ライフサイエンスコーパス: desmin

1 cular endothelial growth factor receptor and desmin).

2 ring the orthologous form of the human R406W-desmin.

3 which did not contain detectable amounts of desmin.

4 , expressing E-cadherin, cytokeratin 18, and desmin.

5 sion levels of podocyte markers, podocin and desmin.

6 of CD31, vascular endothelial cadherin, and desmin.

7 s of nesprin 1, desmin or both nesprin 1 and desmin.

8 rophils, decreased, and fewer HSCs expressed desmin.

9 capacities for nebulin relative to wild-type desmin.

10 ocated within the nebulin-binding regions of desmin.

11 us status in living cells lacking endogenous desmin.

12 acts with the type III intermediate filament desmin.

13 sgenic overexpression of a DRC-linked mutant desmin.

14 ricular myocytes (NRVMs) expressing a mutant desmin.

15 lations and was significantly larger without desmin.

16 eloped to investigate the mechanical role of desmin.

17 d laterally by elastic elements representing desmin.

18 otein, or alphaB-crystallin, a chaperone for desmin.

19 lteration of the pericyte structural protein desmin.

20 myosin heavy chain, smooth muscle actin, and desmin.

21 s in transfected cells expressing the mutant desmin.

22 s, Mip-1a and alphaB-crystallin; the mRNA of desmin, a regeneration-associated molecule, increased.

23 disease caused by dysfunctional mutations in desmin, a type III intermediate filament protein, or alp

24 We focus here on desmin, a type III intermediate filament, which is speci

25 )-mediated DRM is characterized by CryAB and desmin accumulations within cardiac muscle, mitochondria

26 asts induces desmin network disorganization, desmin aggregate formation, and a small decrease in the

27 ss that are characterized by the presence of desmin aggregates and a disorganization of sarcomeres.

28 pic analysis of the explanted heart revealed desmin aggregates and the absence of desmin filaments at

29 min aggregates were located, indicating that desmin aggregates are rigid.

30 d some other membrane proteins, as well, and desmin aggregates in transfected cells expressing the mu

31 at higher Young's moduli were measured where desmin aggregates were located, indicating that desmin a

32 This variant produces desmin aggregates, the main pathological symptom of myof

33 ajor interaction site has been mapped to the desmin alpha-helical rod domain, indicating that the fil

34 taining protein and filamin C, as well as in desmin, alphaB-crystallin and myotilin, result in simila

35 The identified disease proteins (desmin, alphaB-crystallin, myotilin, Zasp, filamin C, an

36 Our data suggest that mutated desmin already markedly impedes myocyte structure and fu

37 onstrate that expression of the E413K mutant desmin also alters the traction forces generation of sin

38 We demonstrate that mutant desmin alters myofibrillar cytoarchitecture, markedly di

39 Desmin amyloids were cytotoxic to myotubes and disrupted

40 oteins involved with cytoskeletal structure (desmin and alpha actinin-2 both decreased), chaperone fu

41 ZASP or filamin C, besides previously known desmin and alphaB-crystallin.

42 type IV IF protein that forms filaments with desmin and also binds alpha-actinin and vinculin.

43 Overall, our data provide a new link between desmin and Ankrd1 regulation, which may be important for

44 y characterized by cytoplasmic aggregates of desmin and associated proteins at the cellular level.

45 245D mutant in myocytes displaces endogenous desmin and C-terminal nebulin from the Z-discs with a co

46 logenesis with disruption of alpha-actinin2, desmin and cardiac ankyrin repeat protein (CARP) was evi

47 ased the expression of podocin but increased desmin and ceramide levels in glomeruli from Asm(+/+) mi

48 le fluorescent proteins genetically fused to desmin and characterized the heterozygous status in livi

49 UF specific desmin and collagen were markedly reduced with EC313 tre

50 ve investigated the impact of overexpressing desmin and expressing a mutant desmin on the passive and

51 ified using dual immunofluorescence (IF) for desmin and GFAP.

52 ression of the intermediate filament protein desmin and interstitial matrix components fibronectin an

53 rim32 down-regulation attenuated the loss of desmin and myofibrillar proteins and reduced atrophy.

54 etal liver mesenchymal cells expressing both desmin and p75 neurotrophin receptor (p75NTR): HSCs in t

55 ical cells, which also express collagen 1a1, desmin and platelet-derived growth factor (alpha polypep

56 abnormalities in staining patterns for both desmin and SPEG.

