ライフサイエンスコーパス: sarcomere

1 d the actin filaments in the skeletal muscle sarcomere.

2 contractile unit in striated muscles is the sarcomere.

3 hat the mutation should reduce the force per sarcomere.

4 ts on the regulatory function of the cardiac sarcomere.

5 erminus to distinct regions (C-zones) of the sarcomere.

6 gy landscape, and the generated force by the sarcomere.

7 on of Nebulin, an essential component of the sarcomere.

8 inding the myosin cross-bridges (XBs) in the sarcomere.

9 nteraction of beta-arrestin localized to the sarcomere.

10 meres that links integrins ultimately to the sarcomere.

11 ue spatial arrangement of cMyBP-C within the sarcomere.

12 ay from the A-band towards the Z-disk of the sarcomere.

13 on of the basic unit of the muscle cell, the sarcomere.

14 the structure and organization of C. elegans sarcomeres.

15 e development of large inhomogeneities among sarcomeres.

16 trix linked together by frequently branching sarcomeres.

17 expression of structural genes and prominent sarcomeres.

18 ffusion and stress transfer between adjacent sarcomeres.

19 tension between actin filaments in adjoining sarcomeres.

20 I myosins are critical components of cardiac sarcomeres.

21 py and by measuring force development of the sarcomeres.

22 function of the underlying muscle fibers and sarcomeres.

23 expulsion, contains bilateral dorsal-ventral sarcomeres.

24 micrometer-scale contractile machines called sarcomeres.

25 The unc-22(sf21) worms have well-organized sarcomeres.

26 ile measuring the individual behavior of all sarcomeres.

27 of its longitudinal tubules across adjacent sarcomeres.

28 f contractile proteins into highly organized sarcomeres.

29 where it contributes to the organization of sarcomeres.

30 n the cKO mice, with NMN treatment restoring sarcomere alignment but not mitochondrial morphology.

31 "Give" then propagated from sarcomere to sarcomere along the myofibril.

32 actile mechanisms that involve shortening of sarcomeres along myofibrils.

33 intermediate filaments that complex with the sarcomere, altering myocyte stiffness, contractility, an

34 l handling of images, the mechanism by which sarcomere and by extension z-line architecture can impac

35 vides evidence that KBTBD13 localizes to the sarcomere and can directly bind actin.

36 imus mutants in Drosophila exhibit disrupted sarcomere and chromosome structure, suggesting that gian

37 tion between H3K27me3 deposition and reduced sarcomere and cytoskeletal gene expression in proliferat

38 pathy, which is often caused by mutations in sarcomere and cytoskeletal proteins and is also associat

39 genes encoding structural components of the sarcomere and desmosome.

40 r correlation suggests PTM cross-talk in the sarcomere and dysregulation of protein kinase A pathways

41 hick filaments bind to thin filaments in the sarcomere and generate pulling forces.

42 ural component at the lateral borders of the sarcomere and is important for mechanical stability and

43 We propose that these early sarcomere and metabolic defects alter cardiac function a

44 ant mice, which display lethal disruption of sarcomeres and aberrant expression of muscle structural

45 se organization of contractile proteins into sarcomeres and coupling of the contractile apparatus to

46 reveal a mechanism whereby KLHL41 stabilizes sarcomeres and maintains muscle function by acting as a

47 tron microscopy revealed significant loss of sarcomeres and mitochondria and increased collagen and g

48 residues) localized to the M-lines of muscle sarcomeres and required for normal sarcomere organizatio

49 erised by a lower beating rate, disorganised sarcomeres and sarcoplasmic reticulum and a blunted resp

50 e more upstream mutation, TTN-Z(-/-)-CMs had sarcomeres and visibly contracted, whereas TTN-A(-/-)-CM

51 of transverse tubules (TTs) that align with sarcomeres and Z-lines as well as longitudinal tubules (

52 at different displacements (0.1-0.3 mum per sarcomere) and nominal speeds (0.4 and 0.8 mum/s).

