Toxic amino acids

Toxic amino acids refer to a diverse group of amino acids that exert harmful effects on biological systems, either as exogenous toxins or endogenous metabolites that accumulate to pathological levels (Cox et al., 2016). This category includes non-proteinogenic amino acids (NPAAs) like beta-methylamino-L-alanine (BMAA), which is linked to neurodegenerative diseases, and endogenous excitotoxins like glutamate, which can cause neuronal death through overstimulation of NMDA and AMPA receptors (Lau & Tymianski, 2010). While not a single therapeutic target, these molecules are the focus of drug development aimed at mitigating their effects through receptor antagonism, metabolic enzyme replacement, or sequestration. For example, drugs like riluzole modulate glutamate levels to treat ALS, while pegvaliase degrades phenylalanine in patients with phenylketonuria (Blau et al., 2010). Understanding the transport and signaling pathways of these molecules is critical for developing neuroprotective and metabolic therapies. They are often studied in the context of environmental exposure and inborn errors of metabolism (Spencer et al., 2001).

Other names

Non-proteinogenic amino acidsExcitotoxic amino acidsNeurotoxic amino acidsNPAAsExcitotoxins

Antagonism of ionotropic glutamate receptors, inhibition of neurotransmitter release, and enzymatic degradation of toxic metabolites (Lau & Tymianski, 2010; Blau et al., 2010).