ライフサイエンスコーパス: sarcomere

1 decay and continue to be incorporated in the sarcomere).

2 ed desmin filaments toward the center of the sarcomere.

3 ing filaments from the opposite sides of the sarcomere.

4 he interaction of cMyBP-C with the TF in the sarcomere.

5 ith cell-scale order that resembles a muscle sarcomere.

6 ng mystery regarding the architecture of the sarcomere.

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

8 onsible for the passive force of the cardiac sarcomere.

9 h the actin filaments of the skeletal muscle sarcomere.

10 not properly integrate in the C-zone of the sarcomere.

11 nesis prior to regulating contraction in the sarcomere.

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

13 nteraction of beta-arrestin localized to the sarcomere.

14 meres that links integrins ultimately to the sarcomere.

15 nd earlier, more rapid shortening of central sarcomeres.

16 ration of single myoblasts lacking organized sarcomeres.

17 f desmin aggregates and a disorganization of sarcomeres.

18 idge power stroke and myofilament sliding in sarcomeres.

19 to the nuclei before its incorporation into sarcomeres.

20 itute a fundamental core component of muscle sarcomeres.

21 contacts with tendon cells and then assemble sarcomeres.

22 nsity of mitochondria tightly packed between sarcomeres.

23 ds of the actin filaments from the adjoining sarcomeres.

24 ss I HDAC, HDAC3, is also present at cardiac sarcomeres.

25 display similar but distinct localization in sarcomeres.

26 rganized actomyosin contractile units called sarcomeres.

27 py and by measuring force development of the sarcomeres.

28 micrometer-scale contractile machines called sarcomeres.

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

30 ile measuring the individual behavior of all sarcomeres.

31 of its longitudinal tubules across adjacent sarcomeres.

32 f contractile proteins into highly organized sarcomeres.

33 where it contributes to the organization of sarcomeres.

34 s associated with desmin at force-generating sarcomeres.

35 of labeled sarcomeres and the translation of sarcomere activity to mechanical output (contractility)

36 complex safety mechanims for protecting the sarcomere against structural disintegration under excess

37 Grafted cardiomyocytes showed enhanced sarcomere alignment and increased connexin 43 expression

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

39 actile mechanisms that involve shortening of sarcomeres along myofibrils.

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

41 fluenced by the structural maturation of the sarcomere and changes in contractile filament protein is

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

43 anogaster, acts at two distinct sites in the sarcomere and controls thin filament length with just tw

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

45 x2, Tjp1, cell survival, Capn3, Sirt2, Csda, sarcomere and cytoskeleton organization and function, Tr

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

47 th each other, were also required for normal sarcomere and/or adhesome structure.

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

49 tyrosination was reduced, MTs uncoupled from sarcomeres and buckled less during contraction, which al

50 found that HDAC3 was localized to A-band of sarcomeres and capable of deacetylating myosin heavy cha

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

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

53 heart, allowing visualization of individual sarcomeres and measurement of the single cardiomyocyte c

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

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

56 in-associated factor, associate with cardiac sarcomeres and that a class I and II HDAC inhibitor, tri

57 ss on the shortening and movement of labeled sarcomeres and the translation of sarcomere activity to

58 ilaments of opposite polarity from adjoining sarcomeres and transmits tension along myofibrils during

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

60 BTHS iPSC-CMs assembled sparse and irregular sarcomeres, and engineered BTHS 'heart-on-chip' tissues

61 Furthermore, the first sarcomeres appear in positions close to the nuclei, rega

62 Sarcomeres are connected in series through a network of

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

64 bifurcated SHG intensity, are illusory when sarcomeres are staggered with respect to one another.

65 t to know its structural organization in the sarcomere, as this will affect its ability to interact w

66 expression, but not DNMT1 or -3b, disrupted sarcomere assembly and decreased beating frequency, cont

67 vide insight into the molecular basis of the sarcomere assembly and muscle dysfunction associated wit

68 Sarcomere assembly and myocardial contraction abnormalit

69 , also known as Drosophila titin, to observe sarcomere assembly during IFM development.

70 uniaxial contractility and alignment, robust sarcomere assembly, and reduced variability and hypersen

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

72 cta at the cytoplasmic face of nuclei before sarcomere assembly.

73 FP to the nucleus during the early stages of sarcomere assembly.

74 s essential to the mechanochemical cycle and sarcomere assembly.

75 IFM) can be used as a model for the study of sarcomere assembly.

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

77 (encoded by the FLNC gene) is essential for sarcomere attachment to the plasmatic membrane.

78 These changes reposition the remaining sarcomeres back into their optimal operating regime.

79 omyocytes contain myofibrils that harbor the sarcomere-based contractile machinery of the myocardium.