53 yocytes localized to the level of A-bands in sarcomeres, and Myo18a knockout embryos at day 10.5 exhi

54 works, including stress fibers (SFs), muscle sarcomeres, and the cytokinetic ring to both generate an

55 od, the muscle sex-specifically remodels its sarcomeres anteriorly-posteriorly to promote copulation

56 Force and shortening in the muscle sarcomere are due to myosin motors from thick filaments

57 Sarcomeres are connected in series through a network of

58 e a reduced myofibrillar fractional-area and sarcomeres are disorganized, contain rod bodies, and hav

59 Sarcomeres are extremely highly ordered macromolecular a

60 The thin and thick filaments of muscle sarcomeres are interconnected by the giant protein titin

61 riants in myosin, the molecular motor of the sarcomere, are clustered in residues that participate in

62 Sarcomeres arise from muscle stress fibers (MSFs) that a

63 relationship between cell-ECM adhesions and sarcomeres assembling de novo remains untested.

64 Defective sarcomere assembly in smyd1a and smyd1b zebrafish mutant

65 les, and loss of Smyhc1 results in defective sarcomere assembly, reduces larval motility and fish sur

66 that are subsequently capped by Tmods during sarcomere assembly, turnover, and repair.

67 ed that cell-ECM adhesions are important for sarcomere assembly.

68 between the extent of cell-ECM adhesion and sarcomere assembly.

69 phy, including an ordered disassembly of the sarcomere associated with upregulation of the E3 ubiquit

70 Mutations in over a dozen genes encoding sarcomere-associated proteins cause HCM.

71 ctional area but not on the number of active sarcomeres because they are arranged in series.

72 traction is due to tension generated in each sarcomere between overlapping arrays of actin and myosin

73 yofibrillar networks though the frequency of sarcomere branching goes down from early to late postnat

74 myocytes differentiate and harbour organized sarcomeres but are fusion-incompetent.

75 The Ca(2+) sensor of the sarcomere, cardiac troponin C (cTnC), plays an important

76 ts pulling nearby actin filaments toward the sarcomere centre.

77 is estimated from the relation between half-sarcomere compliance and force during the force redevelo

78 we have identified a previously unrecognized sarcomere component, a large novel isoform (denoted Myo1

79 Muscle sarcomeres contain giant polypeptides composed of multip

80 isolated myofibrils and reconstituted hybrid sarcomeres containing fast skeletal muscle troponin C.

81 ate and MSFs, allowing their maturation into sarcomere-containing myofibrils.

82 The platform should assess sarcomere content, contraction and relaxation parameters

83 The algorithm determines sarcomere content, sarcomere length, and returns rates o

84 TNNT2 variant-dependent changes in sarcomere contractile function induced graded regulation

85 induced muscle relaxation, muscle fiber- and sarcomere-contractility assays, low-angle x-ray diffract

86 essenger RNAs for nine genes responsible for sarcomere contraction and excitation-contraction couplin

87 tent, sarcomere length, and returns rates of sarcomere contraction and relaxation.

88 r dilated (DCM) cardiomyopathy by disrupting sarcomere contraction and relaxation.

89 at 2% dilutions reduced a multitude of mahi sarcomere contraction properties at various stimulation

90 ps, including an approximate 40% decrease in sarcomere contraction size and a nearly 50% reduction in

91 gest a novel therapeutic direction targeting sarcomere- cytoskeleton interactions to induce sarcomere

92 naling impairs MYH7-mediated, AMPK-dependent sarcomere-cytoskeleton filament interactions and plasma

93 chnology is able to capture adequate dynamic sarcomere data in vivo, and thus we lack fundamental dat

94 During myofibril activation, sarcomeres develop forces that are regulated through com

95 Median mitochondria-sarcomere diffusion distances correlated well with CK to

96 Mitochondria-sarcomere diffusion distances were calculated by using s

97 thy, a fatal muscle disorder associated with sarcomere disarray.

98 Progressive depletion of titin leads to sarcomere disassembly and atrophy in striated muscle.

99 y at the fbxl22 and ilk loci, which regulate sarcomere disassembly and cardiomyocyte protrusion into

100 ts display widespread cardiomyocyte mitosis, sarcomere disassembly and improved left ventricular syst

101 on of cardiomyocytes, such as the control of sarcomere disassembly to allow cytokinesis, require more

102 uding arrhythmia, atrial conduction defects, sarcomere disassembly, and altered cardiac metabolism.

103 muscle atrophy with reduced strength, severe sarcomere disassembly, and lethality from 2 weeks of age

104 bolic disease phenotypes in skeletal muscle; sarcomere disorganization, mitochondrial deformation, up

105 ting CMs utilizing physiologically-relevant, sarcomere displacement length-based contraction criteria

106 role for cMyBP-C N'-terminal domains to damp sarcomere-driven contractile waves (so-called spontaneou

107 rovide force balancing among serially linked sarcomeres during contraction.

108 uctural changes in FVB mouse skeletal muscle sarcomeres during relaxation after tetanus contraction.

109 emonstrates the first large-scale sensing of sarcomere dynamics in vivo, which is a necessary first s

110 ed compounds, force responses and individual sarcomere dynamics upon rapid increases or decreases in

111 weak actin-myosin interactions that underpin sarcomere dynamics.

112 upon rapid increase in [Pi] is determined by sarcomere dynamics.

113 owed rod-shaped morphology, highly organized sarcomeres, elevated cTnI expression and mitochondrial d

114 in that connects the thick filament with the sarcomere end, working as an I-band spring that accounts

115 animals have shown that mavacamten inhibits sarcomere force production, thereby reducing cardiac con

116 he isoforms it encodes in cardiomyocyte (CM) sarcomere formation and function.

117 ing human CMs and is able to support partial sarcomere formation in the absence of full-length titin.

118 We hypothesized that sarcomere formation was caused by the expression of a re

119 for ubiquitination, have been implicated in sarcomere formation.

120 s contractility, or only assess well-aligned sarcomeres found in mature cardiac tissues.

121 ebrate striated muscle where it spans half a sarcomere from the Z-disc to the M-band and is essential

122 a giant elastic protein that spans the half-sarcomere from Z-disk to M-band.

123 FoxO-mediated murf1 expression and protected sarcomeres from degradation in ncx1-deficient hearts.

124 led ATP molecules in relaxed skeletal muscle sarcomeres from rat soleus.

125 luding NPPB levels, directly correlated with sarcomere function and can be used to predict TNNT2 vari

126 ermore, Cronos titin is necessary for proper sarcomere function in human induced pluripotent stem cel

127 translate to electrostatic-based changes in sarcomere function to augment contractility in cardiac m

128 relationships between myosin conformations, sarcomere function, and cell biology, we assessed contra

129 fatty acid metabolism, calcium handling, and sarcomere function, and the activation of a stress-respo

130 nd efficient method to quantitatively assess sarcomere function.

131 NNT2 variants from wildtype controls using a sarcomere functional reporter engineered by inserting td

132 0% of patients with established disease, and sarcomere gene carriers can live to advanced ages withou

133 of HCM are also inconsistent with the single sarcomere gene hypothesis, such as regional left ventric

134 us cardiac disease ascribed solely to single sarcomere gene mutations.

135 The top variants at known sarcomere genes (TTN, MYH6) were associated with longer

136 d with recent discoveries of variants in the sarcomere genes MYH6 and MYL4 points to an important rol

137 (AF), including rare coding mutations in the sarcomere genes MYH6 and MYL4.

138 atients who were also sequenced for the main sarcomere genes.

139 ngation of a single sarcomere, the so-called sarcomere "give".

140 f the force-generating step but results from sarcomere "give".

141 Cross-bridge attachments in the sarcomere have been reported to exhibit a similar stiffn

142 Sarcomeres have long been considered to be arranged end-

143 Furthermore, stretch and sarcomere heterogeneity may modulate the susceptibility

144 ns and determining the relation between half-sarcomere (hs) compliance and force during the force dev

145 f cardiac myosin that targets the underlying sarcomere hypercontractility of hypertrophic cardiomyopa

146 -C is found only in a distinct region of the sarcomere, i.e., the C-zone, are SRX myosin similarly co

147 amics and that 2) the mechanical work of one sarcomere in a myofibril is transmitted to other sarcome

148 evere disorganization of all portions of the sarcomere in body wall muscle.

149 e sarcomere leads to adjustments of adjacent sarcomeres in a mechanism that is dependent on the sarco

150 directly, rapidly, and automatically tracks sarcomeres in beating cardiomyocytes.

151 By rapid measurement of hundreds of sarcomeres in each hiPSC-CM, SarcTrack provides large da

152 software that monitors fluorescently tagged sarcomeres in hiPSC-CMs.

153 Thousands of serially linked sarcomeres in muscle make the shortening (and the shorte

154 used to erase thick filaments in individual sarcomeres in real time before myofibril activation.

155 rsarcomere dynamics and the number of active sarcomeres in series.

156 omere in a myofibril is transmitted to other sarcomeres in series.

157 pproach for rapid manipulation of cMyBP-C in sarcomeres in situ.

158 n the structure and organization of the worm sarcomeres, indicating a crucial role of R805 in UCS-cli

159 the Z-line protein myopalladin implicated in sarcomere integrity.

160 The sarcomere is the smallest functional unit of myofibrils

161 subpopulations are spatially arranged in the sarcomere is unclear, although it has been proposed that

162 The uniaxial force produced by sarcomeres is ideally perpendicular to their z-lines, wh

163 all animals by the contraction of myriads of sarcomeres joined end to end by the Z-bands.

164 orce produced by myofibrils with one or more sarcomeres lacking thick filaments (p < 0.0001) irrespec

165 oarchitecture, markedly disrupts the lateral sarcomere lattice and distorts myofibrillar angular axia

166 We found that the force from one sarcomere leads to adjustments of adjacent sarcomeres in

167 ncrease in active force with the increase of sarcomere length (length-dependent activation).

168 t the application of a preload equivalent to sarcomere length (SL) = 2.2 mum is optimal for the maint

169 g substitutions strongly decreased the slack sarcomere length (SL) at submaximal activating [Ca(2+)]

170 accounts for the rise of passive force with sarcomere length (SL).

171 AT (norm)) and active tension at the resting sarcomere length (T (req), reflecting required contracti

172 bres from Rana esculenta (at 4 degrees C and sarcomere length 2.15 mum), small 4 kHz oscillations and

173 d stiffness remains constant in the range of sarcomere length 2.7-3.1 um, showing the ability of the

174 eres in a mechanism that is dependent on the sarcomere length and the myofibril stiffness.

175 ction to zero with just a few micrometers of sarcomere length change.

176 rdiomyocytes were characterized by measuring sarcomere length changes and calcium transients during e

177 ide fiber optic probe, we captured nanometer sarcomere length changes from thousands of sarcomeres on

178 tors during the diastole-systole cycle under sarcomere length control.

179 by an increase in maximal respiration and/or sarcomere length due to increased volume of individual m

180 We also developed novel methods to quantify sarcomere length from videos of moving myofibrils and to

181 lf-sarcomeres, preventing the development of sarcomere length inhomogeneity.

182 Here, we report dynamic sarcomere length measurement in vivo using a combination

183 comeres within myofibrils, the occurrence of sarcomere length nonuniformities has been well recognize

184 has allowed scientists to directly evaluate sarcomere length nonuniformity.

185 alpha-B crystallin shifted the Fpassive-sarcomere length relation downward to baseline in donor

186 Prestretch shifted the Fpassive-sarcomere length relation further upward in donor and up

187 Alkaline phosphatase shifted the Fpassive-sarcomere length relation upward only in donor.

188 PM had longer sarcomere length than did other muscles.

189 oss-sectional areas normalized to weight and sarcomere length were significantly smaller in the venti

190 The algorithm determines sarcomere content, sarcomere length, and returns rates of sarcomere contrac

191 ectrical stimuli, ultrastructure properties (sarcomere length, mitochondrial density, networks of tra

192 Finally, mdivi-1 increased sarcomere length, potentially due to mdivi-1-induced cha

193 t cellular deformation affects TT shape in a sarcomere length-dependent manner and on a beat-by-beat

194 cteristic of the diastole is adjusted to the sarcomere length-dependent systolic force.

195 rimental temperature and muscle single fiber sarcomere length.

196 izations can be affected by heterogeneity in sarcomere length.

197 imposed on the myofibrils and their initial sarcomere length.

198 n the transparent tail, which correlate with sarcomere length.

199 sence of 0.3 and 3.0 muM OM at two different sarcomere lengths (SLs), short SL (1.9 mum) and long SL

200 ions in the thin-filament regulatory unit to sarcomere-level activation dynamics.

201 Fast sarcomere-level mechanics in contracting intact fibres f

202 relative positions of filament ends, such as sarcomere-like organization.

203 by FTY720 would be of therapeutic benefit in sarcomere-linked HCM.

