Table 2.
Triads, mitochondria, Z line streaming and contractures in muscles from index patients and from relatives that tested positive for MH susceptibility..
| Biopsy | Age # sex | Family | Triad/IMF X 100* | Mitos/IMF X 100* | Mitos shapes ** | Mitos inner Architecture *** | Z line streaming No/fibers **, ## | Contractures No/fibers **, ## |
|---|---|---|---|---|---|---|---|---|
| D8 | 14 F | Relative | 37±13(10) | 35±18(10) | All small and Fragmented (12) | Clear matrix, variable cristae (22) | 2/10 | + 1/10 |
| C6S | 22M | Index case | 38±11(10) | 31±9(10) | Various or round (10) | Clear matrix, variable cristae (20) | + 2/8 | |
| A1E | 23M | Index case | 34±9(13) | 36±18(13) | Variable Few elongated (10) | Clear matrix, variable cristae (23) | 0 | 0 |
| B4E | 47F | Relative | 47±10(14) | 37±19(14) | Variable Few elongated (5) | Some clear matrix, variable cristae (19) | 2/14 | + 1/14 |
| F 12 | 53M | Index case $ | 37±17(9) | 35±24(9) | Variable Few elongated (14) | Mito clusters, odd shapes (23) | + 3/8 | ++ 5/8 |
| A2S | 55F | Relative | 54±14(6) | 34±11(6) | Few, mostly Round (10) | Occasional clear matrix, variable cristae (16) | + 3/5 | |
| I18 | 55 F | Relative | 29±12(7) | 35±20(7) | Fragmented (19) | Fragmented mitos, Cristae OK (26) | 3/10 | ++ 2/10 |
| K22 | 56M | Index case | 19±12(10) | 20±14(10) | Few, round (12) | Some clear matrix, variable cristae (22) | 7/10 | 3/10 |
| J20 | 57M | Relative | 27±14(8) | 39±17(8) | Variable Few elongated (12) | Clear matrix, variable cristae (20) | 2/10 | 2/10 |
| C7E | 59M | Relative | 54±11(15) | 46±20(15) | Elongated Numerous (5) | Normal cristae (15) | 0 | ++ 1/14 |
| H16 | 62M | Relative | 30±14(10) | 38± 17(10) | Mito clusters Unusual Variations(11) | Altered mitos (21) | ++ 1/10 | 0 |
| E10 | 68M | Relative | 51±14(9) | 39± 18(9) | Few, round Few long mito chains (21) | Occasional clear matrix, variable cristae (30) | 0 | 0 |
| M26 | 71M | Index case | 17±10(6) | 38± 21(6) | Variable Fragmented (10) | Normal cristae (16) | +++ 5/6 | 2/6 |
|
L24 (?) |
74M | Index case | ??? | ???? | Very few, small | ????? | 3/17 | 2/27 |
| G 14 | 79M | Relative | 40±13(8) | 23±17(8) | Variable, many Small (9) | Clear matrix, variable cristae (17) | 0 | 0 |
The numbers in parenthesis indicate the number of file profiles used for collecting the data
$ Homozygous
# Age at biopsy in years
* See Methods. Data were obtained from longitudinal sections only
** Obtained from cross sections only
*** Obtained from longitudinal and cross sections