Ferreira 2014 Abstract MiP2014

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Impairment of bioenergetic parameters induced by acute carnosine administration in skeletal muscle of young rats.

Link: Mitochondr Physiol Network 19.13 - MiP2014

Ferreira GC, Macarini JR, Maravai SG, Cararo JH, Dimer NW, Goncalves CL, Kist LW, Bogo MR, Schuck PF, Streck EL (2014)

Event: MiP2014

Serum carnosinase deficiency is an inherited disorder that leads to an accumulation of carnosine in central and peripheral tissues of affected patients [1]. Considering that patients report severe episodes of dystonia and lethargy [2] 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) [3], malate dehydrogenase [4], succinate dehydrogenase [3], and creatine kinase [5] 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.

  1. Murphey WH, Lindmark DG, Patchen LI, Housler ME, Harrod EK, Mosovich L (1973) Serum carnosinase deficiency concomitant with mental retardation. Pediatr Res 7: 601–6.
  2. Lunde H, Sjaastad O, Gjessing L (1982) Homocarnosinosis: hypercarnosinuria. J Neurochem 38: 242–45.
  3. Silva CG, Silva AR, Ruschel C, Helegda C, Wyse AT, Wannmacher CM, Dutra- Filho CS, Wajner M (2000) Inhibition of energy production in vitro by glutaric acid in cerebral cortex of young rats. Metab Brain Dis 15: 123-31.
  4. Kitto GB (1969) Intra- and extramitochondrial malate dehydrogenases from chicken and tuna heart. Methods Enzymol 13: 106–16.
  5. Schuck PF, Leipnitz G, Ribeiro CA, Dalcin KB, Assis DR, Barschak AG, Pulrolnik V, Wannmacher CM, Wyse AT, Wajner M (2002) Inhibition of creatine kinase activity in vitro by ethylmalonic acid in cerebral cortex of young rats. Neurochem Res 27: 1633–9.