L-Serine synthesis in the central nervous system: A review on serine deficiency disorders

The de nova synthesis of the amino acid L-serine plays an essential role in the development and functioning of the central nervous system (CNS). L-Serine displays many metabolic functions during different developmental stages; among its functions providing precursors for amino acids, protein synthesis, nucleotide synthesis, neurotransmitter synthesis and L-serine derived lipids. Patients with congenital defects in the L-serine synthesizing enzymes present with severe neurological abnormalities and underscore the importance of this synthetic pathway. In this review, we will discuss the cellular functions of the L-serine pathway, structure and enzymatic properties of the enzymes involved and genetic defects associated with this pathway. (C) 2009 Elsevier Inc. All rights reserved.