Invited review: Pathophysiology of cardiac muscle contraction and relaxation as a result of alterations in thin filament regulation

Olga M. Hernandez, Philippe R. Housmans, James D. Potter

Research output: Contribution to journalArticle

57 Citations (Scopus)

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 languageEnglish
Pages (from-to)1125-1136
Number of pages12
JournalJournal of Applied Physiology
Volume90
Issue number3
StatePublished - Mar 6 2001
Externally publishedYes

Fingerprint

Familial Hypertrophic Cardiomyopathy
Muscle Relaxation
Muscle Contraction
Myocardium
Troponin C
Contractile Proteins
Tropomyosin
Mutation
Troponin
Sudden Cardiac Death
Actin Cytoskeleton
Adenosine Triphosphatases
Phenotype
Proteins

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 journalArticle

Hernandez, Olga M. ; Housmans, Philippe R. ; Potter, James D. / Invited review : Pathophysiology of cardiac muscle contraction and relaxation as a result of alterations in thin filament regulation. In: Journal of Applied Physiology. 2001 ; Vol. 90, No. 3. pp. 1125-1136.
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