Folate and vitamin B12 are essential cofactors for the methionine/homocysteine cycle in the
brain. These vitamins mediate the remethylation of homocysteine (HCY), which affects the …

To date, inborn errors of neurotransmitters have been defined based on the classic concept
of inborn error of metabolism (IEM), and they include defects in synthesis, catabolism, and …

Thiamine (vitamin B1) is co-factor for three pivotal enzymes for glycolytic metabolism:
pyruvate dehydrogenase, α-ketoglutarate dehydrogenase, and transketolase. Thiamine …

Elevated plasma homocysteine levels have been identified as a significant, independent risk
factor for the development of cognitive decline including Alzheimer's disease. While several …

Folates, in the form of tetrahydrolates, serve as cofactors for enzymes in a biological network
of metabolic pathways that are necessary for the de novo synthesis of purines, thymidylate …

Vitamin B12 and folate are supplied via two major pathways, the conversion of
homocysteine to methionine and the conversion of methyl malonyl coenzyme A to succinyl …

In the brain, neurons and glial cells are equipped with a number of acid–base‐coupled ion
and metabolite transporters, which affect intra‐and extracellular pH. The pH changes can be …

CBS is a vitamin B6-dependent transsulfuration enzyme needed to synthesize cysteine from
methionine, catalyzing the condensation of serine with homocysteine to form cystathionine …

More than 300 new inborn errors or metabolism (IEM) have been described during the last 5
years and most of them have major neurological symptoms (Ferreira et al. 2018; Saudubray …

Homocysteine is a sulfur-containing amino acid that is not synthesized de novo in humans,
but is formed in the organism during the metabolism of the essential amino acid methionine …