Table III.

Conduction phenotypes with prolonged PR interval

Gene
Protein
Model
Conduction defects in addition to AV block
Cacna1d L-type Ca channel KO Abnormal QRS; sinus arrhythmia (41) 
Egf-R EGF-Receptor KO Abnormal QRS; prolonged QT (42) 
EphA3 Eph receptor A3 KO Bradycardia (43) 
Cx40 Connexin 40 KO Prolonged P, QT; abnormal QRS (5, 44) 
Cx43 Connexin 43 KO Abnormal QRS (45, 46) 
Cx45 Connexin 45 KO Isolated AV conduction block (26, 47) 
Hadhb HAC-CoA-dehyd. ENU AV block with sudden death (48) 
Kcnq1 K channel Q1 KO Prolonged QT; abnormal QRS (49) 
LmnA Lamin A KI Abnormal QRS (50) 
Nkx2-5 NK2 TF related 5 KO Prolonged PR in hetero, lack of AV node in KO (51, 52) 
Scn5A Na channel Vα KO Prolonged P; tachycardia (36, 53, 54) 
Tbx5 T-box5 KO AV block, abnormal QRS, ventricular tachycardia (55, 56) 
Agtr1a Ang II - Receptor TG Abnormal QRS (57) 
Tead4 TEA domain 4 TG Abnormal QRS (58) 
Vegfb VEGF B KO Isolated AV block (first degree only) (59) 
Gene
Protein
Model
Conduction defects in addition to AV block
Cacna1d L-type Ca channel KO Abnormal QRS; sinus arrhythmia (41) 
Egf-R EGF-Receptor KO Abnormal QRS; prolonged QT (42) 
EphA3 Eph receptor A3 KO Bradycardia (43) 
Cx40 Connexin 40 KO Prolonged P, QT; abnormal QRS (5, 44) 
Cx43 Connexin 43 KO Abnormal QRS (45, 46) 
Cx45 Connexin 45 KO Isolated AV conduction block (26, 47) 
Hadhb HAC-CoA-dehyd. ENU AV block with sudden death (48) 
Kcnq1 K channel Q1 KO Prolonged QT; abnormal QRS (49) 
LmnA Lamin A KI Abnormal QRS (50) 
Nkx2-5 NK2 TF related 5 KO Prolonged PR in hetero, lack of AV node in KO (51, 52) 
Scn5A Na channel Vα KO Prolonged P; tachycardia (36, 53, 54) 
Tbx5 T-box5 KO AV block, abnormal QRS, ventricular tachycardia (55, 56) 
Agtr1a Ang II - Receptor TG Abnormal QRS (57) 
Tead4 TEA domain 4 TG Abnormal QRS (58) 
Vegfb VEGF B KO Isolated AV block (first degree only) (59) 

Italics show animal models that show the identical phenotype to the one described in this paper. ENU, ENU Mutagenesis screen; HAC-CaA dehyd., hydroxyacyl–Coenzyme A dehydrogenase; KI, knockin; TG, transgenic.

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