Abstract
Cardiac muscle contraction depends on the tightly regulated interactions of thin and thick filament proteins of the contractile apparatus. Mutations of thin filament proteins (actin, tropomyosin, and troponin), causing familial hypertrophic cardiomyopathy (FHC), occur predominantly in evolutionarily conserved regions and induce various functional defects that impair the normal contractile mechanism. Dysfunctional properties observed with the FHC mutants include altered Ca2+ sensitivity, changes in ATPase activity, changes in the force and velocity of contraction, and destabilization of the contractile complex. One apparent tendency observed in these thin filament mutations is an increase in the Ca2+ sensitivity of force development. This trend in Ca2+ sensitivity is probably induced by altering the cross-bridge kinetics and the Ca2+ affinity of troponin C. These in vitro defects lead to a wide variety of in vivo cardiac abnormalities and phenotypes, some more severe than others and some resulting in sudden cardiac death.
| Original language | English |
|---|---|
| Pages (from-to) | 1125-1136 |
| Number of pages | 12 |
| Journal | Journal of Applied Physiology |
| Volume | 90 |
| Issue number | 3 |
| State | Published - Mar 6 2001 |
| Externally published | Yes |
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Keywords
- Actin
- Familial hypertrophic cardiomyopathy
- Myocardium
- Tropomyosin
- Troponin
ASJC Scopus subject areas
- Physiology
- Endocrinology
- Orthopedics and Sports Medicine
- Physical Therapy, Sports Therapy and Rehabilitation
Cite this
Invited review : Pathophysiology of cardiac muscle contraction and relaxation as a result of alterations in thin filament regulation. / Hernandez, Olga M.; Housmans, Philippe R.; Potter, James D.
In: Journal of Applied Physiology, Vol. 90, No. 3, 06.03.2001, p. 1125-1136.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Invited review
T2 - Pathophysiology of cardiac muscle contraction and relaxation as a result of alterations in thin filament regulation
AU - Hernandez, Olga M.
AU - Housmans, Philippe R.
AU - Potter, James D.
PY - 2001/3/6
Y1 - 2001/3/6
N2 - Cardiac muscle contraction depends on the tightly regulated interactions of thin and thick filament proteins of the contractile apparatus. Mutations of thin filament proteins (actin, tropomyosin, and troponin), causing familial hypertrophic cardiomyopathy (FHC), occur predominantly in evolutionarily conserved regions and induce various functional defects that impair the normal contractile mechanism. Dysfunctional properties observed with the FHC mutants include altered Ca2+ sensitivity, changes in ATPase activity, changes in the force and velocity of contraction, and destabilization of the contractile complex. One apparent tendency observed in these thin filament mutations is an increase in the Ca2+ sensitivity of force development. This trend in Ca2+ sensitivity is probably induced by altering the cross-bridge kinetics and the Ca2+ affinity of troponin C. These in vitro defects lead to a wide variety of in vivo cardiac abnormalities and phenotypes, some more severe than others and some resulting in sudden cardiac death.
AB - Cardiac muscle contraction depends on the tightly regulated interactions of thin and thick filament proteins of the contractile apparatus. Mutations of thin filament proteins (actin, tropomyosin, and troponin), causing familial hypertrophic cardiomyopathy (FHC), occur predominantly in evolutionarily conserved regions and induce various functional defects that impair the normal contractile mechanism. Dysfunctional properties observed with the FHC mutants include altered Ca2+ sensitivity, changes in ATPase activity, changes in the force and velocity of contraction, and destabilization of the contractile complex. One apparent tendency observed in these thin filament mutations is an increase in the Ca2+ sensitivity of force development. This trend in Ca2+ sensitivity is probably induced by altering the cross-bridge kinetics and the Ca2+ affinity of troponin C. These in vitro defects lead to a wide variety of in vivo cardiac abnormalities and phenotypes, some more severe than others and some resulting in sudden cardiac death.
KW - Actin
KW - Familial hypertrophic cardiomyopathy
KW - Myocardium
KW - Tropomyosin
KW - Troponin
UR - http://www.scopus.com/inward/record.url?scp=0035109415&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0035109415&partnerID=8YFLogxK
M3 - Article
C2 - 11181629
AN - SCOPUS:0035109415
VL - 90
SP - 1125
EP - 1136
JO - Journal of Applied Physiology
JF - Journal of Applied Physiology
SN - 8750-7587
IS - 3
ER -