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In the present study, we evaluate changes in the expression levels of several nucleoporins and show that the amount of Nup358⁄ RanBP2 within individual NPCs increases during mus-cle diff

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Nup358, a nucleoporin, functions as a key determinant of the nuclear pore complex structure remodeling during

skeletal myogenesis

Munehiro Asally1, Yoshinari Yasuda2, Masahiro Oka1,2, Shotaro Otsuka3, Shige H Yoshimura3, Kunio Takeyasu3and Yoshihiro Yoneda1,2,4

1 Japan Science and Technology Agency, Core Research for Evolutional Science and Technology, Department of Frontier Biosciences, Graduate School of Frontier Biosciences, Osaka University, Japan

2 Department of Frontier Biosciences, Graduate School of Frontier Biosciences, Osaka University, Japan

3 Department of Responses to Environmental Signals and Stresses, Graduate School of Biostudies, Kyoto University, Japan

4 Department of Biochemistry and Molecular Biology, Graduate School of Medicine, Osaka University, Japan

Introduction

Nuclear-cytoplasmic trafficking regulates the

move-ment of molecules into and out of the nucleus and is

necessary for the survival of eukaryotic cells The

pas-sage of molecules such as RNA, proteins and ions

across the nuclear envelope occurs through the nuclear

pore complex (NPC), a huge protein complex

embed-ded in the nuclear envelope Small molecules (< 9 nm

in diameter) pass through the NPC by passive diffu-sion, whereas larger molecules are transported in a facilitated manner [1] The selective nuclear transport

of proteins is directed by specific signal sequences: nuclear localization signals (NLS) for import and nuclear export signals (NES) for export To achieve selective transport, soluble transport factors are

Keywords

NPC remodeling; nuclear pore complex;

nuclear transport; Nup358 ⁄ RanBP2;

skeletal myogenesis

Correspondence

Y Yoneda, Department of Frontier

Biosciences, Graduate School of Frontier

Biosciences, Osaka University, 1–3

Yamada-oka Suita Osaka 565-0871, Japan

Fax: +81 6 6879 4609

Tel: +81 6 6879 4606

E-mail: yyoneda@anat3.med.osaka-u.ac.jp

(Received 23 August 2010, revised 26

November 2010, accepted 3 December

2010)

doi:10.1111/j.1742-4658.2010.07982.x

The nuclear pore complex (NPC) is the only gateway for molecular traf-ficking across the nuclear envelope The NPC is not merely a static nuclear-cytoplasmic transport gate; the functional analysis of nucleoporins has revealed dynamic features of the NPC in various cellular functions, such as mitotic spindle formation and protein modification However, it is not known whether the NPC undergoes dynamic changes during biological processes such as cell differentiation In the present study, we evaluate changes in the expression levels of several nucleoporins and show that the amount of Nup358⁄ RanBP2 within individual NPCs increases during mus-cle differentiation in C2C12 cells Using atomic force microscopy, we dem-onstrate structural differences between the cytoplasmic surfaces of myoblast and myotube NPCs and a correlation between the copy number

of Nup358 and the NPC structure Furthermore, small interfering RNA-mediated depletion of Nup358 in myoblasts suppresses myotube formation without affecting cell viability, suggesting that NUP358 plays a role in myogenesis These findings indicate that the NPC undergoes dynamic remodeling during muscle cell differentiation and that Nup358 is promi-nently involved in the remodeling process

Abbreviations

AFM, atomic force microscopy; DM, differentiation medium; EDMD, Emery–Dreifuss muscular dystrophy; EGFP, enhanced green

fluorescent protein; GM, growth medium; MyHC, myosin heavy chain; NES, nuclear export signal; NLS, nuclear localization signal;

NPC, nuclear pore complex.

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required to interact with both cargo substances and

the NPC One of the best characterized nuclear protein

import pathways is mediated by the importin-a⁄ b

hete-rodimer, which imports basic NLS-containing proteins

Conversely, the nuclear export of leucine-rich

NES-containing proteins is mediated by CRM1 (also known

as exportin-1) [1–3]

