The in vitro motility activity of beta-cardiac myosin depends on the nature of the beta-myosin heavy chain gene mutation in hypertrophic cardiomyopathy.

Abstract:

:Several mutations in the beta-myosin heavy chain gene cause hypertrophic cardiomyopathy. This study investigates (1) the in vitro velocities of translocation of fluorescently-labelled actin by beta-myosin purified from soleus muscle of 30 hypertrophic cardiomyopathy patients with seven distinct beta-myosin heavy chain gene mutations: Thr124Ile, Tyr162Cys, Gly256Glu, Arg403Gln, Val606Met, Arg870His, and Leu908Val mutations; and (2) motility activity of beta-myosin purified from cardiac and soleus muscle biopsies in the same patients. The velocity of translocation of actin by beta-myosin purified from soleus or cardiac muscle of 22 normal controls was 0.48 +/- 0.09 micron s-1. By comparison, the motility activity was reduced in all 30 patients with beta-myosin heavy chain gene mutations (range, 0.112 +/- 0.041 to 0.292 +/- 0.066 micron s-1. Notably, the Tyr162Cys and Arg403Gln mutations demonstrated significantly lower actin sliding velocities: 0.123 +/- 0.044, and 0.112 +/- 0.041 micron s-1, respectively. beta-myosin purified from soleus muscle from four patients with the Arg403Gln mutation had a similar actomyosin motility activity compared to beta-myosin purified from their cardiac biopsies (0.127 +/- 0.045 micron s-1 versus 0.119 +/- 0.068 micron s-1, respectively). Since these seven mutations lie in several distinct functional domains, it is likely that the mechanisms of their inhibitions of motility are different.

authors

Cuda G,Fananapazir L,Epstein ND,Sellers JR

doi

10.1023/a:1018613907574

subject

Has Abstract

pub_date

1997-06-01 00:00:00

pages

275-83

issue

3

eissn

0142-4319

issn

1573-2657

journal_volume

18

pub_type

杂志文章
  • ADF/cofilin regulation from a structural viewpoint.

    abstract::ADF/cofilins disassemble the actin filament and contribute to a wide range of actin dynamics. These proteins are regulated by several factors, including phosphorylation, pH and mechanical forces. Although the cofilin-decorated actin filament structure was published recently, the mechanisms of these regulating factors ...

    journal_title:Journal of muscle research and cell motility

    pub_type: 杂志文章

    doi:10.1007/s10974-019-09546-6

    authors: Narita A

    更新日期:2020-03-01 00:00:00

  • Mechanism of force enhancement during and after lengthening of active muscle: a temperature dependence study.

    abstract::The aim of the present study was to examine the temperature dependence of active force in lengthening and shortening muscle. Experiments were done, in vitro, on bundles of intact fibres (fibre length L(0) ~2 mm; sarcomere length ~2.5 μm) isolated from a rat fast muscle (flexor hallucis brevis) and a ramp length change...

    journal_title:Journal of muscle research and cell motility

    pub_type: 杂志文章

    doi:10.1007/s10974-012-9307-8

    authors: Roots H,Pinniger GJ,Offer GW,Ranatunga KW

    更新日期:2012-10-01 00:00:00

  • Cell-free incorporation of newly synthesized myosin subunits into thick myofilaments.

    abstract::Although a substantial literature exists on the in vitro polymerization of purified myosin, little is known about native thick filament assembly, remodeling or turnover. We have recently described a cell-free system (Bouche et al., 1988) to examine the interactions between thick filaments and soluble, newly synthesize...

    journal_title:Journal of muscle research and cell motility

    pub_type: 杂志文章

    doi:10.1007/BF01774035

    authors: Goldfine SM,Einheber S,Fischman DA

    更新日期:1991-04-01 00:00:00

  • Freeze-fractured sarcoplasmic reticulum in adult and embryonic fast and slow muscles.

    abstract::There is evidence to suggest that 8 nm calcium transport particles in the sarcoplasmic reticulum are involved in the regulation of twitch properties in adult muscles. We have studied ultrastructural characteristics of the sarcoplasmic reticulum in relation to previously defined physiological changes that take place in...

