Effect of in vivo administration of ethylmalonic acid on energy metabolism in rat tissues

Metabolic Brain Disease

Volumn 21, Issue 1, 2006, Pages 29-39

  Ferreira, Gustavo Da C ^a   André, Karina Roth ^a   Schuck, Patrícia Fernanda ^a   Viegas, Carolina M ^a   Tonin, Anelise ^a   Coelho, Daniella De Moura ^a   Wyse, Angela T S ^a   Wannmacher, Clóvis M D ^a   Vargas, Carmen R ^a,b   Wajner, Moacir ^a,b,c  

^a FEDERAL UNIVERSITY OF RIO GRANDE DO SUL   (Brazil)

^b HOSPITAL DE CLÍNICAS DE PORTO ALEGRE   (Brazil)

^c UNIVERSIDADE LUTERANA DO BRASIL   (Brazil)

Author keywords

Energy metabolism; Ethylmalonic acid; Ethylmalonic aciduria; SCAD deficiency

Indexed keywords

ETHYLMALONIC ACID; MALONIC ACID DERIVATIVE; SODIUM CHLORIDE; UNCLASSIFIED DRUG;

ACIDEMIA; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; BODY WEIGHT; BRAIN CORTEX; BRAIN REGION; CONTROLLED STUDY; CORPUS STRIATUM; DRUG EFFECT; ENERGY METABOLISM; IN VIVO STUDY; NEUROCHEMISTRY; NONHUMAN; PATHOPHYSIOLOGY; RAT; RAT STRAIN; RESPIRATORY CHAIN; SKELETAL MUSCLE;

ACIDOSIS; AGE FACTORS; ANIMALS; BUTYRYL-COA DEHYDROGENASE; CEREBRAL CORTEX; ELECTRON TRANSPORT COMPLEX I; ELECTRON TRANSPORT COMPLEX II; ELECTRON TRANSPORT COMPLEX III; ELECTRON TRANSPORT COMPLEX IV; ENERGY METABOLISM; ENZYME ACTIVATION; FEMALE; INJECTIONS, SUBCUTANEOUS; MALE; MALONATES; MUSCLE, SKELETAL; MYOCARDIUM; PREGNANCY; RATS; RATS, WISTAR;

ANIMALIA; RATTUS NORVEGICUS;

EID: 33745075215     PISSN: 08857490     EISSN: None     Source Type: Journal    
DOI: 10.1007/3-540-32849-1_2     Document Type: Article

References (27)