A gradient of the small GTPase, RanGTP⁄ GDP,

across the nuclear envelope regulates the binding and

release of cargo by transport factors RanGTP is

abun-dant in the nucleus as a result of the activity of RCC1,

a guanine nucleotide exchange factor for Ran

Con-versely, the concentration of RanGDP is high in the

cytoplasm as a result of the action of RanGAP, which

promotes cytoplasmic GTP hydrolysis by Ran in

con-junction with RanBP1 and⁄ or Nup358 (also known as

RanBP2) RanGTP interacts with transport factors

and regulates the formation of transport complexes

GTP hydrolysis by Ran in the cytoplasm drives

disso-ciation of the export cargo from the export complex,

whereas RanGTP promotes disassembly of the import

complex in the nucleus [1,2]

The NPC is composed of approximately 30 distinct

proteins, known as nucleoporins [4], whose functional

analyses have recently attracted much attention The

general architecture of the NPC is evolutionarily

con-served in all eukaryotes and consists of filaments on

the cytoplasmic surface and spoke rings and a

basket-like structure on the nuclear side of the envelope [5]

In recent years, nucleoporins have been found to play

dynamic roles in a variety of cellular functions, in

addition to their well known function as structural

components of the nuclear pore For example,

nucleo-porins have been shown to be involved in protein

modification and the regulation of mitotic spindle

for-mation [6] Nup358 and the Nup107–160 subcomplex

are localized to kinetochores during mitosis and play a

role in spindle assembly [7] It has long been suggested

that nucleoporins are ubiquitously expressed in all cell

types and at all developmental stages, although recent

studies indicate that some nucleoporins, such as gp210,

are expressed in a cell type- and tissue-specific manner

[8,9] In addition, the structural composition of the

NPC changes during cell differentiation [10], although

it is still unknown whether alterations in NPC

archi-tecture play an important role in cellular

differentia-tion

C2C12 cells are a well established model system for

skeletal muscle differentiation In high-serum growth

medium (GM), these cells grow as mononuclear

myo-blasts, although they fuse to form multinuclear

myotu-bes when cultured in low-serum differentiation

medium (DM) Dynamic remodeling of the nuclear

envelope was reported during C2C12 skeletal muscle differentiation [11], including changes in the distribu-tion of lamins, which are components of the nuclear envelope lamina Mutations within A-type lamin are known to cause several muscle diseases, including Emery–Dreifuss muscular dystrophy (EDMD), and the over-expression of a lamin A⁄ C mutant in C2C12 cells has been shown to reliably mimic the features of EDMD [12] Inner nuclear envelope proteins are also known to regulate NPC dynamics [13] Understanding NPC dynamics during muscle differentiation will not only provide novel information regarding NPC func-tion, but also will contribute to a better understanding

of the pathogenesis of muscular disorders such as EDMD

In the present study, we examine NPC remodeling and its associated functional changes during skeletal muscle differentiation in a C2C12 murine myoblast cell line We compare the composition, architecture and nuclear-cytoplasmic transport activity of NPCs in myoblasts and myotubes We find that dynamic remodeling of the NPC occurs during muscle cell differentiatin

Results

Expression patterns of nucleoporins are altered from myoblasts to myotubes

To determine the expression levels of NPC compo-nents (nucleoporins) during skeletal muscle cell differ-entiation, we used real-time PCR to quantify the mRNA levels of each nucleoporin in C2C12 cells before and after the induction of differentiation (Fig 1) Approximately half of the nucleoporin mRNAs (i.e Nup107, Nup85, Nup160, Nup43, Nup37, Nup35, Nup205, Nup188, Pom121, Ndc1, Nup155, Nup54, Nup62, NupL1, Nup153 and Nup50) were down-regulated after the induction of myogenesis By contrast, the expression levels of some nucleoporin mRNAs (i.e Nup214, Nup88, NupL2 (CG1), Nup133, Seh1, Nup93, Gp210, Nup98, Rae1 and Tpr) remained largely unchanged, and the remaining nucleoporin mRNAs (i.e Nup358 and Sec13) were slightly up-regu-lated These results indicate that the relative expression levels of individual nucleoporins change during muscle cell differentiation

To examine the protein levels of specific nucleopo-rins, we prepared C2C12 cell lysates at different stages

of muscle cell differentiation and immunoblotted with available antibodies against several nucleoporins Lysates from C2C12 cells grown in GM and from cells differentiated for either 2 or 5 days in DM were

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analyzed As shown in Fig 2A, the protein level of

