ライフサイエンスコーパス: emerin

1 lphaII-spectrin and NMI interact stably with emerin.

2 ses relocalization of the LEM domain protein emerin.

3 fespan than those only lacking lamin A/C and emerin.

4 -relevant and inhibited by direct binding to emerin.

5 in), and is feedback-regulated by binding to emerin.

6 lamina-associated polypeptide 1 rather than emerin.

7 f viral cDNA with chromatin was dependent on emerin.

8 the nuclear envelope proteins lamin A/C and emerin.

9 of MAN1 for binding to selected partners of emerin.

10 ys showed that MAN1-N also bound directly to emerin.

11 rates in the mice lacking both lamin A/C and emerin.

12 lamin B1, supporting functional overlap with emerin.

13 s that Ce-MAN1 overlaps functionally with Ce-emerin.

14 stitution or disease-associated mutations in emerin.

15 those required for direct binding to DNA or emerin.

16 uclear membrane proteins, including LAP2 and emerin.

17 proteins lamina-associated polypeptide 2 and emerin.

18 an essential enzyme, controls two regions in emerin.

19 ssociated polypeptide1 (LAP1) interacts with emerin.

20 this pathology is worsened in the absence of emerin.

21 ble flow was nondirectional in cells lacking emerin.

22 expressing high levels of dominant-negative emerin.

23 sociation with the native LEM-domain protein emerin, a conserved component of nuclear "lamina" struct

24 X-linked EDMD results from mutations in emerin, a lamin A-associated protein.

25 Many regions of human MAN1 are homologous to emerin, a LEM domain nuclear protein, loss of which caus

26 Emerin, a membrane component of nuclear "lamina" network

27 chor TAN lines, prompting us to test whether emerin, a nuclear membrane protein that interacts with l

28 y (X-EDMD) is inherited through mutations in emerin, a nuclear membrane protein.

29 We separated two populations of emerin, A-type lamins and BAF; one population solubilize

30 Life span was unaffected by loss of Ce-emerin alone but was significantly reduced in LEM-2-null

31 plicative myoblasts lacking A-type lamins or emerin also have decreased levels of proteins important

32 These include the mislocalization of emerin, an inner nuclear membrane protein, defects in wh

33 Here we show that emerin, an integral inner-nuclear-envelope protein, is n

34 r dystrophy is caused by loss of function of emerin, an integral protein of the inner nuclear membran

35 Emerin and A-type lamins are proteins of the inner membr

36 Emerin and BAF associated only in histone- and lamin-B-c

37 n to the cytoplasm, reduced interaction with emerin and beta-actin, and activation of glycogen syntha

38 s at the nuclear membrane and interacts with emerin and beta-actin.

39 n properties and immunolocalization, both Ce-emerin and Ce-MAN1 are integral membrane proteins locali

40 Ce-emerin and Ce-MAN1 migrate on SDS-PAGE as 17- and 52-kDa

41 are essential for cell division and that Ce-emerin and Ce-MAN1 share at least one and possibly multi

42 uired for the association of viral cDNA with emerin and for the ability of emerin to support virus in

43 Additionally, emerin and lamin A are also required to maintain nuclear

44 Emerin and lamin A are implicated in regulating muscle-

45 residue) vertebrate proteins associated with emerin and lamin A at the nuclear envelope of muscle cel

46 Mutations in the nuclear envelope proteins emerin and lamin A cause a number of diseases including

47 er-4 phosphorylation inhibits BAF binding to emerin and lamin A, and thereby weakens emerin-lamin int

48 es for the nuclear membrane/lamina proteins, emerin and lamin A/C.

49 al proteins at the nuclear membrane, such as emerin and lamin AC, cause muscular dystrophy and cardio

50 t functions, for example in the targeting of emerin and lamin C to the nuclear envelope.

51 The human nuclear envelope proteins emerin and lamina-associated polypeptide 2alpha (LAP2alp

52 ric, spectrin-repeat proteins that bind both emerin and lamins A/C and form a network in muscle linki

53 g the inner nuclear membrane (INM) proteins, emerin and lamins A/C.

54 We thus conclude that emerin and LAP2alpha are not required for the early repl

55 from emerin knockout, LAP2alpha knockout, or emerin and LAP2alpha double knockout mice to be comparab

56 ittermate-derived MEFs, indicating that both emerin and LAP2alpha were dispensable for HIV-1 and MLV

57 ice deficient for emerin, LAP2alpha, or both emerin and LAP2alpha.