1. Armstrong DL, Masiowski ML, Wood PA (1993) Pathologic characterization of short-chain acyl-CoA dehydrogenase deficiency in BALB/cByJ mice. Am J Med Genet 47:884-892
2. Bennet MJ, Weinberger MJ, Kobory JA, Rinaldo P, Burlina AB (1996) Mitochondrial short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase deficiency: A new defect of fatty acid oxidation. Pediatr Res 39:185-188
3. Bhala A, Willi SM, Rinaldo P, Bennet MJ, Schmid-Sommerfeld E, Hale DE (1995) Clinical and biochemical characterization of short-chain acyl-coenzyme A dehydrogenase. J Pediatr 126:910-915
4. Brusque AM, Mello CF, Buchanan DN, Terracciano ST, Rocha MP, Vargas CR, Wannmacher CM, Wajner M (1999) Effect of chemically induced propionic acidemia on neurobehavioral development of rats. Pharmacol Biochem Behav 64:529-534
5. Corydon MJ, Gregersen N, Lehnert W, Ribes A, Rinaldo P, Kmoch S, Christensen E, Kristensen TJ, Andresen BS, Bross P, Winter V, Martinez G, Neve S, Jensen TG, Bolund L, Kølvraa S (1996) Ethylmalonic aciduria is associated with an amino acid variant of short-chain acyl-coenzyme: A dehydrogenase. Pediatr Res 39:1059-1966
6. Corydon MJ, Vockley J, Rinaldo P, Rhead WJ, Kjeldsen M, Winter V, Riggs C, Babovic-Vuksanovic D, Smeitink J, De Jong J, Levy H, Sewell AC, Roe C, Matern D, Dasouki M, Gregersen N (2001) Role of common gene variations in the molecular pathogenesis of short-chain acyl-CoA dehydrogenase deficiency. Pediatr Res 49:18-23
7. da Silva CG, Ribeiro CA, Leipnitz G, Dutra-Filho CS, Wyse AT, Wannmacher CM, Sarkis JJ, Jakobs C, Wajner M (2002) Inhibition of cytochrome c oxidase activity in rat cerebral cortex and human skeletal muscle by D-2-hydroxyglutaric acid in vitro. Biochim Biophys Acta 1586:81-91
8. Dawson DB, Waber L, Hale DE, Bennet MJ (1995) Transient organic aciduria and persistent lactic acidemia in a patient with short-cahin acyl-Coenzyme: A dehydrogenase deficiency. J Pediatr 126:69-71
9. Dutra JC, Wajner M, Wannmacher CM, Wannmacher LE, Pires RF, Rosa-Junior A (1991) Effect of postnatal methylmalonate administration on adult rat behavior. Braz J Med Biol Res 24:595-605
10. Fischer JC, Ruitenbeek W, Berden JA, Trijbels JM, Veerkamp JH, Stadhouders AM, Sengers RC, Janssen AJ (1985) Differential investigation of the capacity of succinate oxidation in human skeletal muscle. Clin Chim Acta 153:23-36
11. Garcia-Silva MT, Campos Y, Ribes A, Briones P, Cabello A, Santos Borbujo J, Arenas J, Garavaglia B (1994) Encephalopathy, petechiae, and acrocyanosis with ethylmalonic aciduria associated with muscle cytochrome c oxidase deficiency. J Pediatr 125:843
12. Garcia-Silva MT, Ribes A, Campos Y, Garavaglia B, Arenas J (1997) Syndrome of encephalopathy, petechiae, and ethylmalonic aciduria. Pediatr Neurol 17:165-170
13. Hughes BP (1962) A method for the estimation of serum creatine kinase and its use in comparing creatine kinase and aldolase activity in normal and pathological sera. Clin Chim Acta 7:597-603
14. Leipnitz G, Schuck PF, Ribeiro CA, Dalcin KB, Assis DR, Barschak AG, Pulrolnik V, Wannmacher CM, Wyse AT, Wajner M (2003) Ethylmalonic acid inhibits mitochondrial creatine kinase activity from cerebral cortex of young rats in vitro. Neurochem Res 28:771-777
15. Lowry OH, Rosebrough NJ, Farr AL, Randall RJ (1951) Protein measurement with the Folin phenol reagent. J Biol Chem 193:265-275
16. Mello CF, Feksa L, Brusque AM, Wannmacher CM, Wajner M (1999) Chronic early leucine administration induces behavioral deficits in rats. Life Sci 65:747-755
17. Mello CF, Somer JE, Tavaroni V, Graciolli R, Wu V, Torres G, Aguiar F, Wajner M, Wannmacher CM (1994) Effects of postnatal methylmalonate administration on neurobehavioral development of rats. Braz J Med Biol Res 27:655-661
18. Oldendorf WH (1973) Carrier-mediated blood-brain barrier transport of short chain monocarboxylic organic acids. Am J Physiol 224:1450-1453
19. Ozand PT, Rashed M, Millington DS, Sakati N, Hazzaa S, Rahbeeni Z, Alodaib A, Youssef N, Mazrou A, Gascon GG, Brismar J (1994) Ethylmalonic aciduria: An organic acidemia with CNS involvement and vasculopathy. Brain Dev 16:12-22
20. Reichmann H, Maltese WA, De Vivo DC (1988) Enzymes of fatty acid beta-oxidation in developing brain. J Neurochem 51:339-344
21. Roe CR, Ding J (2001) Mitochondrial fatty acid oxidation disorders. In: Scriver CR, Beaudet AL, Sly WS, Valle D (eds) The metabolic and molecular bases of inherited disease, 8th edn. McGraw-Hill, New York, pp 2297-2326
22. Rustin P, Chretien D, Bourgeron T, Gerard B, Rotig A, Saudubray JM, Munnich A (1994) Biochemical and molecular investigations in respiratory chain deficiencies. Clin Chim Acta 228:35-51
23. Schapira AH, Cooper JM, Dexter D, Clark JB, Jenner P, Marsden CD (1990) Mitochondrial complex I deficiency in Parkinson's disease. J Neurochem 54:823-827
24. 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 27:1633-1639
25. Sewell AC, Herwig J, Böhles H, Rinaldo P, Bhala A, Hale DE (1993) A new case of short-chain acyl-CoA dehydrogenase deficiency with isolated ethylmalonic aciduria. Eur J Pediatr 152:922-924
26. Sweetman L (1991) Organic acid analysis. In: Hommes FA (ed) Techniques in diagnostic human biochemical genetics: A laboratory mannual. Wiley-Liss, New York, pp 143-176
27. Tein I, Haslam RHA, Rhead WJ, Bennet MJ, Becker LE, Vockley J (1999) Short-chain acyl-CoA dehydrogenase deficiency: A cause of ophthalmoplegia and multicore myopathy. Neurology 52:366-372