80 In the cardiac sarcomere, both cardiac myosin binding protein C (cMyBP-

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

82 terior domain dissembles the remnants of its sarcomere, but still retains a vestigial attachment to t

83 iation and numerous components of the muscle sarcomere, but the potential involvement of MRTFs in ske

84 cium-dependent tension generation within the sarcomeres, but how this translates into the spectrum of

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

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

87 for functional relationships among the many sarcomere components.

88 Muscle sarcomeres contain giant polypeptides composed of multip

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

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

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

92 for HCM pathobiology and that inhibitors of sarcomere contraction may be a valuable therapeutic appr

93 as Ca(2+) sparks and waves, can cause local sarcomere contraction.

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

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

96 ent with severe chronic hypoxia/ischemia and sarcomere diastolic-length was shortened.

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

98 s via the microendoscope and visualizing the sarcomere displacements, we monitored single motor unit

99 double knockout (dKO) mice were able to form sarcomeres during embryogenesis.

100 Imaging of sarcomere dynamics in vivo in patients has significant c

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

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

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

104 hy (LVH) develops as an adaptive response to sarcomere dysfunction.

105 arcomere proteins are consistent with mutant sarcomeres exhibiting enhanced contractile power, others

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

107 findings reveal a central role for MRTFs in sarcomere formation during skeletal muscle development a

108 We show that nuclear positioning precedes sarcomere formation.

109 es, which serves as template for contractile sarcomere formation.

110 for ubiquitination, have been implicated in sarcomere formation.

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

112 velocities, apparently caused by defects in sarcomere function, because Ca(2+) transients were unaff

113 from the optogenetic assay as descriptors of sarcomere functions.

114 determine their strength of association with sarcomere gene mutation carriage.

115 om 46 genotyped HCM patients with or without sarcomere gene mutations and 10 control hearts.

116 eature in HCM, and an early manifestation of sarcomere-gene mutations in subexpressed family members

117 Mutations in sarcomere genes may distinctly alter calcium handling pa

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

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

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

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

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

123 We then use Sls-GFP in the IFM to show that sarcomeres grow individually and uniformly throughout th

124 monstrate that the presence of hC0C1f in the sarcomere had the greatest effect at short, but not long

125 e springs mimic the combined effects of half-sarcomere heterogeneity and muscle's series elastic comp

126 reducing T from approximately 3 nm per half-sarcomere (hs) and 1,000 s(-1) at high load to approxima

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

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

129 We determined that KLHL40 localizes to the sarcomere I band and A band and binds to nebulin (NEB),

130 tethers actin filaments to the Z-line of the sarcomere in muscles.

131 is a giant filamentous protein of the muscle sarcomere in which stretch induces the unfolding of its

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

133 Seven formins immunolocalize to sarcomeres in diverse patterns, suggesting that they hav

134 compensated for by increasing the number of sarcomeres in series.

135 s new functional length by a chronic loss of sarcomeres in series.

136 eated and regularly spaced dense body of the sarcomeres in the muscle.

137 II appear in a polarized structure called a sarcomere, in which myosin II is localized in the center

138 n iPS cell-derived cardiomyocytes results in sarcomere insufficiency, impaired responses to mechanica

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

140 the mechanical changes that occur within the sarcomere, intercalated disc, costamere, and extracellul

141 The power in the myocardium sarcomere is generated by two bipolar arrays of the moto

142 monstrate that the presence of hC0C1f in the sarcomere is sufficient to induce depressed myofilament

143 The sarcomere is the smallest functional unit of myofibrils

144 desmin cytoskeleton and nebulette-containing sarcomeres is still unclear.

145 for anchoring filaments in the Z-disk of the sarcomere, is composed of immunoglobulin domains.

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

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

148 Increases in sarcomere length (1.9-2.2 mum) and external [Ca(2+)]o (1

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

150 ulation of myofilament Ca(2+) sensitivity by sarcomere length (SL) [length-dependent activation (LDA)

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

152 studied at short (2 mum) and long (2.3 mum) sarcomere length (SL).

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

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

155 und involuntary microscopic contractions and sarcomere length abnormalities.

156 However, it is unclear how changes in sarcomere length and lattice spacing affect cross-bridge

157 llowed muscle fibers to operate at a shorter sarcomere length and maintain optimal thin-thick filamen

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

159 Previous studies show that increases in sarcomere length can reduce thick-to-thin filament spaci

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

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

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

163 approximately 20% slower at 2.5 vs. 2.0 mum sarcomere length due to a slower MgADP release rate (10.

