GLB1 and skeletal dysplasia: This is the case of MPS IVB or Morquio B syndrome, in which there is a deficiency of beta-galactosidase-1, a lysosomal hydrolase that cleaves the terminal beta-galactose from ganglioside substrates and other glycoconjugates, and encoded by gen GLB1. [8] MPS IVB is an autosomal recessive disorder characterized by skeletal dysplasia and corneal clouding.