L-isoaspartate (D-aspartate) O-methyltransferase (EC 2.1.1.77)

 Striking accumulation of protein substrates for L-isoasparate (D-asperate) O-methyltransferase in the Cytosolic fraction of brain, heart, liver, and erythrocytes
 Significant growth retardation
 Fatal Seizures
 Fatality due to seizures, at an average, 42 days after birth

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