Figure 2
From: An immune deficient mouse model for mucopolysaccharidosis IIIA (Sanfilippo syndrome)

Cytoplasmic microvesiculation affecting epithelia, macrophages and neurons is consistent with a lysosomal storage disorder in 8-month-old MPSIIIA-TKO mice SGSH−/−. Fine clear vacuoles (black arrows) distend the cytoplasm of neurons (A), macrophages (C) and Kupffer cells (E). Additionally, there is increased space between axons in the sciatic nerve (C) that could correspond with dilation of myelin sheaths. Images of control tissues (B,D,F) from the MPSIIIA-TKO SGSH+/+ littermates are provided for comparison.