The sphingolipidoses, as a class, are most similar to which one of the following disorders?
ExplanationThe sphingolipidoses and I-cell disease are both lysosomal storage diseases, whereas the other disorders listed do not involve lysosomal dysfunction. Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke (MELAS) is a mitochondrial disorder, and Zellweger’s is a disorder of peroxisomal biogenesis. G6PDH (glucose-6-phosphate dehydrogenase) deficiency and von Gierke disease are single gene mutations which do not alter lysosomal function (although type II glycogen storage disease, Pompe disease, is a lysosomal storage disease).
