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Inherited disorders of sarcomeric proteins

Current Opinion in Neurology, 1999
The most important advances in sarcomeric protein diseases continue to be the identification of mutated genes responsible for human diseases. These have recently included those that encode skeletal muscle alpha-actin in autosomal dominant and autosomal recessive nemaline myopathy, nebulin and slow alpha-tropomyosin in autosomal recessive nemaline ...
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The Sarcomeric Control of Energy Conversion

Annals of the New York Academy of Sciences, 2005
Abstract: The Frank‐Starling Law, Fenn Effect, and Suga's suggestions of cardiac muscle constant contractile efficiency establish the dependence of cardiac mechanics and energetics on the loading conditions. Consistent with these observations, this review suggests that the sarcomere control of contraction consists of two dominant feedbacks: (1) a ...
Amir Landesberg   +3 more
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Is stepwise sarcomere shortening an artefact?

Nature, 1984
A report in 1977 raised the intriguing possibility that sarcomere shortening in muscle may occur in a stepwise fashion, in which episodes of shortening are interrupted by periods of little or no movement. This was taken by its authors to imply the synchronous activity of cross-bridges over a large volume of tissue-behaviour which cannot easily be ...
ALTRINGHAM JD   +2 more
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A numerical study of the stiffness of a sarcomere

Journal of Electromyography and Kinesiology, 1998
Relatively recent experimental findings of the significant compliance of the thin (actin) and thick (myosin) filaments have brought into question a number of conclusions based on the assumption of perfect myofilament rigidity. A new model based on a discrete representation of the relevant structures was used to calculate the theoretical stiffness of a ...
Mario Forcinito   +2 more
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Architecture and function in the muscle sarcomere

Current Opinion in Structural Biology, 1997
Striated muscle sarcomeres in vertebrates comprise ordered arrays of actin and myosin filaments, organized by an elaborate protein scaffold. Recent innovative work in a number of laboratories has greatly improved our knowledge of these structures, their organization and their interactions.
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Sarcomere Function and Crossbridge Cycling

1995
The power of the heart is dictated by the force development and velocity of shortening (V) of the cardiac sarcomere. Both depend on the amount of Ca++ released by the sarcoplasmic reticulum during the action potential. We have investigated the inter-relationship between force (F) sarcomere length (SL) and V and the intracellular Ca++ concentration ([Ca+
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The non-sliding filaments of the sarcomere

Meat Science, 1987
A model is proposed for the 'gap' or 'third' filaments of muscle, here renamed 'T-filaments'. These consist of single titin molecules, spanning the half sarcomere from M-line to Z-line. Of several possibilities for stoichiometry and arrangement, the one most favoured here consists of six T-filaments lying longitudinally on the surface of the A-filament,
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Sarcomere reorganization in mite muscle

Developmental Biology, 1976
Abstract The number of sarcomeres in a given muscle of the mite Tarsonemus randsi was constant in both larval and adult stages, with the exception of the two medial dorsal metapodosomal muscles in males. These muscles have three sarcomeres in larvae and one sarcomere in adults.
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