Inhibition by methylmalonate of glycine uptake by synaptosomes from rat spinal cord.
Methylmalonate is accumulated in the genetically linked deficiency of methylmalonyl-CoA mutase (methylmalonic acidemia). In this condition is also observed an elevation of the glycine levels. This communication reports the inhibition of the synaptosomal glycine uptake by methylmalonate, when present at similar concentrations to those found in methylmalonic acidemia. This inhibition could be responsible, at least in part, for the neurological damage characteristic of this disease, by increasing the glycine levels in the synaptic cleft and thus interfering with the normal function of the inhibitory glycinergic synapsis in the spinal cord.
D000592:Amino Acid Metabolism, Inborn Errors / Q000378:metabolism; D000818:Animals; D005998:Glycine / Q000378:metabolism*; D008314:Malonates / Q000494:pharmacology*; D008764:Methylmalonic Acid / Q000494:pharmacology*; D008765:Methylmalonyl-CoA Mutase / Q000172:deficiency; D018377:Neurotransmitter Agents / Q000378:metabolism; D051381:Rats; D013116:Spinal Cord / Q000378:metabolism*; D013574:Synaptosomes / Q000378:metabolism*
D008314:Malonates; D018377:Neurotransmitter Agents; D008764:Methylmalonic Acid; D008765:Methylmalonyl-CoA Mutase; D005998:Glycine