Mucopolysaccharidosis type I (MPS I) is an autosomal-recessive lysosomal storage disease (LSD) caused by a deficiency of the enzyme α-L-iduronidase (IDUA), involved in the degradation of two glycosaminoglycans (GAGs), dermatan (DS) and heparan sulfate (HS) [1]. Here, MAP3K14 is linked to Scheie syndrome.