ライフサイエンスコーパス: myofibril

1 and 3) Longitudinal splitting of an existing myofibril.

2 molecules of actin in working ex vivo heart myofibril.

3 he contractile and elastic elements within a myofibril.

4 agated from sarcomere to sarcomere along the myofibril.

5 g their maturation into sarcomere-containing myofibrils.

6 re investigated in calcium-activated cardiac myofibrils.

7 g contractions, and of fluorescently labeled myofibrils.

8 ng decreases mitochondrial content among the myofibrils.

9 phosphate transfer from the mitochondria to myofibrils.

10 oups using single muscle fibers and isolated myofibrils.

11 ificant reduction in muscle mass and thinner myofibrils.

12 osphodiesterase activity associated with the myofibrils.

13 ha-actins as well as on skeletal and cardiac myofibrils.

14 ed for cTnI(R146G)- and cTnI(R21C)-exchanged myofibrils.

15 s in mitochondrial volume and compression of myofibrils.

16 ratios of 5:1-7:1) and a mature alignment of myofibrils.

17 enzyme-protein assemblies in biopsy derived myofibrils.

18 composed of tightly-packed mitochondria and myofibrils.

19 time course of relaxation in cardiac muscle myofibrils.

20 Ca(2+) sensitivity than nontransgenic mouse myofibrils.

21 indistinguishable from that of nontransgenic myofibrils.

22 for FMNL1 and FMNL2 in the repair of damaged myofibrils.

23 ed duration of slow-phase relaxation seen in myofibrils.

24 ntractility in single transgenic mouse heart myofibrils.

25 in assembly factors being required to repair myofibrils.

26 ylation in their hearts before isolating the myofibrils.

27 f all O-GlcNAcylated proteins in mouse heart myofibrils.

28 either fetal or adult human skeletal muscle myofibrils.

29 incorporating the complex into rabbit psoas myofibrils.

30 rom several different species of ventricular myofibrils.

31 97 in extracting ubiquitinated proteins from myofibrils.

32 roximately three) in working skeletal muscle myofibrils.

33 the kinetics of transgenic (Tg)-R58Q cardiac myofibrils.

34 1.5, and maintains the alignment of adjacent myofibrils.

35 activity differently in skeletal and cardiac myofibrils.

36 e basic contractile units of skeletal muscle myofibrils.

37 etwork rather than many individual, parallel myofibrils.

38 t rely on stiffness measurements on cells or myofibrils.

39 ng isoforms sets the stereotyped diameter of myofibrils.

40 that involve shortening of sarcomeres along myofibrils.

41 quitinated proteins and rapid destruction of myofibrils.

42 ificantly higher (P < 0.0001) than in HCMsmn myofibrils (0.47 +/- 0.02 and 0.30 +/- 0.02 s(-1), respe

43 Force recordings from myofibrils (15 degrees C) at saturating [Ca(2+)] showed

44 n the mid-region or addition at the end of a myofibril; 2) Sequential addition with an existing myofi

45 hic alleles (R394H, D487N, and 520fs) induce myofibril abnormalities, altered nuclear morphology, and

46 using models made of aqueous suspensions of myofibrils according to muscle fibre types and cellular

47 e properties of isolated human fetal cardiac myofibrils across 8-19 weeks of gestation.

48 ion partner, has been recently implicated in myofibril actin cytoskeleton differentiation, and the my

49 We evaluated studies looking into myofibril activation, relaxation, and force changes prod

50 During myofibril activation, sarcomeres develop forces that are

51 in individual sarcomeres in real time before myofibril activation.

52 vity of force, tension cost, LDA, and single myofibril activation/relaxation parameters were measured

53 filaments in individual sarcomeres within a myofibril affects force production.

54 Myofibrils also showed an apparent increase in Ca(2+) se

55 By assuming that structurally registered myofibrils also tend to beat in phase, we explain the ob

56 actomyosin interactions within an activated myofibril and depleting the thick filaments in one sarco

57 Small-scale approaches such as isolated myofibril and isolated contractile protein biomechanical

58 ofibrillar myopathy that is characterized by myofibril and Z-disc disruption.

59 e second harmonic generation from A-bands of myofibrils and 2-photon fluorescence from fluo-4.

