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

The de novo 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.

L. Tabatabaie, L.W. Klomp, R. Berger, T.J. de Koning
Authors from the NMC: 
2010; 99 (3): 256-262
Published in: 
Molecular Genetics & Metabolism
Date of publication: 
March, 2010
Status of the publication: