Table 1.
Chronic Na+ overload of DMD patient muscles and mdx SMFs
#Reference[Na+]iNotes
Horvath et al., 1955  [Na+]i higher (and [K+]i lower) in dystrophic than in normal muscle fibers Horvath et al. (1955) is referenced in Rudman et al. (1972); biopsy, elemental analysis; fibers of 20 patients with unspecified muscular dystrophies. 
Dunn et al., 1993  Diaphragm, gastrocnemius control: 13.0, 13 mdx: 23.5, 24 Two techniques: Na+ electrode for diaphragm, cyto-volumetrics plus serum and bulk muscle Na+ values for gastrocnemius. Mice. 
Hirn et al., 2008  1.4× more 22Na+ uptake in mdx than in control 22Na+ uptake. Tetrodotoxin reduces uptake in both and makes them identical (see their Fig. 1). Mice. 
Miles et al., 2011  Control: 11.5 mM; mdx: 22.5 mM Na+-dye measurements. Mice. 
Weber et al., 2011, 2012  Total sodium content: volunteers: 25–26 mM; DMD patients: 38 mM 23Na-MRI. First noninvasive observation of chronic Na+ overload; German DMD patient cohort. Authors wondered if ↑myoplasmic Na+ might signify cytotoxic osmotic Na+ loading…but see 10. 
Lehmann-Horn et al., 2012  Sustained small decrease of cytoplasmic Na+ (and H2O) overload (n = 1) 23Na-MRI, pilot study, prolonged off-label eplerenone treatment, one 22-yr-old female DMD patient. 
Altamirano et al., 2014  Vastus lateralis control: 8 mM; mdx: 18 mM Na+ electrodes. Shear-stress stimulation of NO pathway reduced mdx [Na+]i to 10 mM without altering control level (see their Fig. 1 B). Mice. 
Burr et al., 2014  Control: 5.3 mM; mdx: 7.3 mM Na+ electrodes (see their Fig. 10). Mice. 
Glemser et al., 2017  ∼20% drop in muscle Na+ overload (n = 1) 23Na-MRI, pilot study, 6 mo off-label eplerenone treatment, one 7-yr-old male DMD patient (see their Table 1). 
10 Gerhalter et al., 2019  Total sodium content: volunteers: 16.5 mM; DMD patients: 26 mM 23Na-MRI, French DMD patient cohort. Na+ overload regularly observed in the absence of water T2 increases; this is, therefore, nonosmotic Na+ loading (see also 9). 
#Reference[Na+]iNotes
Horvath et al., 1955  [Na+]i higher (and [K+]i lower) in dystrophic than in normal muscle fibers Horvath et al. (1955) is referenced in Rudman et al. (1972); biopsy, elemental analysis; fibers of 20 patients with unspecified muscular dystrophies. 
Dunn et al., 1993  Diaphragm, gastrocnemius control: 13.0, 13 mdx: 23.5, 24 Two techniques: Na+ electrode for diaphragm, cyto-volumetrics plus serum and bulk muscle Na+ values for gastrocnemius. Mice. 
Hirn et al., 2008  1.4× more 22Na+ uptake in mdx than in control 22Na+ uptake. Tetrodotoxin reduces uptake in both and makes them identical (see their Fig. 1). Mice. 
Miles et al., 2011  Control: 11.5 mM; mdx: 22.5 mM Na+-dye measurements. Mice. 
Weber et al., 2011, 2012  Total sodium content: volunteers: 25–26 mM; DMD patients: 38 mM 23Na-MRI. First noninvasive observation of chronic Na+ overload; German DMD patient cohort. Authors wondered if ↑myoplasmic Na+ might signify cytotoxic osmotic Na+ loading…but see 10. 
Lehmann-Horn et al., 2012  Sustained small decrease of cytoplasmic Na+ (and H2O) overload (n = 1) 23Na-MRI, pilot study, prolonged off-label eplerenone treatment, one 22-yr-old female DMD patient. 
Altamirano et al., 2014  Vastus lateralis control: 8 mM; mdx: 18 mM Na+ electrodes. Shear-stress stimulation of NO pathway reduced mdx [Na+]i to 10 mM without altering control level (see their Fig. 1 B). Mice. 
Burr et al., 2014  Control: 5.3 mM; mdx: 7.3 mM Na+ electrodes (see their Fig. 10). Mice. 
Glemser et al., 2017  ∼20% drop in muscle Na+ overload (n = 1) 23Na-MRI, pilot study, 6 mo off-label eplerenone treatment, one 7-yr-old male DMD patient (see their Table 1). 
10 Gerhalter et al., 2019  Total sodium content: volunteers: 16.5 mM; DMD patients: 26 mM 23Na-MRI, French DMD patient cohort. Na+ overload regularly observed in the absence of water T2 increases; this is, therefore, nonosmotic Na+ loading (see also 9). 

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