60 -/-) mice at E17.5, with short, disorganized myofibrils and cardiomyocytes that fail to align in the

61 For proteins from heart myofibrils and cerebrospinal fluid (CSF), compared to on

62 Although myofibrils and desmin filaments were intact at 7 d after

63 ocytes resulted in profound malformations of myofibrils and focal adhesions accompanied by adhesion-d

64 ular to their z-lines, which couple parallel myofibrils and give cardiac and skeletal myocytes their

65 to high salt only increased water-holding of myofibrils and hence did not reproduce myofibrillar prot

66 ibuted mitochondria between poorly organized myofibrils and increased polyubiquitinated protein and a

67 aw loss, decreased water-holding capacity of myofibrils and increased surface hydrophobicity, indicat

68 thawing minced pork reduced water-holding of myofibrils and increased surface hydrophobicity.

69 SDS-PAGE, further decreased water-holding of myofibrils and increased surface hydrophobicity.

70 e generation and transmission of force along myofibrils and lead to myopathy, the mechanism whereby m

71 ith 3D electron microscopy data (~ 30 nm) of myofibrils and mitochondria, both collected from adult r

72 and energetics are related in single cardiac myofibrils and multicellular cardiac muscle strips of th

73 ontractile properties of human fetal cardiac myofibrils and myosin across gestational age 59-134 days

74 sembly, with adults having severely abnormal myofibrils and no flight ability.

75 ) sensitivity of ATPase activity in isolated myofibrils and reconstituted hybrid sarcomeres containin

76 great advance in experiments using isolated myofibrils and sarcomeres that has allowed scientists to

77 the sarcoplasmic reticulum (SR) surrounding myofibrils and specialized for Ca(2+) storage, release,

78 shortening rates similar to those of in vivo myofibrils and stress fibers.

79 etween a dense apical network of filamentous myofibrils and the assembly of basally localized myofibr

80 of the number of contractions imposed on the myofibrils and their initial sarcomere length.

81 ntify sarcomere length from videos of moving myofibrils and to analyze loss of synchronicity of beati

82 oviding a mechanical tether between adjacent myofibrils and to the extracellular matrix and that the

83 If growing isoforms dominate, myofibrils and Z-discs enlarge, eventually resulting in

84 then tend to bind to muscle components like myofibrils and/or biomembranes.

85 u alone on MPS (by tracer incorporation into myofibrils), and for HMB we also measured muscle proteol

86 ed for cTnI(R146G)- and cTnI(R21C)-exchanged myofibrils, and Ca(2+) sensitivity of tension (pCa50) wa

87 troponin and exchanged into rat ventricular myofibrils, and contraction/relaxation kinetics were mea

88 ents with disrupted and haphazardly arranged myofibrils, and edematous mitochondria with loss of cist

89 vealed pathological changes in mitochondria, myofibrils, and mitochondrion-associated membranes in sk

90 e explants, isolated cardiomyocytes, skinned myofibrils, and purified actin/myosin preparations have

91 In severe RV dysfunction, both myofibril- and fibrosis-mediated stiffness contribute to

92 Myofibrils are huge cytoskeletal assemblies embedded in

93 creases following denervation, at times when myofibrils are rapidly degraded.

94 ril; 2) Sequential addition with an existing myofibril as a template; and 3) Longitudinal splitting o

95 disc is critical for fully comprehending how myofibrils assemble and function.

96 together with their interaction partners) in myofibril assembly and after muscle damage.

97 s complete loss of Tmods leads to failure of myofibril assembly and developmental defects.

98 actin sliding velocity, as well as abnormal myofibril assembly compared to cardioblast myosin (EMB-1

99 The double mutation suppresses myofibril assembly defects in pupal indirect flight musc

100 wed normal (Y583S) or altered (T178I, R672C) myofibril assembly followed by progressive disruption of

101 as the H252Q mutation significantly enhances myofibril assembly in comparison with the non-mutant pro

102 In contrast, all alleles permitted normal myofibril assembly in the heterozygous state but caused

103 n filaments in skeletal muscle revealed that myofibril assembly is defective and disorganized in doub

104 Indirect flight muscle myofibril assembly was minimally affected in mutant homo

105 results indicated that TpnC is required for myofibril assembly, and that there is functional special

106 Thus, altered myosin function permits myofibril assembly, but results in a progressive disrupt

107 pupal indirect flight muscles display normal myofibril assembly, myofibril shape, and double-hexagona

108 Myofibril assembly, skeletal muscle structure, and thin

109 restingly, I508K disabled motor function and myofibril assembly, suggesting that productive relay-con

110 During myofibril assembly, thin filament lengths are precisely

111 e pupae from each mutant displayed disrupted myofibril assembly, with adults having severely abnormal

112 ndicating an involvement of both proteins in myofibril assembly.

113 pression of muscle contractile proteins, and myofibril assembly.

114 n of gene transcription, ubiquitination, and myofibril assembly.

115 ocytes consist primarily of desmin, surround myofibrils at Z disks, and transmit forces from the cont

116 mitochondria are densely packed adjacent to myofibrils because adenosine triphosphate (ATP) is neede

117 If blocking isoforms dominate, myofibrils become smaller.

118 ilaments by Trim32, which leads to the later myofibril breakdown by enzymes, whose expression is incr

119 We show that myofibril breakdown is a two-phase process involving the

120 The myofibril breakdown normally observed at 14 d after dene

121 e lower contractile force and have perturbed myofibril bundling compared with controls expressing bot

122 ation decreased pCa50 for cTnI(WT)-exchanged myofibrils but not for either mutation.

123 mechanical behavior of rabbit psoas skeletal myofibrils by replacing endogenous Tm and troponin (Tn)

124 tion of the Ca(2+) sensitivity of ACTC E361G myofibrils by sarcomere length or EMD57033 was indisting

125 t cardiac ECs actively express cardiomyocyte myofibril (CMF) genes and have open chromatin at CMF gen

126 In the heart myofibrils, common protein proteoforms observed were ass

127 ke myofibrils with many individual, parallel myofibrils comprising the bulk of the muscle cell volume

128 cells did not increase their surface area or myofibril content during the observed timeframe.

129 was reduced, cell surface area expansion and myofibril content increase were both dampened, and the b

130 ving coordination of cell size expansion and myofibril content increase.

131 ls of Mef2c and slow myosin heavy chain, and myofibril content.

132 rends toward increased cell surface area and myofibril content.

133 f antiparallel F-actin is tightly coupled to myofibril contraction.

134 anged with the native LC1 of skeletal muscle myofibrils cross-linked with 1-ethyl-3-[3(dimethylamino)

135 te stiffness and applied overstretch induced myofibril defects in 7:1 hPSC-CMs and decreased mechanic

136 The severity of myofibril defects in heterozygotes correlated with the l

137 nsmission electron microscopy (TEM) revealed myofibril degeneration, disorganized mitochondrial crist

138 muscle-restricted depletion of TRAF6 rescues myofibril degradation and preserves muscle fiber size an

139 d FA protein ubiquitination and degradation, myofibril degradation, and subsequent down-regulation of

140 However, myofibrils degraded during aging, correlating with reduc

141 to those of adult ventricular tissue, higher myofibril density and alignment, improved calcium handli

142 diomyocytes was reduced because of decreased myofibril density.

143 that the force produced by a skeletal muscle myofibril depends on its cross-sectional area but not on

144 oss of desmin is critical for the subsequent myofibril destruction, and over time, myofibrillar prote

145 To study the order of events leading to myofibril destruction, we investigated the slower atroph

146 Myotubes, characterized by myofibril development and both spontaneous and stimuli-e

147 terns and nonredundant roles, functioning in myofibril development and maintenance, and provide the f

148 udinally oriented structures associated with myofibril development and remodeling.

149 ith nebulin/nebulette during early stages of myofibril development that is lost upon further maturati

150 Within a muscle type, myofibril diameter is highly invariant and contributes t

151 nown about the underlying mechanisms setting myofibril diameter.

152 vity and relaxation parameters of ACTC E361G myofibrils did not depend on the troponin I phosphorylat

153 A crucial step during myofibril differentiation is the sequential exchange of

154 n regulating the alpha-actin exchange during myofibril differentiation.

155 ofilin2 cooperate in actin regulation during myofibril differentiation.

