentiated myoblasts. This finding sheds new light on the molecular mechanisms of Duchenne Muscular Dystrophy pathogenesis. • Keywords: mdx myoblast, Mitochondrial potential, Reactive oxygen species, Respiration, Mitochondrial organization
• O2k-Network Lab: PL Warsaw Zablocki K
Labels: MiParea: Respiration, Genetic knockout;overexpression
Pathology: Myopathy
Stress:Oxidative stress;RONS
Organism: Mouse
Tissue;cell: Skeletal muscle
Preparation: Intact cells, Permeabilized cells
Regulation: ATP, Ion;substrate transport Coupling state: OXPHOS
HRR: Oxygraph-2k