58 recruitment of the nuclear membrane proteins emerin and Lem-domain-containing protein 2 (LEMD2) to ru

59 Emerin and LEM2 are ubiquitous inner nuclear membrane pr

60 To test the roles of emerin and LEM2 in somatic cells, we used null alleles o

61 This supports a model where emerin and MAN1 are at least partly retained in the inne

62 Emerin and MAN1 are LEM domain-containing integral membr

63 Emerin and MAN1 homologs are both conserved in Caenorhab

64 The nuclear envelope proteins LAP2, emerin and MAN1 share a conserved approximately 40-resid

65 We show that emerin and MAN1, but not LBR, are more mobile in the inn

66 lamin A, and transcription regulators, plus emerin and other LEM-domain nuclear proteins.

67 1 interacts physically and functionally with emerin and plays an essential and selective role in skel

68 in (Em(LEM)) of the nuclear envelope protein emerin and plays an essential role in the nuclear archit

69 Emerin and pUL34 interact physically, as shown by pull-d

70 ER to the NE, where Sig-1Rs bind NE protein emerin and recruit chromatin-remodeling molecules, inclu

71 morphology defects and specific patterns of emerin and SUN2 mislocalization.

72 morphological changes and mislocalization of emerin and SUN2 observed in patient fibroblasts.

73 Specific binding between emerin and the functional C-terminal domain of YT521-B w

74 nfection causes a loss of connection between emerin and the lamina.

75 rk has identified nuclear envelope proteins--emerin and the linker of the nucleoskeleton and cytoskel

76 TAN lines formed normally in cells lacking emerin and were coordinated with the erratic nuclear mov

77 egulates many EDMD-relevant genes (including emerin), and is feedback-regulated by binding to emerin.

78 n IIB specifically coimmunoprecipitated with emerin, and emerin depletion prevented myosin IIB locali

79 We analyzed emr-l, which encodes Ce-emerin, and lem-2, which encodes Ce-MAN1.

80 Mutations in EMD, encoding emerin, and LMNA, encoding A-type lamins, respectively,

81 s is defined by an approximately 40-aa LAP2, Emerin, and MAN1 (LEM) domain (LEM-D) that binds the non

82 y to LEM domain nuclear proteins (e.g. LAP2, Emerin, and MAN1), lamin A, homeodomain transcription fa

83 ly binds 21 known partners, including actin, emerin, and SREBP1, but how these interactions are regul

84 his in turn creates intranuclear polarity in emerin, and thereby controls nuclear actin filaments tha

85 Depletion of the BAF interactors LEMD2 or emerin, and to a lesser extent lamin A/C, increased the

86 nked recessive loss-of-function mutations of emerin, another component of the inner nuclear lamina th

87 However, in the absence of Ce-emerin, approximately 90% reduction of Ce-MAN1 was letha

88 tion of this gene lacking both lamin A/C and emerin are born at the expected Mendelian ratio but had

89 Mice without lamin A/C and emerin are born at the expected Mendelian ratio, are gro

90 The LEM domain and central region of emerin are essential for binding to BAF and lamin A, res

91 The functions of emerin are poorly understood, but EDMD affects mainly sk

92 a human heart cDNA library, with full-length emerin as bait.

93 antibodies against emerin to affinity-purify emerin-associated protein complexes from HeLa cells and

94 ion specifically and selectively reduced GFP-emerin association with BAF by 58% and also increased GF

95 in the second region, is proposed to promote emerin association with BAF in the chromatin/lamin B "ni

96 P4 cells following potent down-regulation of emerin, BAF, or LAP2alpha protein by using short interfe

97 , after affinity purification against a pure-emerin band on a western blot, it stained only the nucle

98 In cells infected with wild-type virus, emerin becomes more mobile in the nuclear membrane, and

99 Emerin belongs to the "LEM domain" family of nuclear pro

100 Emerin binding stimulated the catalytic activity of HDAC

101 l, wild-type BAF but not mutant S4E enhanced emerin binding to lamin A in vitro.