156 ncluding atrial and ventricular enlargement, myofibril disarray, fibrosis and mitochondrial injury, a

157 myocytes induced murf1 expression and caused myofibril disarray, whereas inhibiting Calcineurin activ

158 get gene encoding the p97/VCP ATPase reduced myofibril disassembly and degradation on denervation or

159 ation of polyubiquitin aggregates [6, 7] and myofibril disorganization [8, 9].

160 culminating with contraction impairment and myofibril disruption in cardiomyocytes.

161 rect flight muscles and dramatically reduces myofibril disruption in young adults.

162 ining sarcomeres and transmits tension along myofibrils during muscular contraction.

163 d for tracking cell surface area changes and myofibril dynamics in live embryos.

164 ibility to observe water located outside the myofibrils, easily lost upon storage or cooking.

165 ectron micrographs showed human fetal muscle myofibrils elongate and widen with age, but features suc

166 the early slow phase relaxation for cTnI(WT) myofibrils, especially at Ca(2+) levels that the heart o

167 The results suggest that the myofibrils exhibit nonlinear viscoelastic properties tha

168 oelastic properties that may be derived from myofibril filaments, similar to what has been observed i

169 ic organization and functional remodeling of myofibrils, focal adhesions, and intercalated discs as c

170 Our results suggest that the myofibril force is affected by intersarcomere dynamics a

171 Myofibril force measurements revealed that microgravity

172 Together, our results suggest that cardiac myofibril force production and kinetics of activation an

173 Comparison of switch-off kinetics with myofibril force relaxation kinetics measured in a mechan

174 We investigate the potential roles in myofibril formation and repair of formin proteins, which

175 l muscle sarcomeres are not fully formed but myofibril formation is visible.

176 tions A230P and A1366D significantly disrupt myofibril formation, whereas the H252Q mutation signific

177 c tension.Unexpectedly, training reduced the myofibril fractional area of muscle fibres in both group

178 tension data were corrected for the loss of myofibril fractional area, we observed an increase in te

179 ins, smaller muscle fibre diameter and lower myofibril fragmentation of LW meat, as compared to other

180 In this study we isolated cardiac myofibrils from 3 TTNtv mutants, and 3 with contractile

181 Native myofibrils from ACTC E361G transgenic mice had a 2.4-fol

182 adult rat ventricular myocytes (ARVMs), and myofibrils from both sexes of rats and observed function

183 from TnC, cross-bridge detachment varied in myofibrils from different species and was rate-limited b

184 n and relaxation kinetics on isolated single myofibrils from heart slices after culture.

185 of titin in guiding the assembly of nascent myofibrils from premyofibrils.

186 ificantly smaller in the contracting cardiac myofibrils from Tg-R58Q mice then in control Tg-wild typ

187 tivation and relaxation kinetics of isolated myofibrils from two adult individuals with an R672C subs

188 is unique to AQM, while the dysregulation of myofibril genes, determinant of the mechanical propertie

189 nent of Z-discs, mediates Z-disc and thereby myofibril growth through protein oligomerization.

190 Human fetal cardiac myofibrils have low force and slow kinetics of activatio

191 to mechanical stimulation, being central to myofibril homeostasis and development.

192 he specific targeting of muscle FHOD3 to the myofibrils in cardiomyocytes is abolished in phosphomuta

193 is expressed and incorporated into organized myofibrils in hearts and that its level is increased in

194 his isoform is expressed and integrated into myofibrils in human CMs.

195 three-dimensional structural organization of myofibrils in physiological and proteolysed muscle.

196 we measured the stiffness of skeletal muscle myofibrils in rigor.

197 b-binding protein Smyd1b impair formation of myofibrils in skeletal muscle and lead to the accumulati

198 ucture and function of both mitochondria and myofibrils in skeletal muscle tissues engineered on micr

199 sarcomere is the smallest functional unit of myofibrils in striated muscles.

200 ibrils and the assembly of basally localized myofibrils in ventricular cardiomyocytes.

201 Thus, in contrast to their role in muscle myofibrils, in non-muscle cells, Tmods bind actin-tropom

202 Incorporating sTn into myofibrils increased the off-rate and lowered the Ca(2+)

203 AJ disrupted junction morphology and blocked myofibril integration at cell-cell contacts.

204 vinculin recruitment was required to rescue myofibril integration at nascent contacts.

