A huge body of literature represents that mutations in the cholinergic receptor nicotinic gamma subunit (CHRNG) gene lead to autosomal recessive MPS (6).The acetylcholine receptor (AChR), playing role as an excitatory cation channel, is a member of the superfamily of transmitter-gated ion channels with a critical role in controlling electrical signals between nerves and skeletal muscle cells acting through opening and closing a membrane-spanning pore (7). Here, CHRNG is linked to mucopolysaccharidosis.