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. 2009 Aug 31;132(11):3165–3174. doi: 10.1093/brain/awp221

Figure 3.

Figure 3

(A) Northern blot for mt-tRNAGlu showed severely decreased steady-state levels in skeletal muscle of Patient 11 at 1 month of age and slightly higher, but still significantly decreased levels, after clinical recovery at 9 years of age, as well as in muscle biopsies of her asymptomatic mother (M). Data from skeletal muscle are not shown for Patients 7 and 14. Myoblasts of Patient 14 showed steady-state levels similar to late biopsies and to biopsies of a healthy mother. Patient mt-tRNAGlu steady-state levels normalized to mt-tRNALeu(UUR) levels are expressed relative to normalized control levels. (B) Immunoblotting showed decreased levels of the mitochondrial COXI, COXII and complex I (NDUFB8) subunits in the biopsy of Patient 11 at 1 month of age, which returned to normal at 9 years of age, and was also normal in her asymptomatic mother. The same subunits were slightly increased in myoblasts of Patient 14.