205 mechanism by which Ca(2+) overload disrupts myofibril integrity by activating a Calcineurin-FoxO-MuR

206 or inhibiting proteasome activity preserved myofibril integrity, revealing a MuRF1-mediated proteaso

207 egulation of its expression severely affects myofibril integrity.

208 he impacts of impaired Ca(2+) homeostasis on myofibril integrity.

209 colemmal mitochondria, and those between the myofibrils, interfibrillar mitochondria.

210 2) the mechanical work of one sarcomere in a myofibril is transmitted to other sarcomeres in series.

211 p the hypotheses that 1) force production in myofibrils is largely altered and regulated by intersarc

212 We conclude that force production in myofibrils is largely regulated by intersarcomere dynami

213 Myofibrils isolated from rabbit psoas were activated and

214 Myofibrils isolated from TG(S282A) hearts displayed robu

215 d skeletal muscle pathology in myofibers and myofibrils isolated from young hetero- and homozygous R3

216 microscopy was performed using mouse cardiac myofibrils labeled with antibodies specific to the N- an

217 ation of mitochondrial networks aligned with myofibril lattices.

218 Moreover, instability of P838L myofibrils leads to decreased function during aging of D

219 However, their fetal-like misalignment of myofibrils limits their usefulness for modeling contract

220 myocyte diameters, myocytolysis (perinuclear myofibril loss), accumulation of perinuclear glycogen, i

221 promoting both cardiomyocyte enlargement and myofibril maturation, enhancing the extent of cardiomyoc

222 Isolated myofibril mechanical measurements revealed much lower sp

223 The relative contribution of fibrosis- and myofibril-mediated RV stiffness was determined in RV tra

224 lative contribution of fibrosis-mediated and myofibril-mediated stiffness in rats with mild and sever

225 ffness, whereas in mild RV dysfunction, only myofibril-mediated stiffness was increased in comparison

226 ed fibrosis-mediated stiffness and increased myofibril-mediated stiffness, whereas in mild RV dysfunc

227 arcomeres were poorly formed and the general myofibril network was less stable, incomplete, and/or to

228 s line revealed architectural differences in myofibrils of the distinct cardiomyocyte subtypes.

229 Knockdown phenotypes include global loss of myofibril organization and defective sarcomeric ultrastr

230 re cytotoxic to myotubes and disrupted their myofibril organization compared with desmin monomer or o

231 which exhibit normal thin filament lengths, myofibril organization, and skeletal muscle contractile

232 n (Lmod) isoforms Lmod2 and Lmod3 to control myofibril organization, thin filament lengths, and actom

233 ship between the generated stress and global myofibril organization.

234 The decrease in myofibril passive stiffness was a common feature in all

235 Myofibrils photolabeled with AziPm and Azi-iso identifie

236 g capacity, structural water associated with myofibrils) pointed to evident protein aggregation and l

237 ith relaxation kinetics in the corresponding myofibril preparations.

238 ted by these free radicals were estimated on myofibrils prepared from pork rectus femoris muscle.

239 Our studies demonstrate that myofibrils progressively unbundle in flies that lack Thi

240 Protein kinase A (PKA) phosphorylation of myofibril proteins constitutes an important pathway for

241 the kinetics of thin filament activation and myofibril relaxation as Ca(2+) levels vary.

242 as delayed in systemic CAP2 mutant mice, and myofibrils remained in an undifferentiated stage at the

243 ink between Erbb2 activity and remodeling of myofibrils, revealing an unexpected mechanism with poten

244 ontractile and the elastic elements within a myofibril rules the intersarcomere dynamics, with import

245 In the reconstruction volume, myofibrils, sarcomeric organization, and mitochondria ca

246 Rabbit cardiac myofibrils separated by two-dimensional isoelectric focu

247 t muscles display normal myofibril assembly, myofibril shape, and double-hexagonal arrangement of thi

248 hen purposely altered water distribution and myofibrils shape by means of freezing.