    journal_title:Journal of muscle research and cell motility

    pub_type: 杂志文章

    doi:10.1007/BF01753413

    authors: Gauthier GF,Hobbs AW

    更新日期:1986-04-01 00:00:00

  • Temperature dependent measurements reveal similarities between muscle and non-muscle myosin motility.

    abstract::We examined the temperature dependence of muscle and non-muscle myosin (heavy meromyosin, HMM) with in vitro motility and actin-activated ATPase assays. Our results indicate that myosin V (MV) has a temperature dependence that is similar in both ATPase and motility assays. We demonstrate that skeletal muscle myosin (S...

    journal_title:Journal of muscle research and cell motility

    pub_type: 杂志文章

    doi:10.1007/s10974-012-9316-7

    authors: Yengo CM,Takagi Y,Sellers JR

    更新日期:2012-12-01 00:00:00

  • Reduced expression of MyHC slow isoform in rat soleus during unloading is accompanied by alterations of endogenous inhibitors of calcineurin/NFAT signaling pathway.

    abstract::Under muscle disuse conditions decrease of expression of MyHC of slow type, and sometimes of type IIa, as well as upregulation of expression of IIb and IId/x isoforms were observed. Through dephosphorylation and entry of NFAT molecules to the nucleus calcineurin/NFATc1 signaling pathway promotes upregulation of the sl...

    journal_title:Journal of muscle research and cell motility

    pub_type: 杂志文章

    doi:10.1007/s10974-015-9428-y

    authors: Lomonosova YN,Turtikova OV,Shenkman BS

    更新日期:2016-04-01 00:00:00

  • The effects of ramp stretches on active contractions in intact mammalian fast and slow muscle fibres.

    abstract::The effects of a ramp stretch (amplitude <6% muscle fibre length (L0), speed < 13L0 s(-1)) on twitch tension and twitch tension re-development were examined in intact mammalian (rat) fast and slow muscle fibre bundles. The experiments were done in vitro at 20 degrees C and at an initial sarcomere length of 2.68 microm...

    journal_title:Journal of muscle research and cell motility

    pub_type: 杂志文章

    doi:10.1023/a:1010556623905

    authors: Mutungi G,Ranatunga KW

    更新日期:2001-01-01 00:00:00

  • Genetic control of muscle development: learning from Drosophila.

    abstract::Muscle development involves a complex sequence of time and spatially regulated cellular events leading to the formation of highly specialised syncytial muscle cells displaying a common feature, the capacity of contraction. Analyses of mechanisms controlling muscle development reveals that the main steps of muscle form...

    journal_title:Journal of muscle research and cell motility

    pub_type: 杂志文章,评审

    doi:10.1007/s10974-008-9133-1

    authors: Maqbool T,Jagla K

    更新日期:2007-01-01 00:00:00

  • The troponin I: inhibitory peptide uncouples force generation and the cooperativity of contractile activation in mammalian skeletal muscle.

    abstract::Hodges and his colleagues identified a 12 amino acid fragment of troponin I (TnI-ip) that inhibits Ca(2+)-activated force and reduces the effectiveness Ca(2+) as an activator. To understand the role of troponin C (TnC) in the extended cooperative interactions of thin filament activation, we compared the effect of TnI-...

    journal_title:Journal of muscle research and cell motility

    pub_type: 杂志文章

    doi:10.1007/s10974-013-9336-y

    authors: Schachat F,Brandt PW

    更新日期:2013-05-01 00:00:00

  • Acidosis affects muscle contraction by slowing the rates myosin attaches to and detaches from actin.

    abstract::The loss of muscle force and power during fatigue from intense contractile activity is associated with, and likely caused by, elevated levels of phosphate ([Formula: see text]) and hydrogen ions (decreased pH). To understand how these deficits in muscle performance occur at the molecular level, we used direct measurem...

    journal_title:Journal of muscle research and cell motility

    pub_type: 杂志文章

    doi:10.1007/s10974-018-9499-7

    authors: Jarvis K,Woodward M,Debold EP,Walcott S

    更新日期:2018-08-01 00:00:00

  • Insertion of alpha1S II-III loop and C terminal sequences into alpha1H fails to restore excitation-contraction coupling in dysgenic myotubes.