204 We combine sarcomere mechanics and micrometer-nanometer-scale X-ray

205 known how TnT mutation causes dysfunction of sarcomere microdomains and how these events contribute t

206 pomyosin, and limits binding of PKA to local sarcomere microdomains.

207 nsmission electron microscopy (TEM) revealed sarcomere misalignment and changes to mitochondrial morp

208 ntal analysis, we bidirectionally coupled 50 sarcomere models in series to model calcium diffusion an

209 stratifying variables were the presence of a sarcomere mutation and measures of LVWT.

210 42 participants with overt HCM [positive for sarcomere mutation and negative for LV hypertrophy]).

211 ticipants with preclinical HCM [positive for sarcomere mutation and negative for LV hypertrophy], and

212 characterize and assess phenotypic burden in sarcomere mutation carriers (genotype positive [G+]) and

213 y indicate early disease in preclinical HCM (sarcomere mutation carriers without LV hypertrophy).

214 Thirty-six percent had a sarcomere mutation identified, and 50% had any LGE.

215 s resting obstruction, whereas the other was sarcomere mutation negative and more likely had isolated

216 Those that were sarcomere mutation negative were more likely to have iso

217 One group was sarcomere mutation positive and more likely had reverse

218 phic cardiomyopathy, but also in carriers of sarcomere mutation without left ventricular hypertrophy,

219 duced ejection fraction, HCM patients with a sarcomere mutation, age, and hypertension.

220 ncluding 166 (40% of total) probands with no sarcomere mutation, that is, nonfamilial HCM.

221 Sarcomere mutation-positive patients were more likely to

222 valuate myocardial strain in carriers of HCM sarcomere mutation.

223 nic sarcomeric variants (HCM patients with a sarcomere mutation; 51% versus 43%, P<0.001) despite equ

224 limitations of this hypothesis suggest that sarcomere mutations alone do not adequately explain all

225 Sarcomere mutations and left ventricular (LV) hypertroph

226 Disease-causing sarcomere mutations are absent in ~70% of patients with

227 patients, was significantly associated with sarcomere mutations independent of age, and was extremel

228 esized that a negative family history and no sarcomere mutations represent a nonfamilial subgroup of

229 Pathogenic sarcomere mutations were identified in 22 (79%) of 28 pa

230 f outflow obstruction in young patients with sarcomere mutations.

231 r sarcomere length changes from thousands of sarcomeres on the sub-millisecond timescale during whole

232 rce necessary for contraction, assessment of sarcomere order is paramount in evaluation of cardiac an

233 nd a conserved regulator of body wall muscle sarcomere organization and organelle positioning.

234 of muscle sarcomeres and required for normal sarcomere organization and whole-animal locomotion.

235 on alone, namely, the complete disruption of sarcomere organization in slow and fast muscles.

236 maturation-associated markers important for sarcomere organization, cardiac excitability, and Ca(2+)

237 r results suggest that cMLCK plays a role in sarcomere organization, likely distinct from its role in

238 Overexpression of cMLCK promotes sarcomere organization, while the loss of cMLCK leads to

239 otypes of the survivors, as well as abnormal sarcomere organization.

240 t the Z-band, actin filaments from adjoining sarcomeres overlap and are cross-linked in a regular pat

241 e relatives screened between nonfamilial and sarcomere-positive groups.

242 major cardiac events (P=0.04) compared with sarcomere-positive HCM probands.

243 to equilibrate with in-series stronger half-sarcomeres, preventing the development of sarcomere leng

244 As sarcomeres produce the force necessary for contraction,

245 omyocytes respond to damage by disassembling sarcomeres, proliferating, and repopulating the injured

246 hypertrophic cardiomyopathy (HCM) caused by sarcomere protein (SP) gene mutations is current standar

247 hy (HCM) is caused by pathogenic variants in sarcomere protein genes that evoke hypercontractility, p

248 M) mutation, troponin T (TnT)-R173W, display sarcomere protein misalignment and impaired contractilit

249 odomain interactions, and partially recovers sarcomere protein misalignment as well as impaired contr

250 The giant sarcomere protein titin is important in both heart healt

251 gnificantly increased expression of selected sarcomere proteins (including TNNI3 [troponin I, cardiac

252 heads motif (IHM) structures that with other sarcomere proteins establish an energy-saving, super-rel

253 ssue types that do not express cardiomyocyte sarcomere proteins.