Nup358, a component of the cytoplasmic filaments of

the NPC, gradually increased as muscle cell

differentia-tion proceeded All other nucleoporins tested (Nup160,

Nup98, Nup93 and Nup62) showed no obvious

changes in protein expression levels during

differentia-tion Furthermore, immunofluorescence analysis of

Nup358 revealed a stronger signal in myotubes

com-pared to myoblasts, whereas the Nup98 signal

appeared to be unaltered (Figs 2B and S1) By

con-trast, although anti-Nup358 staining also showed an

intracellular signal (Fig 2B), we considered the

intra-nuclear signal to be nonspecific (Doc S1A) We next

focused on the nuclear envelope signal of anti-Nup358

staining To definitely verify the increased expression

of Nup358 at the NPCs of myotubes, we examined the

Nup358 binding partners, RanGAP and CRM1, which

localize to the nuclear rim in a Nup358-dependent

manner [2] RanGAP staining at the nuclear envelope

was much stronger in myotubes than in myoblasts

(Fig 2C) Although CRM1 staining in myoblasts and

myotubes was comparable when the cells were

permea-bilized after formaldehyde fixation, the differences

became more evident when the cells were fixed with

formaldehyde containing Triton X-100, with stronger

CRM1 signals being observed for myotubes than for

myoblasts (Fig 2D) These immunostaining patterns

of Nup358, CRM1 and RanGAP clearly reveal that

Nup358 expression is up-regulated during skeletal myogenesis in C2C12 cells

To determine whether this increase in Nup358 on the nuclear envelope corresponded to an increase in the number of Nup358 proteins in each NPC, we mea-sured NPC density Consistent with a recent study [14], nuclear pore density was similar in myoblasts and myotubes (Fig 2E), indicating that the composition of the myoblast NPC must differ from that of the myo-tube NPC Specifically, the number of Nup358 pro-teins within individual NPCs increase during skeletal muscle differentiation

An architectural change in the NPC occurs during differentiation from myoblasts to myotubes Because the composition of the NPC changes during differentiation (Figs 1 and 2), we attempted to visual-ize the structure of NPCs at the nanoscale level using atomic force microscopy (AFM) As shown in Fig 3A, AFM images of the NPCs in myoblasts and myotubes were successfully captured It was previ-ously observed that the centers of some NPCs are plugged by complexes passing through the NPC [15–17] Consistent with these studies, we observed that 29.3% of the NPCs in myoblasts were plugged, whereas 54.4% of NPCs in myotubes were plugged (Fig 3B)

Fig 1 The relative mRNA expression levels of nucleoporins differ during muscle differentiation C2C12 cells were grown in GM (blue bars)

or for 2 days in DM (red bars) Nucleoporin mRNA levels were analyzed by real-time PCR (n = 3, PCR reaction; n = 2, RNA extraction) and normalized to tubulin Error bars represent the SEM.

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The NPC characteristics measured in the AFM

profiles were: outer diameter (O), height (H) and upper

rim diameter (Fig 3C) The values were statistically

analyzed (U) using the Mann–Whitney U-test

(P < 0.05 was considered statistically significant) The

outer diameter of NPCs did not differ significantly

between myoblasts and myotubes (Figs 3D and S2A)

Myotubes contain greater amounts of Nup358 than do

myoblasts (Figs 1 and 2) and, because Nup358 is

local-ized in the cytoplasmic filaments of NPCs, we

sus-pected that the heights of myotube NPCs might be

greater than those of myoblasts NPC height appeared

to not be significantly different in myotubes and

myo-blasts (P > 0.05) when unplugged and plugged NPCs

were analyzed separately (Fig 3F)

By contrast, the mean ± SEM upper rim diameter

increased significantly, from 72.5 ± 1.3 nm (n = 99)

in myoblasts to 81.6 ± 1.3 nm (n = 68) in myotubes

(Fig 3E) This trend was observed even when

unplugged and plugged NPCs were analyzed separately

(Fig 3D, E) and the different distributions of NPC

upper rim diameters in myoblasts and myotubes are clearly demonstrated in a histogram (Fig S2C) The upper rim diameter of the NPC increased by approxi-mately 9 nm during myotube formation, whereas the outer diameter and the height of the NPC were not significantly changed Thus, the shape of the NPCs in myoblasts differs from that in myotubes

Nup358 is a cytoplasmically exposed component of the NPC [18] and was increased within individual NPCs during muscle differentiation Thus, we hypothe-sized that increased Nup358 in individual NPCs was responsible for the change in NPC upper rim diameter