    abstract::The L-type Ca2+ channel in skeletal muscle (alpha1S) is essential for excitation-contraction (EC) coupling. Previous studies using chimeras composed of alpha1S together with alpha1C or alpha1M demonstrated the importance of the alpha1S II-III loop and of a smaller subdomain (residues 720-764; 'ECC') in skeletal EC cou...

    journal_title:Journal of muscle research and cell motility

    pub_type: 杂志文章

    doi:10.1023/a:1024830132118

    authors: Wilkens CM,Beam KG

    更新日期:2003-01-01 00:00:00

  • Effects of long-term phasic electrical stimulation on denervated soleus muscle: guinea-pig contrasted with rat.

    abstract::Guinea-pig soleus muscles were denervated and electrically stimulated for periods of 43 to 66 days. Stimuli were in 1 s bursts of 40 Hz pulses, repeated every 5 min. Other guinea-pigs were denervated for 82 days without stimulation and, in a third group, the soleus muscle was necrotized and allowed to regenerate witho...

    journal_title:Journal of muscle research and cell motility

    pub_type: 杂志文章

    doi:10.1023/a:1018619503456

    authors: Lewis DM,al-Amood WS,Schmalbruch H

    更新日期:1997-10-01 00:00:00

  • Expression of a novel combination of fast and slow troponin T isoforms in rabbit extraocular muscles.

    abstract::The properties of extraocular muscles (EOMs) are quite different from those of the trunk and limb. Here we show that there is a novel pattern of troponin T (TnT) expression in EOMs which most likely contributes to the fine control of ocular movement and may reflect their innervation by cranial motoneurons. Three regio...

    journal_title:Journal of muscle research and cell motility

    pub_type: 杂志文章

    doi:10.1007/BF01773894

    authors: Briggs MM,Jacoby J,Davidowitz J,Schachat FH

    更新日期:1988-06-01 00:00:00

  • The effect of length on the sensitivity to phenylephrine and calcium in intact and skinned vascular smooth muscle.

    abstract::The length dependency of the sensitivity to activators of the smooth muscle of different blood vessels is not yet fully understood. Muscle preparations of the aorta, the femoral artery and the portal vein of the rabbit were investigated for the length dependency of the sensitivity to phenylephrine and calcium in both ...

    journal_title:Journal of muscle research and cell motility

    pub_type: 杂志文章

    doi:10.1023/a:1005408015885

    authors: Van Heijst BG,De Wit E,Van der Heide UA,Blangé T,Jongsma HJ,De Beer EL

    更新日期:1999-01-01 00:00:00

  • Characterization of ultrastructural and contractile activation properties of crustacean (Cherax destructor) muscle fibres during claw regeneration and moulting.

    abstract::Long-(SL > 6 microns) and short-sarcomere (SL < 4 microns) fibres were isolated from the claw muscle of the yabby (Cherax destructor) during limb regeneration and at different stages of the moult cycle. Long-sarcomere fibres were more susceptible to the changes resulting from the moult-induced atrophy compared with th...

    journal_title:Journal of muscle research and cell motility

    pub_type: 杂志文章

    doi:10.1007/BF00121136

    authors: West JM,Humphris DC,Stephenson DG

    更新日期:1995-06-01 00:00:00

  • Assembly of the cardiac I-band region of titin/connectin: expression of the cardiac-specific regions and their structural relation to the elastic segments.

    abstract::The giant molecule titin (also called connectin) provides an elastic connection in the I-band between the Z-disk and A-band of striated muscle. This region is assembled in a tissue-specific way by extensive differential splicing events. We have raised monoclonal antibodies against the two N2-line isoforms of titin and...

    journal_title:Journal of muscle research and cell motility

    pub_type: 杂志文章

    doi:10.1007/BF00123361

    authors: Gautel M,Lehtonen E,Pietruschka F

    更新日期:1996-08-01 00:00:00

  • Effects of fatigue and altered pH on isometric force and velocity of shortening at zero load in frog muscle fibres.

    abstract::temporaria (0.9-2.5degrees C) were stimulated to produce a 1 s isometric tetanus at regular intervals until constant mechanical responses were attained. Various degrees of force depression ("fatigue") were produced by decreasing the contraction interval from 30 or 15 min (control to 120, 60, 30 and 15s, respectively. ...