102 -Dreifuss muscular dystrophy by showing that emerin binds directly to a transcriptional repressor, GC

103 Here we show that emerin binds directly to HDAC3, the catalytic subunit of

104 We now report that emerin binds nuclear myosin I (NMI, a molecular motor) d

105 Furthermore, bead-conjugated emerin bound nuclear alphaII-spectrin and NMI equally we

106 NET25 rescues myogenesis after depletion of emerin but not after MAN1 silencing.

107 Overexpression of wild-type emerin, but not emerin mutant P183H (which causes EDMD a

108 binding to emerin in vitro, predicting that emerin can form at least two distinct types of complexes

109 ms for how specific mutations in lamin A and emerin cause cardiac- or muscle-specific disease.

110 in LMNA encoding lamin A/C and EMD encoding emerin cause cardiomyopathy and muscular dystrophy.

111 Mutations in emerin cause X-linked recessive Emery-Dreifuss muscular

112 Loss of human emerin causes Emery-Dreifuss muscular dystrophy (EDMD).

113 Loss of emerin causes Emery-Dreifuss muscular dystrophy, a tissu

114 relevant because loss or mislocalization of emerin causes Emery-Dreifuss muscular dystrophy.

115 Loss of emerin causes Emery-Dreifuss muscular dystrophy.

116 The envelope localization of Ce-lamin, Ce-emerin, Ce-MAN1, and nucleoporins are unaffected by the

117 n regions of HiLands-B and NL as measured by emerin-chromatin interaction.

118 Proposed MAN1-emerin complexes are discussed in the context of EDMD di

119 clusion chromatography yielding six distinct emerin-containing fractions (0.5-1.6 MDa).

120 n lacked ascribed functions, suggesting that emerin could have additional partners.

121 esponse to mechanical strain was impaired in emerin-deficient cells, and prolonged mechanical stimula

122 echanical stimulation increased apoptosis in emerin-deficient cells.

123 Emerin-deficient mouse embryo fibroblasts have abnormal

124 Thus, emerin-deficient mouse embryo fibroblasts have apparentl

125 tiation potential of lamin A/C-deficient and emerin-deficient myoblasts may in part underlie the dyst

126 lar strain experiments, and the integrity of emerin-deficient nuclear envelopes appeared normal in a

127 Only one complete emerin deletion has been reported previously.

128 tional nuclear movement and actin flow as in emerin-depleted cells.

129 ically coimmunoprecipitated with emerin, and emerin depletion prevented myosin IIB localization near

130 A polyclonal rabbit antiserum against emerin did recognize both nuclear membrane and intercala

131 e report that Lim-domain only 7 (Lmo7) binds emerin directly with 125 nM affinity; the C-terminal hal

132 ions to their nuclear envelope integrity and emerin distribution.

133 ins from HeLa cell nuclear lysates that bind emerin either directly or indirectly.

134 sed by mutations in either the X-linked gene emerin (EMD) or the autosomal lamin A/C (LMNA) gene.

135 Several mAbs against different emerin epitopes did not recognize intercalated discs in

136 Emerin expression is not, however, necessary for infecti

137 ectively, these data support a model whereby emerin facilitates repressive chromatin formation at the

138 We determined the binding affinities of emerin for GCL, BAF, and lamin A and analyzed their olig

139 We showed that emerin forms stable complexes with either lamin A plus G

140 GCL co-immunoprecipitates with emerin from HeLa cells.

141 iac defects in EDMD are caused by absence of emerin from intercalated discs.

142 rane helix caused almost complete absence of emerin from muscle with no localization to the nuclear m

143 ow--defects that are mediated by the loss of emerin from the nuclear envelope.

144 t lack the integral nuclear membrane protein emerin, from an individual with X-linked Emery--Dreifuss

145 hts the present understanding of lamin A and emerin function in regulating nuclear architecture, gene

146 Emerin function is likely to be important because, when

147 These results show that emerin functions with myosin IIB to polarize actin flow

148 The 20 kb deletion includes the entire emerin gene and extends well into most of the distal inv

149 ercalated discs were due to a product of the emerin gene and, therefore, cast some doubt upon the hyp

150 Two key results show that Lmo7 regulates emerin gene expression: rat Lmo7 isoforms directly activ

151 inds emerin protein but is also required for emerin gene transcription.

152 ovel deletion of the last three exons of the emerin gene, a carrier had a cardiomyopathy and very low

153 Fourteen had mutations in the X-linked emerin gene, while three showed evidence of autosomal in

154 Because emerin has been suggested to be important for infection

155 ed in Caenorhabditis elegans, but loss of Ce-emerin has no detectable phenotype.

