The glycine receptor (GLAR1) is a neurotransmitter-gated ion channel. Binding of glycine to GLAR1 increases the chloride conductance and thus produces hyperpolarization (inhibition of neuronal firing). Defects in GLRA1 are a cause of startle disease (STHE), also known as hereditary hyperekple, Xenopus/Amphibian,ia or congenital stiff-person syndrome. STHE is a genetically heterogeneous neurologic disorder characterized by muscular rigidity of central nervous system origin,Porcinearticularly in the neonatal period, and by an e, Xenopus/Amphibian,aggerated startle response to une, Xenopus/Amphibian,pected acoustic or tactile stimuli.