254 caused mainly by mutations in genes encoding sarcomere proteins.

255 ticipate in dynamic conformational states of sarcomere proteins.

256 rcomere- cytoskeleton interactions to induce sarcomere re-organization and contractile recovery in DC

257 rcTrack enhances translational prospects for sarcomere-regulating therapeutics and accelerates interr

258 nt and Micu2(-/-) cardiomyocytes had delayed sarcomere relaxation and cytosolic calcium reuptake kine

259 ntraction size and a nearly 50% reduction in sarcomere relaxation velocity compared to controls.

260 Hz stiffness measurements on a population of sarcomeres selected along an intact fibre isolated from

261 unction and transcriptionally, conductive to sarcomere shortening assays.

262 ticulum Ca(2+) load, together with decreased sarcomere shortening in Slc26a6(-/)(-) cardiomyocytes.

263 Variance of the time from peak sarcomere shortening to 50% re-lengthening (RT(50) ) was

264 ly predicting drug-induced inotropic effect (sarcomere shortening) and generating multi-parameter dat

265 tration-dependent increases and decreases in sarcomere shortening, respectively.

266 enosine triphosphate (ATP) is needed to fuel sarcomere shortening.

267 It led to disorganisation of the sarcomere structure and also modified the protein second

268 of actin regulators because it is vital that sarcomere structure and function are maintained during d

269 orm thin-filament lengths crucial for normal sarcomere structure and function.

270 However, whether Ca(2+) directly regulates sarcomere structure has remained elusive.

271 Accordingly, sarcomere structure is often evaluated by staining for z

272 g) and in vivo tools (Caenorhabditis elegans sarcomere structure).

273 type included muscle weight loss, changes in sarcomere structure, as well as a decrease in force prod

274 ng small-angle X-ray diffraction analysis of sarcomere structure, we demonstrate that the proposed in

275 nd EM examinations revealed primarily normal sarcomere structures in female heterozygous animals, whe

276 in independent of other factors present in a sarcomere, such as filament stiffness and regulatory pro

277 ease the fraction of myosin molecules in the sarcomere that are strongly bound to actin, the molecula

278 L variants display reduced contractility and sarcomeres that are less organized.

279 in experiments using isolated myofibrils and sarcomeres that has allowed scientists to directly evalu

280 loped abnormal actin filament bundles within sarcomeres that interconnected Z lines and were cross-li

281 ted with the distinct elongation of a single sarcomere, the so-called sarcomere "give".

282 driven interactions toward the center of the sarcomere, the structural unit of striated muscle.

283 At the level of the sarcomere, the structural unit of the cardiac myocytes,

284 Striated muscle contraction is the result of sarcomeres, the basic contractile unit, shortening becau

285 in filaments (F-actin) are key components of sarcomeres, the basic contractile units of skeletal musc

286 However, sarcomeres, the fundamental units of myocyte contraction

287 They consist of arrays of sarcomeres, the smallest contractile unit of muscles.

288 "Give" then propagated from sarcomere to sarcomere along the myofibril.

289 a smaller diameter, decreased sensitivity of sarcomeres to Ca(2+), decreased [Ca(2+)] transient ampli

290 that, during contraction, allows weaker half-sarcomeres to equilibrate with in-series stronger half-s

291 ril and depleting the thick filaments in one sarcomere unexpectedly reduced force production.

292 e hypertrophic cardiomyopathy, the titin and sarcomere variants that cause dilated cardiomyopathy and

293 imaging results is a common finding for the sarcomere variants that cause hypertrophic cardiomyopath

294 The force produced by myofibrils with intact sarcomeres was significantly higher than the force produ

295 roduces both parallel and serial addition of sarcomeres, we developed an anisotropic growth constitut

296 The mutants showed shortened sarcomeres with no thick filaments and M-lines in slow f

297 t embryos at day 10.5 exhibited disorganized sarcomeres with wavy thick filaments.

298 microfluidic perfusion system to control one sarcomere within a myofibril, while measuring the indivi

299 e depletion of thick filaments in individual sarcomeres within a myofibril affects force production.

300 of contraction assume a uniform behavior of sarcomeres within myofibrils, the occurrence of sarcomer