Table 1.
Mechanical and kinetic parameters of myofibrils from the homozygous K280N cTnT HCM patient and different groups of control patients (top four rows of data) and impact of cTn exchange in K280N and donor myofibrils (bottom six rows of data)
Myofibril typeRTP0kACTDslowslow kRELfast kREL
 kN m−2 kN m−2 s−1 ms s−1 s−1 
Donors (N = 5) 10.4 ± 0.6 (n = 98) 111 ± 5 (n = 96) 0.85 ± 0.02 (n = 118) 186 ± 5 (n = 100) 0.29 ± 0.02 (n = 95) 4.18 ± 0.14 (n = 101) 
LVHao (N = 7) 9.8 ± 1.1 (n = 48) 96 ± 6 (n = 58) 0.71 ± 0.04 (n = 50) 248 ± 13 (n = 54) 0.20 ± 0.02 (n = 54) 3.15 ± 0.20 (n = 54) 
HCMsmn (N = 3) 8.2 ± 0.9 (n = 44) 87 ± 7 (n = 43) 0.74 ± 0.03 (n = 45) 206 ± 7 (n = 44) 0.30 ± 0.02 (n = 44) 4.62 ± 0.20 (n = 44) 
K280N (N = 1) 14.9 ± 1.4* (n = 43) 87 ± 5 (n = 43) 1.73 ± 0.07* (n = 39) 226 ± 11 (n = 43) 0.63 ± 0.05* (n = 42) 3.76 ± 0.16 (n = 42) 
Tn-exchanged batches       
K280N sham treated (N = 1) 15.9 ± 2.9 (n = 12) 89 ± 9 (n = 12) 1.69 ± 0.08 (n = 17) 240 ± 16 (n = 16) 0.69 ± 0.08 (n = 16) 3.90 ± 0.27 (n = 16) 
K280N WT Tn exch (N = 1) 15.7 ± 2.0 (n = 13) 86 ± 7 (n = 14) 1.00 ± 0.09++ (n = 16) 259 ± 27 (n = 15) 0.31 ± 0.05++ (n = 16) 3.13 ± 0.19+ (n = 16) 
Donors sham treated (N = 2) 10.5 ± 0.83 (n = 64) 114 ± 7 (n = 62) 0.86 ± 0.03 (n = 70) 173 ± 6 (n = 62) 0.28 ± 0.02 (n = 58) 4.26 ± 0.18 (n = 63) 
Donors WT Tn exch (N = 2) 10.1 ± 0.7 (n = 72) 91 ± 6+ (n = 63) 0.84 ± 0.03 (n = 70) 179 ± 8 (n = 59) 0.30 ± 0.02 (n = 66) 4.65 ± 0.19 (n = 65) 
Donor sham treated (N = 1) 10.8 ± 1.1 (n = 14) 94 ± 10 (n = 14) 0.81 ± 0.04 (n = 15) 214 ± 7 (n = 15) 0.33 ± 0.04 (n = 15) 3.78 ± 0.32 (n = 15) 
Donor K280N Tn exch (N = 1) 15.9 ± 1.9+ (n = 12) 77 ± 9 (n = 10) 1.04±0.08++ (n = 12) 186 ± 9+ (n = 12) 0.60 ± 0.07++ (n = 13) 4.18 ± 0.62 (n = 13) 
Myofibril typeRTP0kACTDslowslow kRELfast kREL
 kN m−2 kN m−2 s−1 ms s−1 s−1 
Donors (N = 5) 10.4 ± 0.6 (n = 98) 111 ± 5 (n = 96) 0.85 ± 0.02 (n = 118) 186 ± 5 (n = 100) 0.29 ± 0.02 (n = 95) 4.18 ± 0.14 (n = 101) 
LVHao (N = 7) 9.8 ± 1.1 (n = 48) 96 ± 6 (n = 58) 0.71 ± 0.04 (n = 50) 248 ± 13 (n = 54) 0.20 ± 0.02 (n = 54) 3.15 ± 0.20 (n = 54) 
HCMsmn (N = 3) 8.2 ± 0.9 (n = 44) 87 ± 7 (n = 43) 0.74 ± 0.03 (n = 45) 206 ± 7 (n = 44) 0.30 ± 0.02 (n = 44) 4.62 ± 0.20 (n = 44) 
K280N (N = 1) 14.9 ± 1.4* (n = 43) 87 ± 5 (n = 43) 1.73 ± 0.07* (n = 39) 226 ± 11 (n = 43) 0.63 ± 0.05* (n = 42) 3.76 ± 0.16 (n = 42) 
Tn-exchanged batches       
K280N sham treated (N = 1) 15.9 ± 2.9 (n = 12) 89 ± 9 (n = 12) 1.69 ± 0.08 (n = 17) 240 ± 16 (n = 16) 0.69 ± 0.08 (n = 16) 3.90 ± 0.27 (n = 16) 
K280N WT Tn exch (N = 1) 15.7 ± 2.0 (n = 13) 86 ± 7 (n = 14) 1.00 ± 0.09++ (n = 16) 259 ± 27 (n = 15) 0.31 ± 0.05++ (n = 16) 3.13 ± 0.19+ (n = 16) 
Donors sham treated (N = 2) 10.5 ± 0.83 (n = 64) 114 ± 7 (n = 62) 0.86 ± 0.03 (n = 70) 173 ± 6 (n = 62) 0.28 ± 0.02 (n = 58) 4.26 ± 0.18 (n = 63) 
Donors WT Tn exch (N = 2) 10.1 ± 0.7 (n = 72) 91 ± 6+ (n = 63) 0.84 ± 0.03 (n = 70) 179 ± 8 (n = 59) 0.30 ± 0.02 (n = 66) 4.65 ± 0.19 (n = 65) 
Donor sham treated (N = 1) 10.8 ± 1.1 (n = 14) 94 ± 10 (n = 14) 0.81 ± 0.04 (n = 15) 214 ± 7 (n = 15) 0.33 ± 0.04 (n = 15) 3.78 ± 0.32 (n = 15) 
Donor K280N Tn exch (N = 1) 15.9 ± 1.9+ (n = 12) 77 ± 9 (n = 10) 1.04±0.08++ (n = 12) 186 ± 9+ (n = 12) 0.60 ± 0.07++ (n = 13) 4.18 ± 0.62 (n = 13) 

Means ± SE; N, number of patients in the group; n, number of myofibrils; P0, maximal Ca2+-activated tension. For the statistics of the comparison between groups in the first four rows, see Supplemental materials; *, K280N parameters for which the statistical analysis shows a significant difference versus all control groups. For the statistics of the cTn exchange experiments (bottom six rows): +, P < 0.05; ++, P < 0.01 Student’s t test for unpaired observations (exchanged myofibrils vs. sham-treated unexchanged myofibrils).

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