Hypotaurine (2-aminoethane sulinic acid) is the immediate precursor of taurine (2-aminoethane sulfonic acid) which is one of the most abundant free amino acids in the central nervous system. Although hypotaurine and taurine are mainly supplied from blood plasma across the blood-brain barrier in the brain, they are also synthesized from cysteine and cysteamine by astrocytes and neurons. Hypotaurine acts as an endogenous neurotransmitter via action on the glycine receptors. It was shown, that hypotaurine might be used as an effective therapeutics for orofacial pain. Also in vitro studies were conducted to show that hypotaurine activates hypoxia signaling through the competitive inhibition of prolyl hydroxylase domain-2. Moreover, was made a suggestion, that the hypotaurine metabolic pathway may provide a potential new target for glioblastoma diagnosis and therapy.