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Rabinovich-Nikitin I, et al

The severe neonatal-onset form is far more common and manifests almost immediately after birth

Carbohydrates and fats are converted into energy

Maillard reaction (MR) and maillard reaction products (MRP) MR, also known as Maillard conjugation or glycation , is a non-enzymatic process that forms covalent bonds between the NH 2 group of AAs and the carbonyl (C = O) group of reduced sugars 618

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