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. 2018 Jul 31;19(8):2234. doi: 10.3390/ijms19082234

Table 2.

Contractile characteristics of dilated and other cardiomyopathy heart muscles.

Mutation, Gene Expression and Mutated (mut) Allele Expression Phosphorylation Level (Cardiomyopathy/Healthy) Titin Isoforms and Passive Stiffness (Cardiomyopathy/Healthy Maximal Force and Myofibril Density (Cardiomyopathy/Healthy) Contractile Kinetics Parameters (Cardiomyopathy/Healthy) Ca2-Sensitivity of Force (Cardiomyopathy/Healthy) Length Dependent Activation Changes upon Stretch (Cardiomyopathy/Healthy) Effect PKA on EC50 (in µM) Patient Information (Sex, Age and Number of Patients)
Expression Myocyte Myofibril Activation Relaxation +PKA +PKA
Diagnosis Gene with Mutation Mutation and Type: Truncated (T) or Not (NT) Gene Mut Protein TnI MyBP-C LC2 N2AB/
N2B		 Passive Stiffness F max Myofibril Density F max k ACT k TR t LIN k LIN k REL EC 50 n H EC 50 ΔEC50 ΔF ΔEC50 ΔEC50 Sex M/F Age N Refs
IDCM TTN p.(R23464Tfs *41) T 1.0 0 1.05 0.63 * 1.02 1.05 1.06 0.97 1.06 0.91 1.00 M 22 1 [76]
fDCM TTN p.(R23464Tfs*41) T 1.0 0 1.11 0.66 * 0.96 1.12 0.95 0.98 0.18 1.13 1.13 M 37 1 [76]
fDCM TTN p.(Y18923 *) T 0.96 0 1.25 0.62 * 1.04 1.15 1.16 * 0.98 1.06 1.17 1.03 F 22 1 [76]
PPCM TTN p.(K15664Vfs*13) T 1.85 0.4 F 1 [77]
DCM LMNA p.(R331Q) NT 0.5 1.98 * 1 0.64 * 0.63 0.86 * 1 0.78 M/F 45.3 3 [78,79]
fDCM RMB20 p.E913K NT 0.13 13.89 0.8 0.85 0.67 * 0.58 1.13 0.36 0.81 1.77 M 19 1 [80]
fDCM TNNC1 p.G159D NT 1.13 0.49 0.13 0.89 0.59 * 1 1.20 0.97 0.97 0.71 * 0.35 1.17 0.60 * 0.59 * 1.22 M 3 1 [76,81]
fDCM TNNI3 p.K36Q NT 1.0 0.58 * 1.10 1.17 1.05 0.70 * 1.39 1.30 * 1.34 M 15 1 [76]
IDCM TNNI3 p.(R98 *) T 0.39 0 1.08 1.56 1 0.95 0.94 * 0.88 * 0.66 M 46 1 [78,82]
IDCM TNNT2 p.(K217del) NT 0.51 1.08 1.64 1.3 * 1.11 1.07 0.97 M 19 1 [78]
fDCM MYH7 p.E1426K NT 1.08 0.65 * 0.97 1.13 1.19 * 0.70 * 0.76 1.35 * 0.96 M 43 1 [76]
IDCM not stated 0.54 1.98 1 0.98 0.94 1 0.73 M/F 54.6 5 [78]
DCM not stated 1.24 * 0.27 * 51–56 3–4 [83]
DCM not stated 0.5 0.51 1 0.96 3.37 M 45,59 2 [84]
IDCM not stated 0.35 * 0.58 * 1.62 * 1 0.94 0.69 * 1.12 0.76 1.09 8 [85]
IDCM not stated 0.4 * 0.4 * 1.1 1.18 0.88 0.67 1.33 0.8 1.26 M/F 52–62 7 [86]
IDCM not stated 1.78 1.2 1.34 0.68 * 0.81 0.87 0.59 0.86 1.33 3 [80]
IDCM not stated 1.14 F 41–57 6 [87]
PPCM not stated 0.24 * 0.61 * 1.58 1.39 * 0.84 0.65 * 1.01 0.48 * 0.96 F 6 [85]
ICM not stated 0.95 0.93 0.71 * 0.83 * 3 [88]
ICM not stated 0.5 0.72 * 0.98 2.13 M/F 41–65 7 [84]
ICM not stated 0.5 0.5 * 0.83 1 1.04 0.70 * 1.11 0.96 1.19 4 [85]
ICM (7) DCM (2) not stated 0.5 0.57 * 1.06 1.0 0.66 * 0.99 0.98 2.55 * M/F 41–65 10 [84]
HF not stated 0.33 * 0.65 * 1 0.53 * 0.53 * 0.96 0.63 * 0.87 0.98 M/F 42–57 4 [89]
IRCM not stated 0.84 1.20 * 0.05 * 0.52 * F 66 1 [90]
IRCM not stated 1.16 1.92 * 0.07 * 0.33 * M 21 1 [90]
IDCM (9) NCC (2) not stated 0.27 * 0.4–2.8 0.7 * 0.64 * 0.67 * 1 <18 11 [58]

The value changes were normalized to healthy control hearts (Cardiomyopathy/Healthy) if not otherwise specified. The parameters of length dependent activation are shown as a difference in EC50 and Fmax between long and short sarcomere length. Passive stiffness was evaluated as a Young’s modulus or as a passive tension generated by muscle at stretch. In some cases, approximate values are given. * p < 0.05. PPCM—peripartum cardiomyopathy, ICM—ischemic cardiomyopathy, RCM—restrictive cardiomyopathy, HF—heart failure, IDCM—idiopathic DCM, fDCM-familial DCM, IRCM—idiopathic restrictive cardiomyopathy. The table cells are highlighted according to the value changes compared to control: yellow—no changes, green—the value decreased, blue—the value increased.