156 Emerin has proposed roles in nuclear architecture and ge

157 Human fibroblasts that are null for emerin have an autostimulatory growth phenotype.

158 Thus, NET25 and emerin have at least partially overlapping functions dur

159 ) down-regulated for either A-type lamins or emerin have impaired differentiation potentials.

160 lamins and lamina-associated proteins, like emerin, have a regulatory role, as well as a structural

161 ation with BAF by 58% and also increased GFP-emerin hyper-phosphorylation.

162 Some emerin hyperphosphorylation can be inhibited by the prot

163 Infection causes hyperphosphorylation of emerin in a manner dependent upon both pUL34 and pUS3.

164 , we describe the derivation of mice lacking emerin in an attempt to derive a mouse model for EDMD1.

165 These results indicate a new function for emerin in cell polarization and suggest that laminopathi

166 Biochemical roles for emerin in gene expression are discussed.

167 tide libraries and has been used to localize emerin in human and rabbit heart.

168 As the expression of human emerin in mouse knockout cells fails to affect the level

169 studies indicate roles for A-type lamins and emerin in myogenic differentiation and also suggest that

170 role for the inner nuclear membrane protein emerin in orienting human immunodeficiency virus type 1

171 s prompted us to reexamine the role of human emerin in supporting HIV-1 and MLV infection.

172 tantly, BAF competed with GCL for binding to emerin in vitro, predicting that emerin can form at leas

173 f human Lmo7 (hLmo7C) was sufficient to bind emerin in vitro.

174 elated with normal or increased affinity for emerin in vitro.

175 HIV-1 cDNA associated with emerin in vivo, and the interaction of viral cDNA with c

176 Moreover, following siRNA knockdown of emerin in wild-type fibroblasts, the centrosome also bec

177 Thus, disruptions in nesprin/lamin/emerin interactions might play a role in the muscle-spec

178 Emerin interacts with beta-catenin through a conserved a

179 We show that emerin interacts with tubulin, and that nocadozole-treat

180 stinct breakpoints within the 4.7 kb filamin-emerin intergenic region, suggesting that loss of filami

181 The type II inner nuclear membrane protein emerin is a component of the LINC complex that connects

182 Emerin is a nuclear membrane protein that interacts with

183 Emerin is a nuclear membrane protein which is missing or

184 Emerin is a type II inner nuclear membrane (INM) protein

185 When GFP-emerin is expressed in HEK293 cells, beta-catenin is res

186 Emerin is immediately distal to the 26 kb filamin gene,

187 The function of MAN1 is unknown, whereas emerin is known to interact with nuclear lamins, barrier

188 We also find that a significant fraction of emerin is located at the outer nuclear membrane and peri

189 shapen and structurally weakened nuclei, and emerin is mislocalized away from the nuclear envelope.

190 ce of emerin protein and, by extension, that emerin is not a universally important regulator of HIV-1

191 This suggests that emerin is not necessary for localizing chromosomes at th

192 antipoxviral effector, while the presence of emerin is not required.

193 Emerin is rapidly cleared from the INM by a mechanism th

194 However, the LEM domain protein emerin is recruited to cytoplasmic DNA in a BAF-dependen

195 Here we show that one function of Emerin is to regulate the flux of beta-catenin, an impor

196 y in mouse embryonic fibroblasts (MEFs) from emerin knockout, LAP2alpha knockout, or emerin and LAP2a

197 However, two other conserved regions of emerin lacked ascribed functions, suggesting that emerin

198 g to emerin and lamin A, and thereby weakens emerin-lamin interactions during both mitosis and interp

199 ocalization of nesprins and impaired nesprin/emerin/lamin binding interactions were common features o

200 nd cytoskeleton because of perturbed nesprin/emerin/lamin interactions.

201 infect primary cells from mice deficient for emerin, LAP2alpha, or both emerin and LAP2alpha.

202 s been recruited by pUL34 hyperphosphorylate emerin, leading to disruption of its connections with la

203 Disruption of NL proteins such as lamin and emerin leads to developmental defects and human diseases

204 a carrier had a cardiomyopathy and very low emerin levels (<5% of normal) due to skewed X-inactivati

205 Emerin levels in female carriers often deviated from the

206 Lmna+/- mice also lacking emerin live to at least one year and have no significant

207 er-4-nonphosphorylated BAF normally promotes emerin localization at the nuclear envelope.

