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, 99 (3), 256-62

L-serine Synthesis in the Central Nervous System: A Review on Serine Deficiency Disorders


L-serine Synthesis in the Central Nervous System: A Review on Serine Deficiency Disorders

L Tabatabaie et al. Mol Genet Metab.


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.

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