57 thin the coil IB (nebulin-binding) region of desmin and that has been reported to cause human cardiom

58 ing Trim32 levels prevented the loss of both desmin and thin filament proteins.

59 ulin binding has on the assembly kinetics of desmin and three desminopathy-causing mutant desmin vari

60 re analyzed with antibodies specific to 406W-desmin and to intercalated disc proteins.

61 Previous studies have shown that desmin and vimentin directly participate in signal trans

62 n of the intermediate filament (IF) proteins desmin and vimentin in obstructed bladder smooth muscle

63 Furthermore, desmin and vimentin overexpressions did not alter BSM co

64 persisted whereas cytoskeletal proteins (eg, desmin and vimentin) normalized.

65 erived amyloid coaggregated with full-length desmin and was able to template its conversion into fibr

66 o a reduction in the foot-process fusion and desmin, and a recovery of synaptopodin and podocin.

67 muscle gene promoters (Skeletal alpha-actin, Desmin, and alpha-Myosin heavy chain) in skeletal and ca

68 myosin heavy chain, smooth muscle actin, and desmin, and increased markers of dedifferentiation, cell

69 In addition, desmin, and intercalar disc-related proteins expression

70 AE1/AE3, alpha actin, desmin, and myosin antibodies confirmed the presence of

71 myosin heavy chain, smooth muscle actin, and desmin, and negatively with cellular retinol-binding pro

72 nce of three mesenchymal markers - vimentin, desmin, and PDGFbeta receptor, which indicates their mes

73 ti-collagen IV, rat anti-ICAM-2, rabbit anti-desmin, and rat anti-MECA 32; and intravital injection o

74 er (CD133) and mural cell markers (calponin, desmin, and smooth muscle alpha actin), suggesting dedif

75 ltures developed the expression of runx2 and desmin, and the cocultures that were stimulated with EMD

76 Expression of nephrin, podocin, desmin, and transient receptor potential channel C6 in t

77 enchymal markers including alpha-SMA, FSP-1, desmin, and vimentin.

78 immunocytochemistry for vimentin, alpha-SMA, desmin, and vinculin) generated from rabbit corneal fibr

79 yofibroblast markers, including alphaSMA and desmin, and were not myofibroblast precursors in injured

80 composed almost entirely of rhabdomyoblasts (desmin- and myogenin-positive) was discovered along with

81 conditions in which both healthy and mutant desmin are expressed at equimolar levels mimicking an in

82 In skeletal muscle, nesprin 1 and desmin are thought to link the nucleus to the cytoskelet

83 Although some misfolded proteins, such as desmin, are associated with mutations in the genes encod

84 ecessary for ASMC homeostasis and identifies desmin as a novel regulator of microRNA.

85 erall, our data demonstrate a novel role for desmin as an anti-hypertrophic protein necessary for ASM

86 tly higher expression of VWF, COL1, SM22 and Desmin as compared to cell monolayer.

87 sions, and identified through bioinformatics desmin as their major constituent.

88 concurrent dual functional states of mutant desmin assembly complexes underlie the uncoupling of des

89 Desmin-associated myofibrillar myopathy (MFM) has pathol

90 Detyrosinated MTs associated with desmin at force-generating sarcomeres.

91 We show that the coil IB region of desmin binds to C-terminal nebulin (modules 160-164) wit

92 ar interface and in the parenchyma coexpress desmin but not alpha-SMA, i.e., they are not resident my

93 tenderisation protein markers troponin T and desmin by the four proteases was detected by western blo

94 el of alpha smooth muscle actin (alpha-SMA), desmin, calponin, and smoothelin in SMhAFSCs when compar

95 rupted the fine intracytoplasmic staining of desmin, causing clumping of the desmin protein.

96 dered specific for activated stellate cells (desmin, collagen I-a2, Mmp2, Mmp14) and myofibroblasts (

97 fixed-end stress increased as a function of desmin concentration and this relationship was influence

98 utation (Val459Ile) showed milder effects on desmin cytoplasmic network formation and appears to be a

99 ular and functional relationship between the desmin cytoskeleton and nebulette-containing sarcomeres

100 rapid muscle atrophy induced by fasting, the desmin cytoskeleton and the attached Z-band-bound thin f

101 enhances Trim32-mediated degradation of the desmin cytoskeleton, which appears to facilitate the bre

102 TNF-alpha in a genetic heart failure model, desmin-deficient mice.

103 Western blots indicated that postmortem desmin degradation was more rapid in GG than in DG sampl

104 Desmin (DES) mutations cause severe skeletal and cardiac

105 rcalated discs, with disturbed expression of desmin, desmoplakin, connexin43 and vinculin being evide

106 ism probably corresponds to an alteration in desmin dimer and oligomer assembly and its connection wi

107 laments, while CNM-causing mutations lead to desmin disorganization in a CNM mouse model and patient

108 s, whereas the expression of a mutated E413K desmin does not.