164 eater Ca(2+) sensitivity for 2.5 vs. 2.0 mum sarcomere length fibers (pCa50 = 5.68 +/- 0.01 vs. 5.60

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

166 ktr is dependent on sarcomere length in the physiological range 1.85-1.94 mu

167 tachment rate slowed by approximately 15% as sarcomere length increased, due to a slower MgADP releas

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

169 ionally, orthovanadate blunted the effect of sarcomere length on N-cTnC conformational behavior such

170 To study the structural effects of sarcomere length on N-cTnC, we monitored N-cTnC opening

171 cium sensitivity were mimicked by increasing sarcomere length or by deleting the N terminus of the cR

172 lattice spacing, achieved through increased sarcomere length or osmotic compression of the fiber via

173 mpression of fibers at either 2.5 or 2.0 mum sarcomere length produced only slight (and statistically

174 binding protein C (MyBP-C) decreased in the sarcomere length range 2.6-3.0 mum but were constant out

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

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

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

178 e was associated with downward shifted force-sarcomere length relations, indicative of shorter thin f

179 Drosophila melanogaster sarcomere length short (SALS) is a recently identified W

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

181 mily members in hESC-CMs enhances cell size, sarcomere length, force of contraction, and respiratory

182 sed on N-cTnC opening suggested that at long sarcomere length, strongly bound cross-bridges indirectl

183 tr by approximately 50%, irrespective of the sarcomere length, whereas decreasing phosphorylation by

184 We investigated the sarcomere length-dependence of force, a functional assay

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

186 shape morphology and significantly increased sarcomere length.

187 on N-cTnC opening at any tested [Ca(2+)] or sarcomere length.

188 determine whether N-cTnC opening depends on sarcomere length.

189 opomyosin) become diminished by decreases in sarcomere length.

190 rating levels of Ca(2+) and 1.80 and 2.2-mum sarcomere length.

191 e data suggest that skeletal muscle exhibits sarcomere-length-dependent changes in cross-bridge kinet

192 zed trabeculae over a physiological range of sarcomere lengths (1.85-1.94 mum).

193 Ca(2+)] relationships were determined at two sarcomere lengths (SL = 1.9 mum and SL = 2.3 mum).

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

195 oscope for minimally invasive observation of sarcomere lengths and contractile dynamics in any major

196 gth-perturbation analysis at 2.5 and 2.0 mum sarcomere lengths as pCa and [MgATP] varied.

197 tivity of the force-pCa relationship at both sarcomere lengths was greater with osmotic compression v

198 hereby increasing force production at longer sarcomere lengths.

199 ociated and not coupled to shorter diastolic sarcomere lengths.

200 Sarcomere-level mechanics recorded by a striation follow

201 Sarcomere loss varies significantly across the muscle wi

202 in HCM pathogenesis and suggest that mutant sarcomeres manifest irreversible cardiomyocyte defects t

203 Inability to perform in vivo sarcomere measurements with submicrometer resolution is

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

205 larva, the muscle of both sexes has similar sarcomere morphology, but the hermaphrodite sex-determin

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

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

208 could detect tissue-level alterations in HCM sarcomere mutation carriers with and without left ventri

209 e studied 3 groups of genotyped individuals: sarcomere mutation carriers with left ventricular hypert

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

211 3mut) mutations, and IDCM were compared with sarcomere mutation-negative HCM (HCMsmn) and nonfailing

212 CM patients with the R403Q mutation and nine sarcomere mutation-negative HCM patients (HCMsmn).

213 ic reticulum Ca uptake are depressed in both sarcomere mutation-positive and -negative HCM.

214 Enhanced sarcomere stiffness in sarcomere mutation-positive HCM samples was irrespective

215 ivation of the CaMKII pathway is specific to sarcomere mutation-positive HCM, whereas sarcoplasmic en

216 MKII protein abundance was increased only in sarcomere-mutation HCM (P<0.001).

217 phospholamban was 5.5-fold increased only in sarcomere-mutation HCM (P=0.01), as was autophosphorylat

218 Sarcomere mutations and left ventricular (LV) hypertroph

219 gical samples with (n=25) and without (n=10) sarcomere mutations compared with control hearts (n=8).

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

221 hod1, Spag9 and structural components of the sarcomere, Myom1, Tnnt2, Zasp.

222 In sarcomeres, myosin II-mediated sliding of antiparallel F

223 dedifferentiation, including a disorganized sarcomere network, rounding, and conspicuous cell-cycle

224 for the proper assembly and stability of the sarcomere network.

225 Here, we develop a capillary analog of the sarcomere obeying Hill's equation and discuss its analog

226 The sarcomere of muscle is composed of tens of thousands of

227 thin (actin-containing) filaments in intact sarcomeres of heart muscle.

228 arly, in a manner that is reminiscent of the sarcomeres of muscle tissue.