To test this, we used AFM to visualize Nup358-depleted NPCs AFM analysis of NPCs revealed that the upper rim diameter of Nup358-depleted NPCs was smaller than for NPCs in control small interfering RNA (siRNA)-treated cells (Fig 4B) By contrast, the height and outer diameter of Nup358-depleted NPCs did not differ significantly from those of the control NPCs (Fig 4A, C) These results indicate that the Nup358 copy number within individual NPCs is

Fig 2 The level of Nup358 protein increases during skeletal myogenesis (A) C2C12 cells were grown in GM (0 days) or in DM for 2 or

5 days Immunoblotting analysis was performed with antibodies against a-tubulin, MyHC (muscle marker protein), Nup160, Nup98, Nup93, Nup62 and mAb414 (B–D) C2C12 myoblast cells (MB) and myotube cells (MT) were cultured on coverslips The Nup358 cells were fixed and stained with antibodies against Nup358, Nup98 or RanGAP1 (C) The cells were fixed with (D, lower panels) or without (D, upper panels) Triton X-100 and then permeabilized and stained with an antibody against CRM1 Cells were observed with confocal microscopy (LSM510) Scale bars = 5 lm (E) Myoblasts and myotubes were stained for RL1 NPC density was determined by counting the dots on the nuclear envelope (mean ± SD).

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closely correlated with the upper rim diameter We

conclude that the architecture of the NPC changes

during muscle differentiation and that Nup358 is a

critical determinant of the NPC architecture

Passive diffusion rate is the same in myoblasts

and myotubes

We next compared molecular trafficking through the

NPCs in myoblasts and myotubes because the NPC is

the only gateway for nuclear-cytoplasmic traffic We

observed both compositional and architectural

differ-ences between NPCs in these two cell types First, we

used fluorescence recovery after photobleaching analy-sis to examine the passive diffusion rate in C2C12 cells stably expressing enhanced green fluorescent protein (EGFP), a protein small enough to passively diffuse through the NPC The nuclear EGFP was photo-bleached, fluorescence recovery was monitored (Fig 5A) and the relative intensity of fluorescence in the nucleus was plotted over time (Fig 5B) In the early phase, the fluorescence recovery curves for myo-blasts and myotubes overlapped with each other, indi-cating that the passive diffusion rate through the NPC

is the same in myoblasts and myotubes We observed apparent differences in the fluorescence intensity at

Fig 3 Structural differences between myoblast and myotube NPCs (A) Denuded nuclei from myoblasts and myotubes visualized by AFM The area shown is 3 · 3 lm Scale bar = 0.5 lm (B) The percentage of unplugged and plugged NPCs (C) The NPC profile was taken from each NPC image and three measurements were obtained for each NPC profile O, I, and H represent the outer diameter, upper rim diameter and height, respectively Outer and upper rim diameters indicate the distance between two points on the NPC ring base and on the cyto-plasmic ring, respectively Height indicates the vertical distance between the NPC cytocyto-plasmic surface and the base Scale bar = 50 nm (D) The outer diameter was measured and plotted [mean ± SEM; 145.4 ± 2.4 nm, n = 58 (MB); 144.8 ± 3.7 nm, n = 23 (MT) for unplugged and 143.9 ± 2.2 nm, n = 29 (MB); 145.7 ± 2.5 nm, n = 37 (MT) for plugged] (E) The upper rim diameter was measured and plotted [mean ± SEM; 70.2 ± 1.9 nm, n = 58 (MB); 78.1 ± 2.3 nm, n = 23 (MT) for unplugged, and 76.1 ± 1.5 nm, n = 29 (MB); 83.2 ± 1.7 nm, n = 37 (MT) for plugged] (F) The height was measured and plotted [mean ± SEM; 5.5 ± 0.16 nm, n = 116 (MB); 5.43 ± 0.31 nm, n = 46 (MT) for unplugged, and 5.2 ± 0.22 nm, n = 58 (MB); 4.8 ± 0.17 nm, n = 74 (MT) for plugged] Data were statistically compared by Mann–Whitney U-tests.

