L-Serine-13C3 is intended for use as an internal standard for the quantification of L-serine by GC- or LC-MS. L-Serine is a non-essential amino acid.1 It is produced from 3-phosphoglycerate in a multistep biosynthesis catalyzed by phosphoglycerate dehydrogenase (PGDH), phosphoserine aminotransferase (PSAT), and phosphoserine phosphatase (PSP). L-Serine can also be derived from dietary sources, as well as protein and phospholipid turnover. Defective L-serine biosynthesis is associated with neural tube defects and deoxysphingolipid accumulation in patients with Neu-Laxova syndrome, a rare disease characterized by a mutation in PGDH.1,2 Dysregulation of L-serine phosphorylation is associated with disease progression in patients with neurodegenerative diseases, including Alzheimer’s disease and amyotrophic lateral sclerosis.
1.Holm, L.J., and Buschard, K.L-serine: A neglected amino acid with a potential therapeutic role in diabetesAPMIS127(10)655-659(2019) 2.Metcalf, J.S., Dunlop, R.A., Powell, J.T., et al.L-serine: A naturally-occurring amino acid with therapeutic potentialNeurotox. Res.33(1)213-221(2018)