Figure 1.

Blocking of oligodendrocyte differentiation in the NPC. Specific markers allow identifying the differentiation status of cells of the oligodendrocyte lineage from progenitors (OPs) to myelinating OLs. The stage when the maturational block due to NPC mutation occurs is indicated. The markers indicated are: A2B5 and GD3 antigens, cell surface gangliosides; NG2, cell surface chondroitin sulfate proteoglycan; PDGFRα, platelet-derived growth factor receptor alpha; DM20, a splice variant of the proteolipid protein; CNP, 2′,3′-Cyclic-nucleotide 3′-phosphodiesterase; O₄ antigen, cell surface sulfatide; O₁, galactocerebroside; PLP: proteolipid protein; MBP, myelin basic protein; MAG, myelin-associated glycoprotein; MOG, myelin oligodendrocyte protein.