Reduction of gangliosides, phospholipids and cholesterol content in cerebral cortex of rats caused by chronic hypermethioninemia.
Neurological dysfunction is observed in patients with severe hypermethioninemia, whose physiopathology is still poorly understood. In the current study we investigated the effect of chronic administration of methionine on the content and species of gangliosides and phospholipids, as well as on the concentration of cholesterol in rat cerebral cortex. Wistar rats received subcutaneous injections of methionine (1.34-2.68 micromol/g of body weight), twice a day, from the 6th to the 28th day of age and controls received saline. Animals were killed 12h after the last injection. Results showed that methionine administration significantly decreased the total content of lipids in cerebral cortex of rats. We also observed that this amino acid significantly reduced the absolute quantity of the major brain gangliosides (GM1, GD1a, GD1b and GT1b) and phospholipids (sphingomyelin, phosphatidylcholine and phosphatidylethanolamine). We also showed that Na+,K+-ATPase activity and TBARS were changed in cerebral cortex of rats subjected to hypermethioninemia. If confirmed in human beings, these data could suggest that the alteration in lipid composition, Na+,K+-ATPase activity and TBARS caused by methionine might contribute to the neurophysiopathology observed in hypermethioninemic patients.
Stefanello
Francieli M
FM
Departamento de Bioquímica, ICBS, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos, 2600-Anexo, CEP 90035-003, Porto Alegre, RS, Brazil.
Kreutz
Fernando
F
Scherer
Emilene B S
EB
Breier
Ana C
AC
Vianna
Luciene P
LP
Trindade
Vera M T
VM
Wyse
Angela T S
AT
D000818:Animals; D002540:Cerebral Cortex / Q000378:metabolism*; D002784:Cholesterol / Q000378:metabolism*; D002855:Chromatography, Thin Layer; D005260:Female; D005732:Gangliosides / Q000378:metabolism*; D007202:Indicators and Reagents; D050356:Lipid Metabolism / Q000187:drug effects; D015227:Lipid Peroxidation / Q000187:drug effects; D008715:Methionine / Q000097:blood / Q000633:toxicity*; D010743:Phospholipids / Q000378:metabolism*; D011247:Pregnancy; D051381:Rats; D017208:Rats, Wistar; D000254:Sodium-Potassium-Exchanging ATPase / Q000378:metabolism; D013570:Synaptic Membranes / Q000187:drug effects / Q000378:metabolism; D017392:Thiobarbituric Acid Reactive Substances / Q000378:metabolism
D005732:Gangliosides; D007202:Indicators and Reagents; D010743:Phospholipids; D017392:Thiobarbituric Acid Reactive Substances; D002784:Cholesterol; D008715:Methionine; D000254:Sodium-Potassium-Exchanging ATPase