109 Herein, we have created the nesprin-desmin double-knockout (DKO) mouse, eliminating a major

110 We demonstrate that desmin down-regulation by WFA via targeting the conserve

111 Collectively, these data suggest that the desmin E245D mutation interferes with the ability of neb

112 ignificantly delayed dynamics for the mutant desmin E245D relative to wild-type desmin in fluorescenc

113 ikingly perturbed in myocytes expressing the desmin-E245D mutant because most sarcomeres contained el

114 Expression of the desmin-E245D mutant in myocytes displaces endogenous des

115 cells transdifferentiated into NG2/PDGFRbeta/desmin-expressing cerebrovascular pericytes, enwrapping

116 t-related transcription factor 2 (runx2) and desmin expression by real-time polymerase chain reaction

117 thmatic patients show a negative correlation desmin expression in airway smooth muscle cell (ASMC) an

118 alization pattern for nebulette and impaired desmin expression were noted in the proband and chimeric

119 SMCs, which stably suppresses 90% endogenous desmin expression.

120 runx2 and desmin expressions were increased in stimulated cocultur

121 ula occludens-1, mesenchymal markers such as desmin, fibroblast-specific protein-1, and matrix metall

122 scle cells, vascular smooth muscle cells and desmin(+) fibroblasts by lineage tagging.

123 duced alpha-smooth muscle actin (alpha-SMA), desmin, fibronectin, and collagen I expression, suggesti

124 of this disease, it is essential to analyze desmin filament structures under conditions in which bot

125 desmin polymerization induced disassembly of desmin filaments and destruction of thin filament compon

126 evealed desmin aggregates and the absence of desmin filaments at intercalated discs.

127 process involving the initial disassembly of desmin filaments by Trim32, which leads to the later myo

128 er denervation, inducing the dissociation of desmin filaments caused an accumulation of ubiquitinated

129 ssembly complexes underlie the uncoupling of desmin filaments from intercalated discs and their struc

130 ation and Trim32-dependent ubiquitination of desmin filaments increased rapidly and stimulated their

131 lette extends outward from Z-disk-associated desmin filaments toward the center of the sarcomere.

132 Although myofibrils and desmin filaments were intact at 7 d after denervation, i

133 Desmin filaments were proposed to maintain the integrity

134 enervation required not only dissociation of desmin filaments, but also gene induction by PAX4.

135 accompanied by increased phosphorylation of desmin filaments, which promoted desmin ubiquitylation b

136 s influenced by the cellular location of the desmin filaments.

137 y in vitro interaction between nebulette and desmin filaments.

138 In vitro, R406W-desmin formed unusually thick filaments that organized i

139 These data suggest that desmin forms seeding-competent amyloid that is toxic to

140 Moreover, a purified desmin fragment (aa 117 to 348) containing both amyloido

141 Finally, desmin fragment amyloid persisted when introduced into m

142 Desmin fragment-derived amyloid coaggregated with full-l

143 Mutations in the human desmin gene cause myopathies and cardiomyopathies.

144 ytes is evident since mutations in the human desmin gene cause severe myopathies and cardiomyopathies

145 In cultured human podocytes, CB1R and desmin gene expression were increased and podocin and ne

146 nterfering (si)RNA-mediated knockdown of the desmin gene in HASMCs, recombinant HASMCs (reHASMCs), up

147 This mutation in the desmin gene is one of those leading to desminopathies, a

148 plice donor site mutation (IVS3+3A>G) of the desmin gene located on chromosome 2q35.

149 f equimolar mixtures of mutant and wild-type desmin generated chimeric filaments of seemingly normal

150 othelial cells, stimulating proliferation of desmin(+) hepatic stellate-like cells and enforcing a pr

151 ate that by interacting with desmoplakin and desmin, iASPP is an important regulator of desmosomal fu

152 Our study demonstrates that linkage to desmin IF networks via plectin is crucial for formation

153 ly for diseases, the related muscle-specific desmin IF networks.

