Table 1.
Summary of changes in skeletal muscle satellite cells, MRFs, mitochondria and microRNAs.
| Disease progression in SODG93A mice | ALS patients | ||||
|---|---|---|---|---|---|
| P40 | P60 | P90 | P120 | ||
| Satellite cells | ↓ In satellite cell number both in slow and fast fibers (Manzano et al., 2013) | ↑ In satellite cell number in slow fibers and ND in satellite cell number in fast fibers (Manzano et al., 2013) | ↓ In satellite cell number in slow fibers and ↑ in satellite cell number in fast fibers (Manzano et al., 2013) | ↑ In satellite cell number in slow fibers and ND in satellite cell number in fast fibers (Manzano et al., 2013) | Muscle tissue: ND in satellite cell number ↑ Co staining of MyoG/Pax7 ↓ Co-staining of MyoD/Pax7, Myf5. (Pradat et al., 2011) |
| MRFs | ND in Pax7, Myf5, Myod1 and Myog mRNA. ND in Pax7, MYF5, MYOD1, MYOG protein (Manzano et al., 2013) | ↑ Pax7 and Myog mRNA. ↑ MYF5 protein (Manzano et al., 2013) |
↑ Myf5, Myod1 and Myog mRNA. ↑ Pax7, MYF5, MYOD1 protein (Manzano et al., 2013) |
↑ Pax7, Myf5, Myod1 and Myog mRNA. ND in Pax7, MYF5, MYOD1 and MYOG protein (Manzano et al., 2013) |
Myoblast cultures: ↑ MyoD mRNA, ND Pax7 mRNA, (Scaramozza et al., 2014). Myotube cultures: ↓ MyoG protein (Scaramozza et al., 2014) |
| Mitochondria | ↓ PGC-1a, NRF-1, Tfam, mnSOD mRNA. ND in PGC-1a, NRF-1, Tfam, mnSOD, AChRε protein (Thau et al., 2012) | ↓ migration rate of mt-PAGFP (↓ mt-fusion and fission). ND in mt-morphology. Mt-membrane depolarization. Fragmentation of the mt-network (Dupuis et al., 2004; Faes and Callewaert, 2011; Bozzo et al., 2016) | ↓ PGC-1a, PGC-1β, ERRa, NRF-1, Mfn1, Mfn2 and COXIV mRNA (Russell et al., 2012) ↓ PGC-1a and Utrn mRNA. ND in PGC-1a, NRF-1, Tfam, mnSOD, AChRε protein (Thau et al., 2012) |
↓ PGC-1a, PGC-1β, ERRa, NRF-1, Mfn1, Mfn2 and COXIV mRNA (Russell et al., 2012) ↓ PGC-1a, NRF-1, Tfam, mnSOD mRNA. ↓ NRF-1 protein (Thau et al., 2012) |
↓ NADH:CoQ oxidoreductase, cytochrome c oxidase (COX), mitochondrial DNA and mitochondria Mn-SOD (Wiedemann et al., 1998; Vielhaber et al., 2000) ↓ PGC-1a, PGC-1β, ERRa, NRF-1, Mfn1, Mfn2 and COXIV mRNA. ↓ PGC-1a, Mfn1 and COXIV protein. ND in Mfn2 and NRF-1. ↓ CS and COX activity (Russell et al., 2012) ↓ PGC-1a, NRF-1, NRF-2, Utrn mRNA. ND in PGC-1a, NRF-1, Tfam, mnSOD protein (Thau et al., 2012) |
| miRNAs | ND in miR-206 (at post-natal age of 1 month) (Williams et al., 2009) | ↑ miR-206 (at post-natal age of 8 months). ND in miR-206 (at post-natal of 5 months) (Williams et al., 2009) |
↑ miR-23a,-29b,-31,-206,-455. (Russell et al., 2012) | ||
P40, post-natal age of 40 days; MRF, myogenic regulatory factor; ND, no significant difference compared to control group; mt, mitochondrial.