A child with recurrent ear infections, failure to thrive, developmental delay, coarse facial features, restricted joint mobility, corneal clouding. Mass spectrometry shows deficient phosphorylation of mannose residues on glycoproteins. What is the dx? & Normally, where should these proteins go to?
a) Dx: I-cell disease; Normally lysosomes
b) Dx: Cystic fibrosis; Normally Golgi apparatus
c) Dx: Gaucher disease; Normally lysosomes
d) Dx: Von Gierke disease; Normally peroxisomes