154 hat beta-synemin mediates the association of desmin IFs with Z disks, whereas alpha-synemin stabilize

155 We show that the expression of this mutant desmin in C2C12 myoblasts induces desmin network disorga

156 iASPP interacts with desmoplakin and desmin in cardiomyocytes to maintain the integrity of de

157 This dissociates a direct role for desmin in corneal cell proliferation.

158 he mutant desmin E245D relative to wild-type desmin in fluorescence recovery after photobleaching in

159 Moreover, loss of desmin in HASMCs increases the phosphorylation of Akt, i

160 , significantly attenuated the expression of desmin in pericytes.

161 nt accumulation of the intermediate filament desmin in the myofibers of the patients.

162 muscle had myofibrillar disorganization and desmin inclusions.

163 st show that the overexpression of wild-type-desmin increases the overall rigidity of the cells, wher

164 pression of nephrin, synaptopodin, WT-1, and desmin, indicating that PI3KC2alpha deficiency specifica

165 or NF-kappaB inhibitor inhibits the loss of desmin-induced Ankrd1 up-regulation, suggesting Akt/NF-k

166 Desmin interacts with nebulin establishing a direct link

167 microdomains that anchor a three-dimensional desmin intermediate filament (IF) web.

168 ion in cardiomyocytes of the extrasarcomeric desmin intermediate filament system is frequently observ

169 Desmin intermediate filaments (DIFs) form an intricate m

170 ells to reach high indentation depths, where desmin intermediate filaments are typically located.

171 Furthermore, while microtubules and desmin intermediate filaments associate closely with car

172 Our findings reveal a novel role for desmin intermediate filaments in modulating actin filame

173 In striated muscle, desmin intermediate filaments interlink the contractile

174 d that a balance of dynamic microtubules and desmin intermediate filaments is required to maintain nu

175 ssing a mutant form of the gene encoding for desmin intermediate filaments, p.D399Y.

176 istic alterations, including accumulation of desmin intermediate filaments.

177 Desmin is an abundant muscle-specific intermediate filam

178 Desmin is an intermediate filament protein in skeletal m

179 Our earlier study showed that desmin is an intracellular load-bearing protein that inf

180 We previously showed that desmin is an intracellular load-bearing protein, which i

181 Thus, during atrophy, the initial loss of desmin is critical for the subsequent myofibril destruct

182 The intermediate filament protein desmin is encoded by the gene DES and contributes to the

183 ylation of the intermediate filament protein desmin is significantly increased (5.7-fold, p < 0.005)

184 ar to neurodegeneration-associated proteins, desmin itself may form amyloid.

185 Heterozygous and homozygous R405W-desmin knock-in mice develop both a myopathy and a cardi

186 sue, we further generated and analyzed R405W-desmin knock-in mice harboring the orthologous form of t

187 t are recapitulated in the hearts from R405W-desmin knock-in mice of both genotypes.

188 ated from young hetero- and homozygous R349P desmin knock-in mice, which carry the orthologue of the

189 ly subjected immortalized heterozygous R349P desmin knock-in myoblasts to magnetic tweezer experiment

190 ese cables (but not their size) increases in desmin knockout muscle (a fibrosis model).

191 on shared poor growth in culture and lack of desmin labelling, we believe that PTP dysregulation may

192 1B (lamin A/C), LGMD1C (caveolin-3), LGMD1D (desmin), LGMD1E (DNAJB6), and more recently for LGMD1F (

193 coding for the intermediate filament protein desmin may cause skeletal and cardiac myopathies, which

194 These results suggest that desmin may play an important role in ASMC homeostasis.

195 These results suggest that Ankrd1 and desmin may play important roles on ASMC homeostasis.

196 compared with wild-type, nesprin 1(-/-) and desmin(-/-) mice.

197 ry the orthologue of the most frequent human desmin missense mutation R350P.

198 d their myofibril organization compared with desmin monomer or other nondesmin amyloids.

199 nravel short distance structural patterns of desmin mutants in filaments.

200 e demonstrated filament formation defects of desmin mutants, associated with arrhythmogenic right ven

201 a, we suggest that for some filament-forming desmin mutants, the molecular etiology of desminopathy r

202 Here we show that desmin-mutated cells display a 39% increased median elas

203 Desmin-mutated cells required higher forces than wild-ty

204 N5A polymorphisms were identified in 2 and a desmin mutation in 1.

205 chanisms initiated by the heterozygous R406W-desmin mutation in the development of a severe and early

206 Here, we examined a desmin mutation, E245D, that is located within the coil

207 ardiomyopathy caused by a heterozygous R406W-desmin mutation.