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steady state, although this is likely a result of a

difference in the ratio of the bleached nuclear area to

the total cell volume (Doc S1B)

Nuclear export appears to be more active in

myotubes than in myoblasts

We next compared the nuclear transport activities

before and after muscle differentiation, using

microin-jection to examine the rate of active

nuclear-cytoplas-mic protein transport Either GST-SV40NLS-GFP

(NLS-GFP) or GST-GFP-RevNES-SV40NLS

(GFP-NES-NLS) recombinant protein was microinjected into

the cytoplasm of C2C12 cells, and the rate of nuclear

transport was observed using time-lapse fluorescence

microscopy The nuclear import efficiency of

NLS-GFP did not differ significantly in myoblasts and

myo-tubes (Fig 6A and Videos S1 and S2), suggesting that

nuclear import activity is almost identical in myoblasts

and myotubes

By contrast, the subcellular localization of

microin-jected GFP-NES-NLS was clearly different in the two

cell types In myoblasts, GFP-NES-NLS was primarily

localized to the nucleus, whereas, in myotubes, it was

evenly distributed between the nucleus and the

cyto-plasm (Fig 6B and Videos S3 and S4) It is important

to note that myotubes are polykaryons, whereas

myo-blasts have one nucleus To address the possible effect

of this difference on the subcellular distribution of

GFP-NES-NLS, myoblasts were fused using

polyethyl-ene glycol and GFP-NES-NLS was microinjected into

the cytoplasm of the fused cells (Fig 6D) The

locali-zation in the fused cells was very similar to that in

nor-mal myoblasts, indicating that the differential

distribution of GFP-NES-NLS observed in myoblasts

and myotubes is not simply a result of the difference

in nuclear number Additionally, treatment of myotu-bes with leptomycin B, a specific inhibitor of CRM1-mediated nuclear export, caused the accumulation of GFP-NES-NLS proteins in the nucleus (Fig 6C), showing that GFP-NES-NLS protein is actively shut-tled across the nuclear envelope in myotubes Taken together with the data from the NLS injections, these results strongly suggest that nuclear export efficiency is increased in myotubes relative to myoblasts

siRNA-mediated Nup358 depletion suppresses myotube formation

To determine whether Nup358 is necessary for C2C12 differentiation, we performed siRNA experiments using two different sets of siRNA duplexes against Nup358 As indicated, both Nup358 siRNAs effec-tively reduce Nup358 expression (Fig 7A) Immunoflu-orescence staining demonstrated a marked reduction in the nuclear rim signal for Nup358 in siNup358-treated cells (Fig 7B) Furthermore, the nuclear rim signal of RanGAP, which localizes to the nuclear envelope in a Nup358-dependent manner, was clearly diminished (Fig 7B) Other nucleoporins (Nup214, Nup98 and Nup62) remained unaltered at the nuclear rim in Nup358-depleted cells (Fig 7B), indicating that the depletion of Nup358 did not affect all NPC compo-nents

Microinjection analysis of the GFP-NES-NLS trans-port substrates suggested that nuclear imtrans-port and export of proteins was globally unaffected in Nup358-depleted cells (Figs 7C and S3) Under these condi-tions, the siRNA-treated C2C12 myoblasts were cultured in DM and then stained for myosin heavy chain (MyHC; green) and DNA (blue) As shown in Fig 7D, the efficiency of multinuclear myotube forma-tion was clearly decreased in Nup358-depleted cells, whereas the control cells (left two panels) efficiently underwent myotube formation These results indicate that Nup358 is specifically involved in muscle cell differentiation

Discussion

In the present study, we have demonstrated that both the composition and the architecture of NPCs are altered during the differentiation of C2C12 cells Our real-time PCR data indicate that the mRNA expres-sion levels of several nucleoporins differ between myo-blasts and myotubes (Fig 1) The mRNA levels of a large proportion of nucleoporins decreased during dif-ferentiation (i.e Nup107, Nup85, Nup160, Nup43, Nup37, Nup35, Nup205, Nup188, Pom121, Ndc1,

Fig 4 Nup358 depletion decreases the upper rim diameter of the

NPC C2C12 cells were transfected with nontargeting siRNA or

Nup358 siRNA oligonucleotides (siNup358-2) and incubated for

48 h The outer diameter (A), upper rim diameter (B) and height (C)

of the NPC were measured and shown in a graph as in Fig 3.

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Nup155, Nup54, Nup62, NupL1, Nup153 and Nup50).

