Table 1.

Biochemical data (absolute values)a

ParameterCSIIIIIIII + IIIIVCoQ10
Kidney cortex
    Patients
        116114233432274.5
        2290N/A46241.62243.7
    Controls
        P38830.634.014.03.416326
        P5013144.064.042.011.533894
        P9720167.678.374.717.0465180
    P (Mann-Whitney)NSN/ANSNS<0.03NS<0.04
Skeletal muscle
    patients
        135014.039.057.06.560312
        23567.146.054.04.72760.8
    Controls
        P327429.060.0101.120.990249.2
        P5019528.033.046.018.055628.2
        P9714017.125.329.87.836317.6
    P (Mann-Whitney)<0.03NSNSNS<0.05NS<0.02
  • a Biochemical measurements in renal cortex and skeletal muscle homogenates. Complexes I through IV and citrate synthase (CS) values are expressed in nmol/min per mg protein. CoQ10 values are expressed in μ mol/g tissue. N/A, not available).