208 the structural effects of five heterozygous desmin mutations on filament formation in vitro and in l

209 of expression of certain disease-associated desmin mutations.

210 yte nuclear factor 4alpha(+) hepatocytes and desmin(+) myofibroblasts surrounding reactive ducts in D

211 ns showed the tumor cells to be positive for desmin, myogenin, and myogenic differentiation 1 (MyoD1)

212 Mutations in alphaB-crystallin, desmin, myotilin, Zasp, or filamin-C can cause MFMs and

213 ctin positive, weakly calponin positive, and desmin negative.

214 his mutant desmin in C2C12 myoblasts induces desmin network disorganization, desmin aggregate formati

215 Our data suggest that a well-organized desmin network is required to accommodate an optimal con

216 The desmin network's pivotal role in myocytes is evident sin

217 of mature pericytes and immunoreactivity for desmin, NG-2, platelet-derived growth factor receptor be

218 ere misalignment and stress in wild-type and desmin null fibers.

219 Here, we report that ASMCs of desmin null mice (ASMCs(Des-/-)) show hypertrophy and up

220 During fixed-end contraction, desmin null simulations also demonstrated greater sarcom

221 arly with fiber strain in both wild-type and desmin null simulations and was significantly larger wit

222 verexpressing desmin and expressing a mutant desmin on the passive and active mechanical properties o

223 ed in the periportal region do not coexpress desmin or alpha smooth muscle actin (alpha-SMA).

224 global ablation murine models of nesprin 1, desmin or both nesprin 1 and desmin.

225 s by transgenic overexpression of the mutant desmin or CryAB(R120G) both in intact mice and in vitro.

226 Murine BSM strips overexpressing desmin or vimentin generated less force in response to K

227 ysfunction associated with overexpression of desmin or vimentin is mediated via c-Jun N-terminal kina

228 BSM tissue in which increased expression of desmin or vimentin was induced by adenoviral transductio

229 Upon depletion of desmin (or nesprin [nuclear envelope spectrin repeat pro

230 on of EMT markers alpha-smooth muscle actin, desmin, or FSP-1.

231 SPEG localizes in a double line, flanking desmin over the Z lines, and is apparently in alignment

232 orneal opacity, we identify a novel role for desmin overexpression in corneal haze.

233 the ability of a comparable level of mutant desmin overexpression to accumulate ubiquitinated protei

234 Presumably, the loss of the desmin-p.

235 The in vitro experiments of desmin-p.A120D reveal a severe intrinsic filament format

236 The functional experiments of desmin-p.H326R did not demonstrate any differences from

237 CD34, Ano1, NTPDase2, connexin 43, vimentin, desmin, PDGFbeta receptor and merlin/NF2.

238 Desmin peptides corresponding to putative amyloidogenic

239 and CD31(+) endothelial cells or LacZ(+) and desmin(+) pericytes confirmed that chimeric mice transpl

240 zed with either CD31(+) endothelial cells or desmin(+) pericytes.

241 d with disrupted co-localization of ECs with desmin(+) perivascular cells, and reduction of blood flo

242 Thus, during fasting, desmin phosphorylation increases and enhances Trim32-med

243 rthermore, overexpression of an inhibitor of desmin polymerization induced disassembly of desmin fila

244 sed alpha smooth muscle actin (alphaSMA) and desmin-positive cells and collagen deposition, independe

245 n glomerular development as stromal-derived, desmin-positive cells fail to coalesce near forming neph

246 there was a significant decrease in GFAP and desmin-positive cells, compared to WT mice ( approximate

247 alized nuclei, myofibrillar disorganization, desmin-positive inclusions, and thickened Z-bands.

248 ution, mGFP expression was observed again in desmin-positive peri-sinusoidal HSCs; no mGFP expression

249 EBS-MD myofibers, including the presence of desmin-positive protein aggregates and a concurrent disa

250 ctin in skeletal muscle display pathological desmin-positive protein aggregation and misalignment of

251 phic rhabdomyosarcomas (MyoD-, Myogenin- and Desmin-positive), whereas introduction of the same oncog

252 imulations with only a fraction of wild-type desmin present, fixed-end stress increased as a function

253 staining of desmin, causing clumping of the desmin protein.

