Ferreira 2014 Abstract MiP2014
|Impairment of bioenergetic parameters induced by acute carnosine administration in skeletal muscle of young rats.|
Serum carnosinase deficiency is an inherited disorder that leads to an accumulation of carnosine in central and peripheral tissues of affected patients . Considering that patients report severe episodes of dystonia and lethargy  and that the pathophysiological mechanisms involved in serum carnosinase deficiency remain poorly understood, we investigated the in vivo effects of carnosine on bioenergetic parameters, namely respiratory complexes (CI–III, CII, and CII–III) , malate dehydrogenase , succinate dehydrogenase , and creatine kinase  activities, and the expression of mitochondrial-specific transcription factors (NRF-1, PGC-1α, and TFAM) in skeletal muscle of young Wistar rats. We observed a significant decrease of CI–III and CII activities in animals receiving carnosine acutely, as compared to the control group. However, no significant alteration in respiratory complexes, citric acid cycle enzymes, and creatine kinase activities were found between rats receiving carnosine chronically and control group animals. Compared to the control group, mRNA levels of NRF-1, PGC-1α and TFAM were unchanged. The present findings indicate that energy dysfunction occurs in skeletal muscle of rats receiving carnosine acutely, which suggests that a putative mechanism might be responsible for the muscle damage observed in serum carnosinase-deficient patients.
Labels: Pathology: Inherited
Organism: Rat Tissue;cell: Skeletal muscle
Enzyme: Complex I, Complex II;succinate dehydrogenase, Complex III
Event: C3, Poster MiP2014
1-Lab Neurochem, Univ Fed Rio de Janeiro; 2-Lab Inborn Errors Metabolism, Univ Extremo Sul Catarinense; 3-Lab Bioenergetics, Univ Extremo Sul Catarinense, Brazil; 4-Lab Genomics Molec Biol, Pontifícia Univ Católica Rio Grande do Sul; Brazil. - [email protected]
References and acknowledgements
Supported by UNESC, CNPq and NENASC.
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