ライフサイエンスコーパス: myofibril

1 molecules of actin in working ex vivo heart myofibril.

2 agated from sarcomere to sarcomere along the myofibril.

3 and 3) Longitudinal splitting of an existing myofibril.

4 composed of tightly-packed mitochondria and myofibrils.

5 time course of relaxation in cardiac muscle myofibrils.

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

7 indistinguishable from that of nontransgenic myofibrils.

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

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

10 ntractility in single transgenic mouse heart myofibrils.

11 in assembly factors being required to repair myofibrils.

12 ylation in their hearts before isolating the myofibrils.

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

14 either fetal or adult human skeletal muscle myofibrils.

15 incorporating the complex into rabbit psoas myofibrils.

16 rom several different species of ventricular myofibrils.

17 97 in extracting ubiquitinated proteins from myofibrils.

18 roximately three) in working skeletal muscle myofibrils.

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

20 1.5, and maintains the alignment of adjacent myofibrils.

21 to a similar extent in cTn compared with sTn myofibrils.

22 quitylates MyBP-C, MyLC1, and MyLC2, even in myofibrils.

23 ssembly and structural integrity of striated myofibrils.

24 ut similar pCa 4 activity to, sTn-containing myofibrils.

25 membranated rabbit psoas fibres and isolated myofibrils.

26 nascent myofibrils and culminating in mature myofibrils.

27 cterized by aggresome-like inclusions in the myofibrils.

28 e methods, to examine its effect on maturing myofibrils.

29 marker protein, in both skeletal and cardiac myofibrils.

30 ic localization of Smn is conserved in mouse myofibrils.

31 arcomere structure in indirect flight muscle myofibrils.

32 quitinated proteins and rapid destruction of myofibrils.

33 re investigated in calcium-activated cardiac myofibrils.

34 g contractions, and of fluorescently labeled myofibrils.

35 ng decreases mitochondrial content among the myofibrils.

36 phosphate transfer from the mitochondria to myofibrils.

37 oups using single muscle fibers and isolated myofibrils.

38 ificant reduction in muscle mass and thinner myofibrils.

39 osphodiesterase activity associated with the myofibrils.

40 that involve shortening of sarcomeres along myofibrils.

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

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

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

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

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

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 During myofibril activation, sarcomeres develop forces that are

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

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

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

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

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

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

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

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

57 rmation of premyofibrils followed by nascent myofibrils and culminating in mature myofibrils.

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

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

60 n filaments in sarcomeres of striated muscle myofibrils and in the erythrocyte membrane skeleton.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

78 d and sarcomere lengths found in the hinge B myofibrils appear to be due to the longitudinal addition

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

80 However, premyofibril and nascent myofibril arrays have not been detected in early cardiom

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

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

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

84 ntly as a "scaffold and ruler" system during myofibril assembly and as an elastic protein during stre

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

86 Myofibril assembly and disassembly are complex processes

87 demonstrate that skNAC plays a vital role in myofibril assembly and function during muscle cell diffe

88 nity, abolition of actin sliding, defects in myofibril assembly and rapid degeneration of muscle stru

89 proteins, UNC-98 and UNC-96, are involved in myofibril assembly and/or maintenance, especially myosin

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

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

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

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

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

95 sttranslational microtubule modification and myofibril assembly, and is only partly compensated by up

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

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

98 ctural genes whose products are required for myofibril assembly, function, and regulation.

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

100 Myofibril assembly, skeletal muscle structure, and thin

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

102 During myofibril assembly, thin filament lengths are precisely

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

104 ndicating an involvement of both proteins in myofibril assembly.

105 pression of muscle contractile proteins, and myofibril assembly.

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

107 erlap of the thin and thick filaments during myofibril assembly.

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

109 To further characterize these differences, myofibril ATPase activity as a function of pCa and label

110 off) on maximal activation and relaxation in myofibrils because they allow rapid and large changes in

111 ht chains 1 and 2 (MyLC1 and MyLC2) from the myofibril, before any measurable decrease in myosin heav

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

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

114 The myofibril breakdown normally observed at 14 d after dene

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

116 MyHC is protected from ubiquitylation in myofibrils by associated proteins, but eventually underg

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

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

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

120 ment of ATPase activity showed that skeletal myofibrils containing >96% cTn had a higher pCa 9 ATPase

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

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

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

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

125 rends toward increased cell surface area and myofibril content.

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

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

128 FLNc, CAP provides another link between the myofibril cytoskeleton and the plasma membrane of muscle

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

130 iated with a cellular hypertrophic response, myofibril degeneration, and a marked decrease in cardiac

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

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

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

134 However, myofibrils degraded during aging, correlating with reduc

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

136 diomyocytes was reduced because of decreased myofibril density.

