Table 1.
Distribution of a representative set of pathogenic missense substitution sites in troponin
Troponin subunit or subunit regionSize of region (aa)Sites with pathogenic substitutionDensity of sites with pathogenic substitution (%)P vs. troponinP vs. TnI-TnTSites without pathogenic substitutionSites without pathogenic and with VUS substitutionDensity of sites without pathogenic and with VUS substitution (%)P vs. TnI-TnT
Troponin 
All TnI, TnT, TnC 659 52 7.9   607 44 7.2  
All except TnC TnI,TnT 498 51 10.2   447 41 9.2  
Unseen by cryo-EM (see legend) 190 11 5.8  0.0064 179 12 6.7 NS 
TnI 
All 1–210 210 26 12.4  NS 184 19 10.3 NS 
N 2/3 1–136 136 2.2  0.0001 133 6.8 NS 
C 1/3 137–210 74 23 31.1  <0.0001 51 10 19.6 NS 
TnT 
All 1–288 288 25 8.7  NS 263 22 8.4 NS 
Cryo-EM tail helix 87–150 64 10 15.6  NS 54 11.1 NS 
TnC 
All 1–161 161 0.6 <0.0001  160 1.9  
N-lobe 1–85 85 0.0010  85  
C-lobe 86–161 76 0.012  75 4.0  
Troponin subunit or subunit regionSize of region (aa)Sites with pathogenic substitutionDensity of sites with pathogenic substitution (%)P vs. troponinP vs. TnI-TnTSites without pathogenic substitutionSites without pathogenic and with VUS substitutionDensity of sites without pathogenic and with VUS substitution (%)P vs. TnI-TnT
Troponin 
All TnI, TnT, TnC 659 52 7.9   607 44 7.2  
All except TnC TnI,TnT 498 51 10.2   447 41 9.2  
Unseen by cryo-EM (see legend) 190 11 5.8  0.0064 179 12 6.7 NS 
TnI 
All 1–210 210 26 12.4  NS 184 19 10.3 NS 
N 2/3 1–136 136 2.2  0.0001 133 6.8 NS 
C 1/3 137–210 74 23 31.1  <0.0001 51 10 19.6 NS 
TnT 
All 1–288 288 25 8.7  NS 263 22 8.4 NS 
Cryo-EM tail helix 87–150 64 10 15.6  NS 54 11.1 NS 
TnC 
All 1–161 161 0.6 <0.0001  160 1.9  
N-lobe 1–85 85 0.0010  85  
C-lobe 86–161 76 0.012  75 4.0  

The left two columns delineate troponin regions analyzed. The middle columns concern pathogenic sites, defined in publications as amino acid residues with at least one confirmed pathogenic or likely pathogenic substitution (Walsh et al., 2017; Ho et al., 2018; Alfares et al., 2015). The right four columns concern troponin residues with no pathogenic missense mutations and at least one missense VUS (VUS substitution site) observed in cardiomyopathy subjects. P values for the observed densities were calculated as described in Materials and methods. NS indicates P > 0.05. Areas unseen by cryo-EM are TnI 1–40 and TnT residues 1–86, 151–198, and 273–288.

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