254 We demonstrate that R406W-desmin provokes its severe cardiotoxic potential by a no

255 a drug or by expression of dominant-negative desmin reduces type I collagen expression, primarily due

256 with high affinity, whereas binding of this desmin region containing the E245D mutation appears to e

257 einaceous deposits are characteristic of the desmin-related cardiomyopathies and crystallin cardiomyo

258 erapeutic efficacy of doxycycline (Doxy) for desmin-related cardiomyopathy (DRC) and to elucidate the

259 expression or voluntary exercise ameliorated desmin-related cardiomyopathy (DRC).

260 allin (CryAB) gene triggers a severe form of desmin-related cardiomyopathy (DRCM) characterized by ac

261 lation of the mutant protein associated with desmin-related cardiomyopathy in cultured cardiomyocytes

262 ryAB (alphaB-crystallin) causative for human desmin-related cardiomyopathy were used for a total geno

263 rdiomyocytes in transgenic (TG) mice, causes desmin-related cardiomyopathy, a protein conformational

264 allin R120G mutation (CryAB(R120G)) model of desmin-related cardiomyopathy, accumulate cytotoxic misf

265 as been observed in proteinopathies, such as desmin-related cardiomyopathy, and implicated in many co

266 nction were tested in a proteotoxic model of desmin-related cardiomyopathy, caused by cardiomyocyte-s

267 ice representing a well-established model of desmin-related cardiomyopathy, we demonstrated that CR-P

268 n aggregates in mice with CryAB(R120G)-based desmin-related cardiomyopathy.

269 o the pathophysiological changes observed in desmin-related myopathies.

270 iniscent of a major histological hallmark of desmin-related myopathies.

271 G), which carries a mutation associated with desmin-related myopathy (DRM), results in an altered sar

272 n in alphaB-crystallin (CryAB(R120G)) causes desmin-related myopathy (DRM).

273 diomyopathy missense mutation, R157H, or the desmin-related myopathy mutation, R120G.

274 ose a mechanism by which mutant desmin slows desmin remodeling in myocytes by retaining nebulin near

275 rowth pattern and cellular adhesion, reduced desmin RNA expression, and some other membrane proteins,

276 tations in the head, rod, or tail domains of desmin (S46F, E245D, and T453I).

277 es in Z-bands of human cardiac myocytes with desmin, SERCA2, PLB, and AKAP18.

278 all, our data demonstrate that nesprin 1 and desmin serve redundant roles in nuclear anchorage and th

279 patic fibrosis was evaluated by staining for desmin, Sirius Red/Fast Green, and vimentin.

280 We propose a mechanism by which mutant desmin slows desmin remodeling in myocytes by retaining

281 This model suggests that desmin stabilizes Z-disks and enables greater stress pro

282 ventricular tissue slices revealed a loss of desmin staining within the intercalated disk and severe

283 Levels of the pericyte structural protein, desmin, substantially increased after noise exposure in

284 (IFs) in cardiomyocytes consist primarily of desmin, surround myofibrils at Z disks, and transmit for

285 ched actin components causes accumulation of desmin tangles in the cytoplasm.

286 hat, although nebulin M160-164 bound to both desmin tetrameric complexes and mature filaments, all th

287 myocytes by 70% and resulted in a failure of desmin to align with Z disks and disrupted cell-cell jun

288 mechanisms that regulate the association of desmin to sarcomeres and their role in desminopathy are

289 Conclusions: Disrupting the tethering of desmin to the nucleus results in a loss of nuclear homeo

290 rcomeric (myosin) and cytoskeletal proteins (desmin, tubulin) also underwent 4HNE adduction.

291 rylation of desmin filaments, which promoted desmin ubiquitylation by Trim32 and degradation.

292 desmin and three desminopathy-causing mutant desmin variants carrying mutations in the head, rod, or

293 The disease-mutant desmin variants E245D and T453I exhibited increased bind

294 latelet-derived growth factor receptor beta, desmin, vimentin, tissue inhibitor of metalloproteinase-

295 406W mutation on the molecular properties of desmin were addressed by cell transfection and in vitro

296 Increased expression levels of desmin were associated with the induction of hypoxia ind

297 immunoprecipitation, while alpha-synemin and desmin were insoluble.

298 that expressed alpha-smooth muscle actin or desmin were lost, along with expression of cyclooxygenas

299 Pericytes, cells that express both CD31 and desmin, were found both in the walls of tumor-associated

300 issues express alpha-smooth muscle actin and desmin, which are markers for uterine differentiation.

301 n studies disclosed a dual behavior of R406W-desmin with both its integration into the endogenous int