    journal_title:Journal of muscle research and cell motility

    pub_type: 杂志文章

    doi:10.1007/BF00713270

    authors: Edman KA,Mattiazzi AR

    更新日期:1981-09-01 00:00:00

  • Cellular mechanisms and local progenitor activation to regulate skeletal muscle mass.

    abstract::Skeletal muscle hypertrophy is a result of increased load, such as functional and stretch-overload. Activation of satellite cells and proliferation, differentiation and fusion are required for hypertrophy of overloaded skeletal muscles. On the contrary, a dramatic loss of skeletal muscle mass determines atrophy settin...

    journal_title:Journal of muscle research and cell motility

    pub_type: 杂志文章,评审

    doi:10.1007/s10974-010-9204-y

    authors: Cassano M,Quattrocelli M,Crippa S,Perini I,Ronzoni F,Sampaolesi M

    更新日期:2009-12-01 00:00:00

  • Shortening properties of two biochemically defined muscle fibre types of the Norway lobster Nephrops norvegicus L.

    abstract::Mechanical properties of myofibrillar bundles from single chemically skinned fibres from the superficial abdominal flexor muscle of the Norway lobster Nephrops norvegicus were measured, and the protein content of these fibres was analysed by SDS-PAGE. Two slow fibre phenotypes (S1, S2) were distinguished on the basis ...

    journal_title:Journal of muscle research and cell motility

    pub_type: 杂志文章

    doi:10.1023/a:1005481725344

    authors: Holmes JM,Hilber K,Galler S,Neil DM

    更新日期:1999-04-01 00:00:00

  • Filament lengths in frog semitendinosus and tibialis anterior muscle fibres.

    abstract::In frog semitendinosus muscle the descending limb of the length-tension curve is shifted rightward relative to that of tibialis anterior. Both the plateau right corner and the zero-force intercept are equally shifted. To investigate the reason for this shift, we compared filament lengths in the two muscles. Single fib...

    journal_title:Journal of muscle research and cell motility

    pub_type: 杂志文章

    doi:10.1007/BF00115451

    authors: Trombitás K,Frey L,Pollack GH

    更新日期:1993-04-01 00:00:00

  • The gradual expression of troponin T isoforms in chicken wing muscles.

    abstract::Troponin T (TnT) is one of the muscle regulatory proteins and is thought to be related to unique contractile properties in diverse muscles and also to myogenesis. The expression of TnT isoforms in the chicken wing muscles was investigated by two-dimensional gel electrophoresis and immunoblotting with an antiserum agai...

    journal_title:Journal of muscle research and cell motility

    pub_type: 杂志文章

    doi:10.1023/a:1016369300532

    authors: Miyazaki JI,Akutsu S,Satow N,Hirao C,Yao Y

    更新日期:2001-01-01 00:00:00

  • Fibre types in Limulus telson muscles: morphology and histochemistry.

    abstract::Using a variety of techniques, we have demonstrated the presence of at least two fibre types in Limulus median telson levator muscle. By light and electron microscopy, large (2,156 microns 2 mean cross-sectional area) fibres have A-bands of 4.1 microns, one-half I bands of 2.15 microns and Z lines less than or equal t...

    journal_title:Journal of muscle research and cell motility

    pub_type: 杂志文章

    doi:10.1007/BF01739856

    authors: Levine RJ,Davidheiser S,Kelly AM,Kensler RW,Leferovich J,Davies RE

    更新日期:1989-02-01 00:00:00

  • Rapid decrease in active tension generated by C2C12 myotubes after termination of artificial exercise.

    abstract::We found that the active tension of C2C12 myotubes that had been subjected to artificial exercise for ~10 days decreased rapidly after termination of the artificial exercise. When differentiated C2C12 myotubes were subjected to continuous 1 Hz artificial exercise for ~10 days, the active tension increased to ~4× compa...

    journal_title:Journal of muscle research and cell motility

    pub_type: 杂志文章

    doi:10.1007/s10974-010-9230-9

    authors: Fujita H,Hirano M,Shimizu K,Nagamori E

    更新日期:2010-12-01 00:00:00

  • Are mechanically sensitive regulators involved in the function and (patho)physiology of cerebral palsy-related contractures?

