Since GlyRα2 is downregulated and replaced mainly by GlyRs containing α1β after birth in the spinal cord, GlyRα1 gain-of-function mutations leading to a temporally enhanced intracellular chloride ion concentration may reduce the overall number and size of the synaptically expressed α1-containing GlyRs, resulting in SD symptoms. Here, GARS1 is linked to Salla disease.