This correlated with a significant accumulation of the mitochondrial markers TOMM40 and cytochrome b-c1 complex subunit 1, (Cbc1cs1; encoded by UQCRC1), which increased 1.7- and 1.6-fold, respectively, in MPS IIIa mice compared with WT controls (Fig. 7, B and C). This evidence concerns the gene UQCRC1 and mucopolysaccharidosis.