208 Changes in emerin localization, nuclear shape, and lamin organizati

209 -autointegration factor (BAF), the LEM (LAP, emerin, MAN) binding partner of emerin, was required for

210 F, but not by a BAF mutant lacking the Lap2, emerin, Man1 (LEM)-protein binding domain.

211 BAF binds directly to LEM (LAP2, emerin, MAN1) domain nuclear membrane proteins, includin

212 eh2p-two conserved members of the LEM (Lap2, emerin, MAN1) family of integral inner nuclear membrane

213 Emerin, MAN1, and LAP2 are integral membrane proteins of

214 Emerin, MAN1, and LBR are three integral inner nuclear m

215 e then studied the diffusional mobilities of emerin, MAN1, and LBR using fluorescence recovery after

216 cleoporin ELYS, lamin B1, and four proteins (emerin, MAN1, LAP1, and LBR) of the inner nuclear membra

217 mediated by its ESCRT-II domain and the LAP2-emerin-MAN1 (LEM) family of integral inner nuclear membr

218 This network includes LAP2-emerin-MAN1 domain (LEM-D) proteins that associate with

219 ning nuclear size, and propose that the Lap2-Emerin-Man1 domain protein Lem2 acts as a barrier to mem

220 that Lem2p (LEM2), a member of the LEM (Lap2-Emerin-Man1) family of inner nuclear membrane proteins,

221 mediated by Heh2, a member of the LEM (Lap2-emerin-MAN1) family of integral inner nuclear membrane p

222 The loss of emerin may be the basis of nuclear morphological deforma

223 ed that loss of the nuclear envelope protein emerin may mediate the consequences of GATA6 suppression

224 nd skeletal muscle nuclei which already lack emerin may offer an alternative explanation of why these

225 iscovered that these "unascribed" domains of emerin mediate direct binding to a transcriptional repre

226 imetic BAF missense mutant S4E, but not S4A, emerin mislocalized from the nuclear envelope, suggestin

227 mapped to six specific sites throughout the emerin molecule using phage-displayed peptide libraries

228 ated a luciferase reporter gene in vivo, and emerin mRNA expression decreased 93% in Lmo7-downregulat

229 was due, in at least two families, to skewed emerin mRNA expression from the normal and mutated allel

230 Overexpression of wild-type emerin, but not emerin mutant P183H (which causes EDMD and selectively d

231 In contrast, expression of an emerin mutant, lacking its APC-like domain (GFP-emerinDe

232 ws that DNA analysis is necessary to exclude emerin mutations in suspected X-linked EDMD.

233 e model for disease pathogenesis of LMNA and emerin mutations is cell-specific perturbations of the m

234 genes in response to strain, suggesting that emerin mutations may act through altered transcriptional

235 rays), including EDMD patients with LMNA and emerin mutations.

236 odel for downstream consequences of LMNA and emerin mutations.

237 UL34 protein (pUL34) and US3 protein (pUS3), emerin no longer colocalizes with lamins, suggesting tha

238 In fibroblasts depleted of emerin, nuclei moved nondirectionally or completely fail

239 We used antibodies against Ce-MAN1, Ce-emerin, nucleoporins, and Ce-lamin to determine the timi

240 Yet emerin null mice are essentially normal, suggesting the

241 in mice also induces muscle abnormalities in emerin null mice.

242 Emerin-null cells also had significantly less HDAC3 at t

243 Emerin-null cells exhibit an epigenetic signature simila

244 mulated the catalytic activity of HDAC3, and emerin-null cells exhibit increased H4K5 acetylation, wh

245 wn to be misregulated in X-EDMD patients and emerin-null mice was confirmed by real-time PCR.

246 In emerin-null or -deficient human dermal fibroblasts we fi

247 growth is not impaired in cells derived from emerin-null transgenic mice.

248 py the detached centrosome characteristic of emerin-null/deficient cells.

249 Emerin O-GlcNAcylation was reduced ~50% by S53A or S54A

250 ced emerin production, but normal amounts of emerin of normal size were found in leucocytes and lymph

251 Inhibition by emerin of YT521-B-dependent splice site selection in viv

252 Here, we examined the function of emerin on nuclear mechanics and strain-induced signaling

253 Mutations in either emerin or lamin A/C cause Emery-Dreifuss muscular dystro

254 omorphic (LEM-2-null and heterozygous for Ce-emerin) or null for both proteins.