249 le myosin activity repressed the assembly of myofibrils, showing that subcellular tension drives the

250 It is surprising that myofibril-specific force from both control and flight MA

251 Myofibril stability is required for normal muscle functi

252 Mutations that disrupt myofibril stability result in individuals who develop pr

253 defects in myosin ATPase, in vitro motility, myofibril stability, and muscle function associated with

254 eviously unknown role for TRIM32 proteins in myofibril stability.

255 Furthermore, the myofibril stiffness during shortening was greater than t

256 During stretching, the myofibril stiffness was independent of both displacement

257 During shortening, the myofibril stiffness was independent of displacement, but

258 ocytes demonstrated a gradual increase in RV myofibril stiffness, which was partially restored by pro

259 stiffness is mainly determined by increased myofibril stiffness.

260 is dependent on the sarcomere length and the myofibril stiffness.

261 sed power was attributed in part to improved myofibril structure (increased sarcomere length and Z-ba

262 l cells markedly enhanced the contractility, myofibril structure and calcium handling of human engine

263 ovel roles for BAG3 and Hsc70 in stabilizing myofibril structure and inhibiting myofibrillar degenera

264 reased actin sliding velocity and stabilizes myofibril structure compared to EMB.

265 thickness that was accompanied by disrupted myofibril structure in adult flies.

266 la indirect flight muscle (IFM) and assessed myofibril structure, skinned fibre mechanical properties

267 Pase activities, in vitro actin motility and myofibril structure/stability.

268 xpected, mechanical stretch rapidly disrupts myofibril structures in bag3 knockdown cardiomyocytes.

269 Force measurements on myofibrils substituted with C-terminal truncated TnI sho

270 , we employed a rapid solution-switch single myofibril technique that allows for the study of contrac

271 Single cardiomyocytes contain myofibrils that harbor the sarcomere-based contractile m

272 In nontransgenic mouse myofibrils, the Ca(2+) sensitivity of force was increase

273 In R672C-containing muscle myofibrils, the initial, slower phase of relaxation had

274 ayed by the mitochondria located between the myofibrils, the interfibrillar mitochondria.

275 sume a uniform behavior of sarcomeres within myofibrils, the occurrence of sarcomere length nonunifor

276 In mature myofibrils, this interaction is limited to longitudinall

277 eins are concentrated at attachment sites of myofibrils to the membrane.

278 g development they are localized to immature myofibrils together with their binding partner, filamin

279 shortening than males, and female ARVMs and myofibrils took longer to relax.

280 nimal histological abnormalities with intact myofibril ultrastructure.

281 oskeletal connections between the Z-disc and myofibrils under mechanical stress.

282 tropy for adenine nucleotides in the cardiac myofibril, using homogenization theory and atomistic thi

283 dge slow relaxation kinetics in single R403Q myofibrils was significantly higher (P < 0.0001) than in

284 In permeabilized cells/myofibrils, we found robust myofilament length-dependent

285 sing a custom-built atomic force microscope, myofibrils were first placed in a rigor state then stret

286 on of major structural gene transcripts, and myofibrils were formed.

287 Rat ventricular myofibrils were isolated and endogenous cTn was exchange

288 In the relaxed state, myofibrils were labeled by anti-pSer-35 but not by anti-

289 function depends on the proper formation of myofibrils, which are tandem arrays of highly organized

290 ay be limited by the structural order of the myofibrils, which in turn is regulated by their elastic

291 res essential biophysical characteristics of myofibrils while lacking numerous molecular constituents

292 ion system to control one sarcomere within a myofibril, while measuring the individual behavior of al

293 ear effect was observed on force relaxation: myofibrils with D137L/G126R or D137L Tm showed prolonged

294 The force produced by myofibrils with intact sarcomeres was significantly high

295 long the length of the muscle into tube-like myofibrils with many individual, parallel myofibrils com

296 nificantly higher than the force produced by myofibrils with one or more sarcomeres lacking thick fil

297 adults show more severe cracking and frayed myofibrils with some disruption of the myofilament latti

298 Treatment of myofibrils with the AMPK holoenzyme increased cTnI Ser-1

299 formation of extended sarcomeric arrays, or myofibrils, within a large volume of cytoplasm.

300 evealed severe myocyte damage with elongated myofibrils without gross necrosis.