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

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

231 C is a thick filament protein that regulates sarcomere organization and rigidity.

232 Although sarcomere organization is not sustained in older flies a

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

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

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

236 Nebulin sets actin thin filament length in sarcomeres, potentially by stabilizing thin filaments in

237 the thin filament towards the centre of the sarcomere, producing, under unloaded conditions, a filam

238 rdiomyopathy (HCM) is caused by mutations in sarcomere protein genes, and left ventricular hypertroph

239 uely defined by Fourier decomposition of the sarcomere protein spatial framework.

240 Human mutations that truncate the massive sarcomere protein titin [TTN-truncating variants (TTNtvs

241 se data suggest that the interaction between sarcomere proteins and nuclei is not dependent on proper

242 of power generation by mutant and wild-type sarcomere proteins are consistent with mutant sarcomeres

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

244 rt muscle that can be caused by mutations in sarcomere proteins.

245 ciated mutations are found in genes encoding sarcomere proteins.

246 caused mainly by mutations in genes encoding sarcomere proteins.

247 domain first disassembles the dorsal-ventral sarcomere region and develops filopodia that elongates a

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

249 sensitive to the structural state of muscle sarcomeres, SHG functional imaging can give insight into

250 tion-incompetent RyR2 displayed depressed AM sarcomere shortening and reduced in vivo atrial contract

251 f R9C-PLB mutation on calcium transients and sarcomere shortening in adult cardiomyocytes.

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

253 the cardiac cycle and prematurely truncates sarcomere shortening in the facilitation of rapid relaxa

254 on frequency accelerate Ca(2+)-transient and sarcomere shortening kinetics in R21C myocytes from olde

255 Translation of sarcomere shortening to mechanical output was highest in

256 functional measurements (calcium transient, sarcomere shortening) from isolated myocytes (n=42-104 m

257 n association with enhanced calcium cycling, sarcomere shortening, and beta-adrenergic responsiveness

258 significantly reduced fractional shortening, sarcomere shortening, and relaxation velocities, apparen

259 In sarcomeres, sHSP binding to titin was actin filament ind

260 it contractile dynamics and the structure of sarcomeres, skeletal muscle's contractile units.

261 icantly lower cell viability, destruction of sarcomeres, smaller action potentials, and lower conduct

262 Enhanced sarcomere stiffness in sarcomere mutation-positive HCM s

263 increased myofilament force development and sarcomere stiffness.

264 Ca(2+) signals and sarcomere strain correlated in space and time with short

265 Ca(2+) activity and sarcomere strain were also imaged in paced cardiac myocy

266 that increased titin Ig unfolding, including sarcomere stretch and the expression of stiff titin isof

267 movement of myosin toward actin but, rather, sarcomere stretch-induced simultaneous structural rearra

268 In this believed novel approach, we examine sarcomere structure by measuring the multiple resonant r

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

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

271 sed, and the sliding-filament theory for the sarcomere suggested how its different parameters can be

272 diffusely localized around the Z-line of the sarcomere, suggesting a Ca(2+)-dependent mechanism of en

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

274 cs consists of actin filaments from adjacent sarcomeres that are cross-linked by alpha-actinin homodi

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

276 and muscle's series elastic component.) Half-sarcomeres that shortened by > approximately 10 nm when

277 to simulate the mechanical behavior of half-sarcomeres that were connected in series with springs of

278 category of proteins, including those of the sarcomere, the cytoskeleton, and desmosomes.

279 The sarcomere, the fundamental unit of muscle contraction, i

280 nsduction and mechanotransmission within the sarcomere, the intercalated disc, and at the sarcolemma.

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

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

283 Finally, we show that in contracting sarcomeres, the activating effect of C1 is apparent only

284 Sarcomeres, the functional units of contraction in stria

285 and reforming myofibrillar components of the sarcomere throughout cell cycle progression.

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

287 date an optimal conformation of nebulette on sarcomeres to bind and recruit cardiac alpha-actin.

288 ckled less during contraction, which allowed sarcomeres to shorten and stretch with less resistance.

289 Sarcomere transcript and protein levels were analyzed in

290 r physiology, no extant technology can image sarcomere twitch dynamics in live humans.

291 However, the sarcomeres were abnormally small and disorganized, causi

292 By contrast, sarcomeres were assembled in the hypomorphic ttn mutants

293 When half-sarcomeres were linked to stiff springs (so that they di

294 As a result, sarcomeres were poorly formed and the general myofibril

295 Unlike actomyosin in sarcomeres, which cycles through contraction and relaxat

296 mounts of Sls in the IFM using RNAi leads to sarcomeres with smaller Z-discs in their core, whilst th

297 pendent activation properties of the cardiac sarcomere, with relative contributions of ~67 and ~33% f

298 tile actin meshwork is organized like muscle sarcomeres, with repeating myosin II filaments separated

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

300 positioned nuclei and the linearly arranged sarcomeres, yet the relationship between these two featu