255 ent NL partnership between the LEM-D protein Emerin/Otefin and BAF, revealing that BAF functions with

256 F contributions in the ovary, a tissue where Emerin/Otefin function is essential.

257 In most tissues, only Emerin/Otefin is required for NL enrichment of BAF, reve

258 These data suggest that, similar to emerin/otefin mutants, BAF depletion activates the NL ch

259 BAF knockdown causes phenotypes that mirror emerin/otefin mutants.

260 action (RT-PCR) validations in both LMNA and emerin patient muscle.

261 ter region was expected to result in reduced emerin production, but normal amounts of emerin of norma

262 to be important because, when it is mutated, emerin promotes both skeletal muscle and heart defects.

263 efficiently infects cells in the absence of emerin protein and, by extension, that emerin is not a u

264 Thus, Lmo7 not only binds emerin protein but is also required for emerin gene tran

265 e been studied both by DNA sequencing and by emerin protein expression.

266 Mouse embryo fibroblasts ablated for emerin protein through gene knockout support the same le

267 26 kb filamin gene, and flanking the filamin-emerin region are two large inverted repeats.

268 in many laminopathies, whereby lamin A/C and emerin regulate gene expression through modulation of nu

269 ional repressor, GCL, and by suggesting that emerin-repressor complexes might be regulated by BAF.

270 ed from the INM by a mechanism that requires emerin's LEM domain to mediate vesicular trafficking to

271 These findings support the hypothesis that emerin scaffolds a variety of functionally distinct mult

272 Although mice lacking emerin show no overt pathology, muscle regeneration in t

273 hy, and Lmna(LCO/LCO) cells displayed normal emerin targeting and exhibited only very minimal alterat

274 t-mediated turnover of a single INM protein, emerin, that is potentiated by ER stress.

275 ximately 40-residue LEM homology domain with emerin, the protein mutated in X-linked Emery-Dreifuss m

276 ylated BAF has a positive role in localizing emerin; this role may be disease relevant because loss o

277 isrupt nuclear localization of lamin A/C and emerin, thus linking together two common mechanisms of i

278 Furthermore, we identified emerin, TMEM43, and ELYS as potential interaction partne

279 ese interactions, we used antibodies against emerin to affinity-purify emerin-associated protein comp

280 In mammalian cells, BAF recruits emerin to chromatin during nuclear assembly.

281 le-null animals used the maternal pool of Ce-emerin to develop to the larval L2 stage, then arrested.

282 bust to target green fluorescent protein and emerin to intranuclear sites that contained the UL31 pro

283 iral cDNA with emerin and for the ability of emerin to support virus infection.

284 amin B1 is not essential for localization of emerin to the nuclear lamina.

285 , TBLR1, and NCoR each co-immunoprecipitated emerin, validating one putative complex.

286 Infection of primary macrophages lacking emerin was abortive in that viral cDNA localized to the

287 Although emerin was abundant in the membranes of cardiomyocyte nu

288 ina" structure, or with a complex containing emerin was confirmed by GFP pull down.

289 A 'bipartite' binding site for YT521-B in emerin was identified using alanine substitution or dise

290 This distribution of emerin was similar to that of lamin A, a candidate gene

291 he LEM (LAP, emerin, MAN) binding partner of emerin, was required for the association of viral cDNA w

292 Using recombinant bead-conjugated emerin, we affinity-purified seven proteins from HeLa ce

293 thesis that MAN1 function might overlap with emerin, we tested different polypeptide fragments of MAN

294 wo nuclear envelope defects (EDMD LMNA, EDMD emerin) were highly related disorders and were also rela

295 -membrane-associated proteins, lamin A/C and emerin, were mislocalized throughout the nucleoplasm.

296 ties of three proteins-SUN2, nesprin-2G, and emerin-were reduced in fibroblasts from children with HG

297 Therefore emerin, which bridges the interface between the inner nu

298 c expression of the nuclear envelope protein emerin, which is mislocalized in Lmna mutant cells and a

299 pes were reproduced by direct suppression of emerin with siRNA.

300 only lamin-A can ensure the localization of emerin within the NL.