Notably, most of the nucleoporins for which mRNA

expression was maintained during differentiation (e.g.,

Nup358, Nup214, Nup88 and NupL2 (CG1), Nup98,

Rae1 and Tpr) are involved in the nuclear export of

proteins or mRNA, indicating that the relative

expres-sion of the nucleoporins involved in nuclear export

increased during myogenesis Consistent with this, we

found that nuclear export in myotubes was more active

than that in myoblasts (Fig 6), whereas the rates of

nuclear import (Fig 6) and passive diffusion (Fig 5)

remained constant

We also showed protein expression levels of some

nucleoporins by immunoblotting (Fig 2) Although

real-time PCR showed decreased expression of Nup107

and Nup62, their protein expression appeared to be

equivalent, as indicated by immunoblotting An

expla-nation for such discrepancies of nucleoporin expression

levels between qPCR and immunoblot results was

offered by a recent study [14] demonstrating that some scaffold nucleoporins, such as members of the Nup107-160 subcomplex, have extremely long protein half-lives compared to other nucleoporins Thus, pro-teins levels remain stable, even if mRNA expression levels are decreased We specifically demonstrated that the copy number of Nup358 within individual NPCs increases during C2C12 muscle differentiation, at both mRNA (Fig 1) and protein (Fig 2) levels Nup358 plays a supportive role in CRM1-mediated nuclear export by stimulating CRM1 recycling [2] Nup358 provides a platform for the disassembly of CRM1 export complexes and the re-import of free CRM1 to the nucleus [19] As shown in Fig 2D, CRM1 signals

on the NPC were increased in myotubes compared to myoblasts, coinciding with the increase of Nup358

in myotube NPCs Although we were unable to demonstrate the direct effects of Nup358 on protein export in myotubes (Fig 7C), it is possible that the

Fig 5 The efficiency of passive diffusion though the NPC is the same in myoblasts and myotubes (A) C2C12 cells stably expressing EGFP were photobleached for 4 s and fluorescence recovery was monitored immediately after bleaching (B) Data were normalized to the pre-bleaching value of 100% and plotted against time [mean ± SEM; n = 5 (myoblasts), n = 12 (myotubes)].

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relative increase in several nucleoporins, which are

known to function in protein export, rather than the

increase of Nup358 alone, may cause the activation of

CRM1 in myotubes It is therefore likely that an

increase in Nup358 during myogenesis supports the

CRM1 activity and is involved in the activation of

nuclear protein export in myotubes

It is highly possible that a selective increase in the

efficiency of nuclear protein export triggers the

redistri-bution of a number of key molecules important for cell

differentiation and that the difference in nuclear export

activity between myotubes and myoblasts affects cell

differentiation from myoblasts to myotubes For

exam-ple, HDAC5 (histone deacetylase 5) is known to move

from the nucleus to the cytoplasm during myotube

for-mation in a differentiation-dependent manner and is

involved in the control of cellular differentiation in

C2C12 [20,21] Similarly, the regulation of

nuclear-cytoplasmic transport by importin-a subtype switching

was shown to trigger cell differentiation [22] Further

studies will be required to elucidate this possibility

The structure of the NPC changes to allow

adapta-tion to different cellular environments during

differen-tiation [8–10] In the present study, using AFM, we

found that the architecture of the NPC changes during

muscle differentiation and that Nup358 is a key

deter-minant of NPC architecture In addition, we showed

that the depletion of Nup358 prevented myotube for-mation (Fig 7) These results indicate that Nup358 affects the structure of the NPC and plays a role in muscle cell differentiation Recent evidence suggests that the NPCs are closely related to the transcriptional machinery [6] and thus it is also possible that the structural changes induced by Nup358 modulate the transcription of genes required for cell differentiation Furthermore, Nup358 is known to be a

multifunction-al protein that acts as both an multifunction-allosteric activator for kinesin [23] and a SUMO E3 ligase [24] Nup358 may therefore contribute to muscle differentiation in C2C12 cells in various ways

In conclusion, in the present study, we demonstrate that the NPC is functionally and structurally regulated during muscle cell differentiation and that Nup358 is required for muscle cell differentiation and also is likely involved in the remodeling of the NPC

Materials and methods

Cells and antibodies

C2C12 myoblast cells were cultured in GM consisting of DMEM (D5796; Sigma, St Louis, MO, USA) supplemented with 10% fetal bovine serum The cells were maintained in an incubator at 5% CO2and 37C To induce differentiation,

Fig 6 Active nuclear export through NPCs

is facilitated in myotubes

GST-SV40NLS-GFP (A) or GST-GST-SV40NLS-GFP-RevNES-SV40NLS (B)

recombinant protein was microinjected with

an injection marker into the cytoplasm of

C2C12 cells and then incubated for 10 min

at 37 C (for NLS substrate, see Videos S1

and 2; for NES-NLS substrate, see Videos

S3 and 4) Scale bars = 50 lm (C)

GST-GFP-RevNES-SV40NLS protein was

microin-jected with an injection marker into the

cytoplasm and then incubated in

LMB-con-taining medium for 10 min at 37 C Scale

bar = 20 lm (D) C2C12 myoblast cells

were fused using polyethylene glycol (PEG).

