Effects of a R133W beta-tropomyosin mutation on regulation of muscle contraction in single human muscle fibres.

Effects of a R133W beta-tropomyosin mutation on regulation of muscle contraction in single human muscle fibres. Research Abstract Details 

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Effects of a R133W beta-tropomyosin mutation on regulation of muscle contraction in single human muscle fibres. Abstract Text:

A novel R133W beta-tropomyosin (beta-Tm) mutation, associated with muscle weakness and distal limb deformities, has recently been identified in a woman and her daughter. The muscle weakness was not accompanied by progressive muscle wasting or histopathological abnormalities in tibialis anterior muscle biopsy specimens. The aim of the present study was to explore the mechanisms underlying the impaired muscle function in patients with the beta-Tm mutation. Maximum force normalized to fibre cross-sectional area (specific force, SF), maximum velocity of unloaded shortening (V0), apparent rate constant of force redevelopment (ktr) and force-pCa relationship were evaluated in single chemically skinned muscle fibres from the two patients carrying the beta-Tm mutation and from healthy control subjects. Significant differences in regulation of muscle contraction were observed in the type I fibres: a lower SF (P<0.05) and ktr (P<0.01), and a faster V0 (P<0.05). The force-pCa relationship did not differ between patient and control fibres, indicating an unaltered Ca2+ activation of contractile proteins. Collectively, these results indicate a slower cross-bridge attachment rate and a faster detachment rate caused by the R133W beta-Tm mutation. It is suggested that the R133W beta-Tm mutation induces alteration in myosin-actin kinetics causing a reduced number of myosin molecules in the strong actin-binding state, resulting in overall muscle weakness in the absence of muscle wasting.

Effects of a R133W beta-tropomyosin mutation on regulation of muscle contraction in single human muscle fibres. Publishing Authors By Initials

For similar macromolecular substances: polymers: biopolymers: microfilament proteins: tropomyosin research abstracts see: macromolecular substances: polymers: biopolymers: microfilament proteins: tropomyosin research

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Effects of a R133W beta-tropomyosin mutation on regulation of muscle contraction in single human muscle fibres. Journal Published:

PUBLICATION TYPE: Research Support, Non-U.S. Gov

Journal: The Journal of physiology

VOLUME: 581

Page Numbers: 1283-92

Journal Abbreviation: J. Physiol. (Lond.)

ISSN: 0022-3751

DAY: 12

MONTH: 04

YEAR: 2007

Effects of a R133W beta-tropomyosin mutation on regulation of muscle contraction in single human muscle fibres. Information

Number of References:

LANGUAGE: eng

NlmUniqueID: 266262

Effects of a R133W beta-tropomyosin mutation on regulation of muscle contraction in single human muscle fibres. Keywords Mesh Terms:

KEYWORDS: Tropomyosin

MESH TERMS: metabolism

Chemical & Substance for Abstract: Effects of a R133W beta-tropomyosin mutation on regulation of muscle contraction in single human muscle fibres. Information

Substance Name: Myosins

Registry Number: EC 3.6.1.4

Grant and Affiliation Information for Effects of a R133W beta-tropomyosin mutation on regulation of muscle contraction in single human muscle fibres.

AFFILIATION: Department of Clinical Neurophysiology, Uppsala University Hospital, Entrance 85, 3rd floor, SE-751 85 Uppsala, Sweden. julien.ochala@neurofys.uu.se

Country: England

AGENCY: United States NIAMS

GRANT: AR047318

ACRONYM: AR

MEDLINETA: J Physiol

REFSOURCE: J Physiol. 2007 Jun 15;581(Pt 3):889

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