    abstract::Skeletal muscle tissue is mechanosensitive, as it is able to sense mechanical impacts and to translate these into biochemical signals making the tissue adapt. Among its mechanosensitive nature, skeletal muscle tissue is the largest metabolic organ of the human body. Disturbances in skeletal muscle mechanosensing and m...

    journal_title:Journal of muscle research and cell motility

    pub_type: 杂志文章,评审

    doi:10.1007/s10974-017-9489-1

    authors: Pingel J,Suhr F

    更新日期:2017-08-01 00:00:00

  • Poly-Ig tandems from I-band titin share extended domain arrangements irrespective of the distinct features of their modular constituents.

    abstract::The cellular function of the giant protein titin in striated muscle is a major focus of scientific attention. Particularly, its role in passive mechanics has been extensively investigated. In strong contrast, the structural details of this filament are very poorly understood. To date, only a handful of atomic models f...

    journal_title:Journal of muscle research and cell motility

    pub_type: 杂志文章

    doi:10.1007/s10974-005-9017-6

    authors: Marino M,Svergun DI,Kreplak L,Konarev PV,Maco B,Labeit D,Mayans O

    更新日期:2005-01-01 00:00:00

  • What do we know about what happens to myometrial function as women age?

    abstract::Much has been written about the effects of aging on reproductive function, especially female fertility. Much less is known about how aging may affect the contractility of the smooth muscle within the uterus, the myometrium. The myometrium is active through a woman's entire life, not just during pregnancy. Here we will...

    journal_title:Journal of muscle research and cell motility

    pub_type: 杂志文章

    doi:10.1007/s10974-012-9300-2

    authors: Arrowsmith S,Robinson H,Noble K,Wray S

    更新日期:2012-08-01 00:00:00

  • Slow motor units in female rat soleus are slower and weaker than their male counterparts.

    abstract::The aim of the study was to investigate sex-related differences in contractile properties, parameters of action potentials, and mechanisms of force regulation of motor units in the rat soleus muscle, which is a frequent experimental model in animal research. It was revealed that the mean mass of the muscle in males wa...

    journal_title:Journal of muscle research and cell motility

    pub_type: 杂志文章

    doi:10.1007/s10974-015-9408-2

    authors: Drzymała-Celichowska H,Krutki P

    更新日期:2015-06-01 00:00:00

  • Spontaneous contractions and stretch-evoked responses of isolated lymph nodes.

    abstract::In this study the spontaneous activities of prescapular lymph nodes, which were isolated from calves, goats and sheep and mesenteric lymph nodes of guinea-pigs were recorded and analysed in the frequency domain. Stretch-evoked contractions of the mesenteric lymph nodes were also recorded and their frequency characteri...

    journal_title:Journal of muscle research and cell motility

    pub_type: 杂志文章

    doi:10.1007/BF00711961

    authors: Tümer A,Oztürk-Demir N,Basar-Eroğlu C,Noyan A

    更新日期:1983-02-01 00:00:00

  • Therapeutic clenbuterol treatment does not alter Ca2+ sensitivity of permeabilized fast muscle fibres from exercise trained or untrained horses.

    abstract::Clenbuterol is a beta2-adrenoceptor agonist primarily used for treating bronchospasm and alleviating the symptoms of chronic obstructive pulmonary disease (COPD) in the horse. In other species (rats, mice, sheep, and cattle), chronic high doses of clenbuterol (typically in the milligram per kilogram body weight range)...

    journal_title:Journal of muscle research and cell motility

    pub_type: 杂志文章

    doi:10.1023/a:1027377731137

    authors: Plant DR,Kearns CF,McKeever KH,Lynch GS

    更新日期:2003-01-01 00:00:00

  • Z-band proteins in the flight muscle and leg muscle of the honeybee.

    abstract::Monoclonal antibodies (mAb's) have been raised against proteins in preparations of Z-discs isolated from honeybee fibrillar flight muscle. These antibodies have identified four Z-disc antigens on immunoblots of honeybee fibrillar proteins. Antibody alpha binds to the 90-100 kD protein, alpha-actinin; mAb P interacts w...

    journal_title:Journal of muscle research and cell motility

    pub_type: 杂志文章

    doi:10.1007/BF01766491

    authors: Saide JD,Chin-Bow S,Hogan-Sheldon J,Busquets-Turner L

    更新日期:1990-04-01 00:00:00