scholarly journals Structural Kinetics of Cardiac Troponin C Mutants Linked to Familial Hypertrophic and Dilated Cardiomyopathy in Troponin Complexes

2007 ◽  
Vol 283 (6) ◽  
pp. 3424-3432 ◽  
Author(s):  
Wen-Ji Dong ◽  
Jun Xing ◽  
Yexin Ouyang ◽  
Jianli An ◽  
Herbert C. Cheung
Keyword(s):  
Dilated Cardiomyopathy ◽  
Cardiac Troponin ◽  
Troponin C ◽  
Cardiac Troponin C ◽  
Kinetics Of

scholarly journals Knock-in mice harboring a Ca2+ desensitizing mutation in cardiac troponin C develop early onset dilated cardiomyopathy

2015 ◽  
Vol 6 ◽  
Author(s):  
Bradley K. McConnell ◽  
Sonal Singh ◽  
Qiying Fan ◽  
Adriana Hernandez ◽  
Jesus P. Portillo ◽  
...  
Keyword(s):  
Dilated Cardiomyopathy ◽  
Early Onset ◽  
Cardiac Troponin ◽  
Troponin C ◽  
Cardiac Troponin C

The Dilated Cardiomyopathy G159D Mutation in Cardiac Troponin C Weakens the Anchoring Interaction with Troponin I†

Biochemistry ◽  
2008 ◽  
Vol 47 (41) ◽  
pp. 10950-10960 ◽  
Author(s):  
Olga K. Baryshnikova ◽  
Ian M. Robertson ◽  
Pascal Mercier ◽  
Brian D. Sykes
Keyword(s):  
Dilated Cardiomyopathy ◽  
Cardiac Troponin ◽  
Troponin I ◽  
Troponin C ◽  
Cardiac Troponin C

scholarly journals Dilated cardiomyopathy mutations and phosphorylation disrupt the active orientation of cardiac troponin C

2021 ◽  
pp. 167010
Author(s):  
Zabed Mahmud ◽  
Prabhpaul S. Dhami ◽  
Caleb Rans ◽  
Philip B. Liu ◽  
Peter M. Hwang
Keyword(s):  
Dilated Cardiomyopathy ◽  
Cardiac Troponin ◽  
Troponin C ◽  
Cardiac Troponin C ◽  
Cardiomyopathy Mutations

scholarly journals Cardiomyopathy-Related Mutations in Cardiac Troponin C, L29Q and G159D, Have Divergent Effects on Rat Cardiac Myofiber Contractile Dynamics

2012 ◽  
Vol 2012 ◽  
pp. 1-11 ◽  
Author(s):  
Sampath K. Gollapudi ◽  
Murali Chandra
Keyword(s):  
Papillary Muscle ◽  
Cardiac Troponin ◽  
Thin Filament ◽  
Muscle Fibers ◽  
Troponin C ◽  
Wild Type ◽  
Cycling Rate ◽  
Cardiac Troponin C ◽  
Maximal Tension ◽  
Kinetics Of

Previous studies of cardiomyopathy-related mutations in cardiac troponin C (cTnC)—L29Q and G159D—have shown diverse findings. The link between such mutant effects and their divergent impact on cardiac phenotypes has remained elusive due to lack of studies on contractile dynamics. We hypothesized that a cTnC mutant-induced change in the thin filament will affect global myofilament mechanodynamics because of the interactions of thin filament kinetics with bothCa2+binding and crossbridge (XB) cycling kinetics. We measured pCa-tension relationship and contractile dynamics in detergent-skinned rat cardiac papillary muscle fibers reconstituted with the recombinant wild-type rat cTnC (cTnCWT),cTnCL29Q, andcTnCG159Dmutants.cTnCL29Qfibers demonstrated a significant decrease inCa2+sensitivity, butcTnCG159Dfibers did not. Both mutants had no effect onCa2+-activated maximal tension. The rate of XB recruitment dynamics increased incTnCL29Q(26%) andcTnCG159D(25%) fibers. The rate of XB distortion dynamics increased incTnCG159Dfibers (15%). Thus, thecTnCL29Qmutant modulates the equilibrium between the non-cycling and cycling pool of XB by affecting theon/offkinetics of the regulatory units (Tropomyosin-Troponin); whereas, thecTnCG159Dmutant increases XB cycling rate. Different effects on contractile dynamics may offer clue regarding howcTnCL29QandcTnCG159Dcause divergent effects on cardiac phenotypes.

scholarly journals Zebrafish With Antisense-Knockdown of Cardiac Troponin C as a Model of Hereditary Dilated Cardiomyopathy

2009 ◽  
Vol 73 (9) ◽  
pp. 1595-1596 ◽  
Author(s):  
Nobuyuki Ohte ◽  
Ichiro Miyoshi ◽  
David C. Sane ◽  
William C. Little
Keyword(s):  
Dilated Cardiomyopathy ◽  
Cardiac Troponin ◽  
Troponin C ◽  
Cardiac Troponin C ◽  
Antisense Knockdown
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