Recombinant GST-GFP-RevNES-SV40NLS

protein was microinjected with an injection

marker into the cytoplasm of PEG-fused

myoblasts The cells were incubated in

DMEM for 10 min at 37 C Scale

bar = 20 lm.

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GM was replaced with DM consisting of DMEM with 3%

horse serum for 2–5 days For poly(ethylene glycol) fusion,

cells were incubated in 50% PEG-8000 in DMEM (D5796;

Sigma) for 1 min and washed with NaCl⁄ Pi Cells were

then cultured for 2 h in an incubator at 5% CO2 and

37C

Anti-Nup98 sera were kindly provided by Tachibana

et al [25] Antibodies purchased from commercial sources were: mAb414 (MMS-120p; Covance, Princeton, NJ, USA), anti-Nup214 (sc-26055; Santa Cruz Biotechnology, Santa Cruz, CA, USA), anti-Nup160 (sc-27401; Santa Cruz Biotechnology), anti-Nup107 (A301-787A; Bethyl,

Mont-Fig 7 Depletion of Nup358 from C2C12 cells by siRNA C2C12 cells were mock-transfected (no siRNA) or transfected with nontargeting siRNA or Nup358 siRNA oligonucleotides (siNup358-1, -2) and incubated for 48 h (A) Immunoblotting analysis with mAb414 was performed

48 h after transfection Nup62 was used as a loading control (B) Immunofluorescence was performed with antibodies against Nup358, Ran-GAP1, Nup214, Nup98 and Nup62 Scale bars = 10 lm (C) C2C12 cells were transfected with nontargeting siRNA or Nup358 siRNA oligo-nucleotide and incubated for 48 h Recombinant GST-GFP-RevNES-SV40NLS protein was microinjected with an injection marker into the cytoplasm of C2C12 cells transfected with siRNA oligonucleotides (D) C2C12 cells were mock-transfected (no siRNA) or transfected with nontargeting siRNA or Nup358 siRNA oligonucleotides (siNup358-1, -2) The cells were induced to differentiate 2 days after transfection and cultured in DM without transfection reagents for 2.5 days The cells were fixed and stained with anti-MyHC (green) and Hoechst 33342 (blue) Scale bar = 100 lm.

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gomery, TX, USA), anti-Nup358 (PA1-082; Affinity

BioRe-agents, Golden, CO, USA), anti-Nup93 (551976; BD

Pharmingen, San Diego, CA, USA), anti-Nup62 (N43620;

Transduction Lab, Lexington, KY, USA), anti-skeletal

myosin heavy chain (M4276; Sigma) and anti-a-tubulin

(T9026; Sigma)

Plasmids and recombinant proteins

For recombinant GST-GFP-NES-NLS, synthesized

oligo-nucleotides for RevNES (5¢-GATCTCCTCTTCAGCTA

CCACCGCTTGAGAGACTTACTCTTGATTGTAACGA

GGATA-3¢ and 5¢-AGCTTATCCTCGTTACAATCAA

GAGTAAGTCTCTCAAGCGGTGGTAGCTGAAGAGG

A-3¢) were annealed and inserted into the BglII and HindIII

sites of pEGFP-NLS The oligonucleotide fragment for

NES-NLS was flanked with BglII and BamHI, and then

inserted into the BamHI site of pGEX-hGFP Recombinant

GST-GFP-NES-NLS and GST-NLS-GFP proteins were

expressed and purified as described previously [3]