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

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

139 esidual flight ability decreased rapidly and myofibrils developed peripheral defects.

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

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

142 udinally oriented structures associated with myofibril development and remodeling.

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

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

145 d localisation of sarcomeric proteins, gross myofibril disarray and growth-retarded hearts.

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

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

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

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

150 culminating with contraction impairment and myofibril disruption in cardiomyocytes.

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

152 sate for the reduced membrane linkage of the myofibrils due to the loss of the dystroglycan-sarcoglyc

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

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

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

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

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

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

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

160 g myosin folding and assembly into organized myofibril filaments.

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

162 Myofibril force measurements revealed that microgravity

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

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

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

166 isms that regulate sarcomere assembly during myofibril formation are poorly understood.

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

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

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

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

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

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

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

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

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

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

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

178 ronment of an active cross-bridge in cardiac myofibrils from transgenic (Tg) mice is altered by the D

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

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

181 signaling pathways that ultimately result in myofibril growth.

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

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

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

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

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

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

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

189 yzed zebrafish embryos with poorly organized myofibrils in skeletal muscles.

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

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

192 Ultrastructural analysis of the myofibrils in wild-type and Ate1 knockout mouse hearts s

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

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

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

196 egulation of its expression severely affects myofibril integrity.

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

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

199 ith the dramatic restructuring of peripheral myofibrils into concentric rings.

200 muscle proteins is coordinated to build the myofibril is unknown.

201 eins assemble into sarcomeric arrays to form myofibrils is controversial.

202 which disintegration of Z disks and then of myofibrils is followed by ectopic accumulation of multip

203 Myofibrils isolated from TG(S282A) hearts displayed robu

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

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

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

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

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

209 Isolated myofibril mechanical measurements revealed much lower sp

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

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

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

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

214 tion, the actomyosin motor is assembled into myofibrils, multiprotein machines that generate and tran

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

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

217 hing of four or five molecules of actin of a myofibril on Olympus coverslips coated with SIF decrease

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

219 monstrated that Hsp90alpha1 is essential for myofibril organization in skeletal muscles of zebrafish

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

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

222 pha2 had no effect on muscle contraction and myofibril organization.

223 ship between the generated stress and global myofibril organization.

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

225 operative (pCa50 = 6.14, nH = 1.46) than sTn myofibrils (pCa50= 5.90, nH = 3.36).

226 Myofibrils photolabeled with AziPm and Azi-iso identifie

227 ith relaxation kinetics in the corresponding myofibril preparations.

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

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

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

231 sible for the discrimination of CMs, whereas myofibril proteins have a lesser contribution.

232 g the existence of feed back regulation from myofibril proteins to CSN-5 protein levels.

233 ling factors; Acta1, Acta2, Myl3, and Myom1, myofibril proteins; and calcium-activated potassium-chan

234 HL-null hearts developed lipid accumulation, myofibril rarefaction, altered nuclear morphology, myocy

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

236 pport system that surrounds and supports the myofibril result in dilated cardiomyopathy and congestiv

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

238 ion of SALS and other sarcomeric proteins in myofibrils reveals that the full length of thin filament

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

240 Rabbit cardiac myofibrils separated by two-dimensional isoelectric focu

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

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

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

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

245 Myofibril stability is required for normal muscle functi

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

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

248 eviously unknown role for TRIM32 proteins in myofibril stability.

249 se approaches enabled us to detect the three myofibril stages in developing hearts supporting a three

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

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

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

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

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

255 stiffness is mainly determined by increased myofibril stiffness.

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

257 ular structure of the rod, subtle changes in myofibril structure and decreased ability to maintain sa

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

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

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

261 bsence of arginylation results in defects in myofibril structure that delay their development and aff

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

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

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

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

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

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

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

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

270 In mature myofibrils, this interaction is limited to longitudinall

271 eir development and affect the continuity of myofibrils throughout the heart, predicting defects in c

272 formation and a failure in the attachment of myofibrils to membrane complexes.

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

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

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

276 nimal histological abnormalities with intact myofibril ultrastructure.

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

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

279 nction by overexpressing CapZbeta2 increased myofibril vulnerability and fragmentation under mechanic

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

281 tion of pCa and labeled Tn exchange in rigor myofibrils was used to estimate Tn dissociation rates fr

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

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

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

285 Rat ventricular myofibrils were isolated and endogenous cTn was exchange

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

287 pCa-ATPase activity relation showed that cTn myofibrils were more calcium sensitive but less cooperat

288 Skeletal muscle myofibrils were used as an example, because they contain

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 In myofibrils with M80Q sTnC(F27W) (decreased k(off)), maxi

295 the cardiomyocytes fail to assemble striated myofibrils with regulated F-actin lengths.

296 d down by RNAi, primary muscles display thin myofibrils with shortened sarcomeres and increased sarco

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.