Table 1. The degree of chimerism correlates with the degree of corrections.
WT | chim10–30 | chim5–10 | chim<5 | mdx | |
Xgal staining (% of cells or skeletal muscle fibers) | |||||
heart | 0 | 10–30 | <5 | <5 | 0 |
liver, lung, brain | 0 | 5–30 | <5 | UD | 0 |
quadriceps | 0 | 50–70 | <5 | UD | 0 |
pectoralis | 0 | 50–90 | 10–30 | <5 | 0 |
diaphragm | 0 | 20–50 | UD | UD | 0 |
Dystrophin (WB, % relative to WT) | |||||
heart | 100 | 10–30 | 5–10 | <5 | 0 |
quadriceps | 100 | 60–100 | <5 | 0 | 0 |
pectoralis | 100 | 70–100 | 5–10 | 0 | 0 |
diaphragm | 100 | 30–60 | 10–15 | <5 | 0 |
Fiber cross-sectional area (µm2) | |||||
diaphragm (mode) | 500 | 500 | 400 | 400 | 300 |
Central nucleation (%) | |||||
quadriceps, TA, pectoralis | <10 | 10–30 | ND | 60–90 | 60–90 |
diaphragm | <5 | <10 | 20–40 | 25–45 | 20–40 |
Muscle thickness (µm) | |||||
diaphragm | 160–180 | 180–250 | 540–600 | 550–650 | 450–550 |
Fiber count across width | |||||
diaphragm | 6–10 | 5–10 | 8–13 | 12–18 | 18–24 |
Mononuclear invasion* | |||||
dorsal diaphragm, pectoralis, TA | − | ++ | +++ | ++++++ | ++++++ |
Dystrophin glycoprotein complex (IMF)(dbv-α, synt, nNOS, SG-β) | |||||
diaphragm | ++++++ | ++++++ | +++ | ++ | ++ |
Sarcolemmal utrophin (IMF) | |||||
diaphragm | − | ++ | ND | ++++++ | ++++++ |
eMHC (IMF, fold increase by microarrays) | |||||
diaphragm, pectoralis | − | ++, UC | ++++, ND | ++++++, ND | ++++++, 24 |
Mechanical stress (g/mm2) | |||||
EDL (tetanus) | 46.4±2.5** | 42.8±3.6 | ND | 22.2±2.2 | 20.0±7.9** |
EDL (twitch) | 6.9±0.7** | 7.0±0.8 | ND | 4.8±0.4 | 5.2±2.0** |
chim10–30: chimeric mice that incorporated between 10 and 30% of ESCs (n = 9); chim5–10 (n = 2); chim<5 (n = 12); WT (n>10); mdx (n>10).
UC: unchanged; UD: undetectable; ND: not determined; IMF: immunofluorescence.
dbv-α: dystrobrevin-α; syntrophin: synt; neuronal nitric oxide synthase: nNOS; sarcoglycan β: SG-β.
++low degree; ++++++ high degree.
WT and mdx twitch and tetanic stress values are unpublished data from A.Wolff's dissertation (Virginia Polytechnic Institute and State University, 2007).