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Table 1 Relative expression of proteins of energy metabolism in muscle biopsies of neuromuscular diseases

From: Quantitative analysis of proteins of metabolism by reverse phase protein microarrays identifies potential biomarkers of rare neuromuscular diseases

   Rare Diseases  
   Metabolic   Muscular Dystrophies    Neurodegenerative Intesive-Care Unit myopathy
  Controls Mitochondrial Glycogenosis DMD BMD C. Xp21 LGMD 2C NCL ICU
   Complex I type V       
Mitochondrial Markers          
PDH E1α 1.0 ± 0.1 1.3 ± 0.1 0.6 ± 0.1 1.3 ± 0.2 1.3 ± 0.4 0.7 ± 0.1 0.8 ± 0.1 0.7 ± 0.1 0.6 ± 0.1
SDH 1.0 ± 0.1 1.4 ± 0.1 ** 0.9 ± 0.1 1.4 ± 0.2 * 1.1 ± 0.2 0.9 ± 0.2 0.6 ± 0.1 * 1.0 ± 0.2 0.8 ± 0.1
CS 1.0 ± 0.1 1.5 ± 0.2 * 0.8 ± 0.1 1.1 ± 0.3 0.8 ± 0.1 1.4 ± 0.4 0.5 ± 0.2 1.1 ± 0.3 1.2 ± 0.1
Hsp60 1.0 ± 0.1 1.3 ± 0.1 0.8 ± 0.1 1.7 ± 0.2 ** 1.2 ± 0.2 0.9 ± 0.2 1.3 ± 0.3 0.9 ± 0.2 0.8 ± 0.1
CPT1-M 1.0 ± 0.1 0.9 ± 0.1 1.0 ± 0.1 1.2 ± 0.2 0.7 ± 0.0 * 0.9 ± 0.0 0.7 ± 0.2 1.0 ± 0.1 1.0 ± 0.1
HADHA 1.0 ± 0.1 1.6 ± 0.2 * 1.1 ± 0.2 0.7 ± 0.1 0.7 ± 0.1 0.8 ± 0.2 0.8 ± 0.3 1.1 ± 0.2 1.4 ± 0.2
NADH-9 1.0 ± 0.1 1.8 ± 0.2 ** 1.2 ± 0.1 0.9 ± 0.1 0.9 ± 0.2 1.0 ± 0.2 0.6 ± 0.2 1.0 ± 0.1 1.2 ± 0.2
COX-1 1.0 ± 0.1 1.2 ± 0.1 0.6 ± 0.1 * 1.5 ± 0.1 * 1.3 ± 0.3 0.9 ± 0.1 0.9 ± 0.2 0.8 ± 0.1 0.7 ± 0.1
α-F1 1.0 ± 0.1 1.1 ± 0.1 0.7 ± 0.2 1.4 ± 0.1 * 1.3 ± 0.5 0.8 ± 0.1 1.0 ± 0.2 1.1 ± 0.2 0.7 ± 0.1
β-F1 1.0 ± 0.1 1.3 ± 0.1 * 0.9 ± 0.1 1.2 ± 0.2 1.2 ± 0.1 1.2 ± 0.3 0.8 ± 0.1 0.8 ± 0.1 1.0 ± 0.1
IF1 1.0 ± 0.1 1.0 ± 0.1 0.7 ± 0.1 1.1 ± 0.2 1.1 ± 0.2 0.7 ± 0.1 0.8 ± 0.2 0.7 ± 0.1 * 0.6 ± 0.1 *
SOD2 1.0 ± 0.1 0.8 ± 0.1 1.0 ± 0.1 0.5 ± 0.1 * 0.5 ± 0.1 * 0.7 ± 0.2 0.8 ± 0.3 0.8 ± 0.1 1.0 ± 0.1
Cytoplasmic Markers          
β-Actin 1.0 ± 0.1 1.1 ± 0.1 1.3 ± 0.2 1.0 ± 0.1 1.1 ± 0.3 1.0 ± 0.1 0.8 ± 0.2 1.1 ± 0.1 1.2 ± 0.2
GAPDH 1.0 ± 0.1 0.6 ± 0.1 * 0.8 ± 0.1 0.4 ± 0.1 * 0.9 ± 0.3 0.6 ± 0.1 0.3 ± 0.1 * 0.6 ± 0.1 0.5 ± 0.1 *
PK 1.0 ± 0.1 1.0 ± 0.1 0.8 ± 0.1 0.8 ± 0.2 1.0 ± 0.2 0.8 ± 0.0 0.7 ± 0.1 0.6 ± 0.1 * 0.7 ± 0.1 *
LDH-A 1.0 ± 0.1 0.7 ± 0.1 0.6 ± 0.2 0.4 ± 0.1 * 0.6 ± 0.2 0.5 ± 0.0 0.3 ± 0.0 * 0.4 ± 0.1 * 0.4 ± 0.1 *
GPD1 1.0 ± 0.1 1.1 ± 0.1 0.9 ± 0.1 0.4 ± 0.1 ** 0.7 ± 0.2 0.6 ± 0.1 * 0.6 ± 0.2 * 0.5 ± 0.1 * 0.7 ± 0.1
PYGM 1.0 ± 0.1 1.5 ± 0.2 * 0.0 ± 0.0 ** 0.2 ± 0.1 ** 0.7 ± 0.3 0.7 ± 0.1 0.5 ± 0.1 * 0.5 ± 0.1 * 0.4 ± 0.1 **
ACO1 1.0 ± 0.1 1.1 ± 0.1 0.9 ± 0.1 0.8 ± 0.1 1.1 ± 0.3 0.9 ± 0.2 0.7 ± 0.2 1.0 ± 0.1 0.7 ± 0.1
Ratios          
β-F1/GAPDH 1.0 ± 0.1 2.5 ± 0.3 ** 1.1 ± 0.2 3.1 ± 0.8 ** 2.2 ± 1.0 * 1.6 ± 0.4 2.9 ± 0.6 ** 1.3 ± 0.3 1.7 ± 0.3 *
BEC Index 1.0 ± 0.1 2.1 ± 0.2 ** 1.4 ± 0.2 1.6 ± 0.3 * 1.8 ± 0.5 * 1.9 ± 0.2 * 2.5 ± 0.5 ** 1.9 ± 0.5 * 2.5 ± 0.5 **
β-F1/LDH-A 1.0 ± 0.1 2.2 ± 0.3 ** 1.8 ± 0.5 * 3.5 ± 0.6 ** 2.7 ± 0.8 ** 1.9 ± 0.4 * 2.5 ± 0.5 ** 1.8 ± 0.3 ** 3.2 ± 0.7 **
  1. The table summarizes the expression of nineteen proteins involved in different mitochondrial and cytoplasmic activities of metabolism and of the ratios derived from them in different muscle myopathies when compared to controls. Values are expressed as fold of control. The results shown are the mean values ± S.E.M. *, p < 0.05 and **, p < 0.001 when compared to controls.