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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.
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Journal of Translational Medicine

ISSN: 1479-5876

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