Real-time PCR

Total RNA from C2C12 cells was purified using the RNeasy

kit (Qiagen, Valencia, CA, USA) and cDNA was synthesized

using SuperScript III reverse transcriptase (Invitrogen,

Carlsbad, CA, USA) Quantitative real-time PCR was

per-formed using FastStart SYBR Green Master (Roche, Basel,

Switzerland) with an ABI PRISM 7900HT (Applied

Biosys-tems, Foster City, CA, USA) Primer sets were designed by

Primer Bank [26] and are listed in Table S1

RNA interference

Cells were either mock-transfected or transfected with

syn-thesized siRNAs [nontargeting,

5¢-GCAGCAUCUUUAAU-GAAUAdTdT-3¢ and 5¢-AUAAGUAAUUUCUACGACG

dTdT-3¢; Nup358, 5¢-CCAGUCACUUACAAUUAAAd

TdT-3¢ and 5¢-UUUAAUUGUAAGUGACUGGdTdT-3¢

(siNup358-1), 5¢-UGAAGCACAUGCUAUAAAAdTdT-3¢

and 5¢-UUUUAUAGCAUGUGCUUCAdTdT-3¢

(siNup-358-2)] Transfection with a specific siRNA was performed

using RNAi Max (Invitrogen) in accordance with the

manu-facturer’s instructions The transfected cells were harvested

48 h (for Nup358) or 72 h (for Nup107) after transfection

and fixed for immunofluorescence, lysed for immunoblotting

or used for induced myotube formation in DM without

siR-NA reagents

Immunoblotting and immunofluorescence

For immunoblotting, C2C12 cells were lysed in NP-40

buf-fer (150 mm sodium chloride, 1% NP-40 and 50 mm Tris,

pH 8.0), analyzed by SDS⁄ PAGE and immunoblotted

using Immobilon-P (Millipore, Billerica, MA, USA) with

standard semidry methods The ECL detection reagent (GE Healthcare, Milwaukee, WI, USA) was used for pro-tein visualization

For immunofluorescence, C2C12 cells were grown on glass coverslips (Matsunami, Osaka, Japan) The cells were washed twice in NaCl⁄ Pi, fixed with 3.7% formaldehyde in NaCl⁄ Pi for 10 min, and permeabilized with 0.5% Triton X-100 in NaCl⁄ Pifor 5 min For Nup358 staining, the cells were permeabilized simultaneously with fixation After incu-bation with 5% skim milk in NaCl⁄ Pi, antibodies were incubated with the cells for 1 h The cells were washed with NaCl⁄ Piand incubated with Alexa 488- or 546-conjugated antibodies for 1 h Samples were then thoroughly washed with NaCl⁄ Pi and mounted in NaCl⁄ Pi-glycerol plus 1,4-diazabicyclo[2.2.2]octane DNA was counterstained with Hoechst 33342 All procedures for immunofluorescence were performed at room temperature The stained cells were observed with a laser-scanning LSM510 microscope (Carl Zeiss, Oberkochen, Germany)

Calculation of NPC density

For the measurement of NPC density, methanol⁄ acetone-fixed cells were stained with mAb414 NPC number in a

2· 2 lm area was counted for both myoblast and myotube nuclei The fluorescence intensity of a spot was used to deter-mine how many pores existed in a diffraction-limited area

AFM

AFM was performed in contact mode with a Molecular Force Probe 3D (MFP-3D; Asylum Research, Santa Bar-bara, CA, USA) using a microcantilever OMCL TR-400 PSA (Olympus, Tokyo, Japan) To prepare the AFM sam-ple, myoblast and myotube cells were cultured on an eight-well slide glass (MP Biomedicals, LLC, Santa Ana, CA, USA) The cells were treated with hypotonic buffer (40 mm NaCl, 5.4 mm KCl, 0.8 mm MgCl, 1 mm NaH2PO4 and

10 mm Hepes, pH 7.4) for 3 min and then treated with buf-fer X (1% Triton X-100, 75 mm KCL, 15 mm NaCl and

20 mm Mops, pH 7.4) for 6 min Denuded nuclei were fur-ther washed with buffer W (15.5 mm NaCl, 70 mm KCl, 6.5 mm K2HPO4and 1.5 mm NaH2PO4) and fixed with 1% glutaraldehyde and 3.7% formaldehyde in NaCl⁄ Pi for

15 min Finally, the nuclei were rinsed with Milli-Q water (Millipore) and air-dried Data were analyzed with Igor-Pro (Wavemetrix Inc., Portland, OR, USA) and statistical anal-yses were performed with GraphPad Prism 4 (GraphPad Software Inc., San Diego, CA, USA)

Fluorescence recovery after photobleaching

To establish stable cell lines, transfection was carried out with Effectene Transfection Reagent (Qiagen) Ssp1-digested pIRES-puro3-EGFP was transfected into C2C12

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