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Tiêu đề Functional interaction between RNA helicase II⁄Gua and ribosomal protein L4
Tác giả Hushan Yang, Dale Henning, Benigno C. Valdez
Trường học Baylor College of Medicine
Chuyên ngành Pharmacology
Thể loại báo cáo khoa học
Năm xuất bản 2005
Thành phố Houston
Định dạng
Số trang 15
Dung lượng 478,08 KB

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Nội dung

We cotransfected FLAG-tagged human RPL4 with hemagglutinin HA-tagged wild-type, DEVD mutant or SAT mutant form of human Gua into HeLa cells and did immunopreci-pitation with anti-FLAG re

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ribosomal protein L4

Hushan Yang, Dale Henning and Benigno C Valdez

Department of Pharmacology, Baylor College of Medicine, Houston, Texas, USA

Ribosome biogenesis is a complicated cellular process

which occurs in the nucleolus [1] The entire scenario

begins at the end of mitosis and includes ribosomal

DNA transcription, pre-ribosomal RNA (pre-rRNA)

modifications and processing as well as assembly of

rRNAs and ribosomal proteins into preribosome

sub-units which are then exported to the cytoplasm to

form the mature ribosomes [2] Errors in this process

are reported to be associated with several diseases

[3–8] The availability of genetic manipulations makes

ribosome biogenesis much better studied in yeast than

in higher eukaryotes such as mammalian and frog

sys-tems, resulting in the identification of more than 80

yeast ribosomal proteins and numerous trans-acting

elements including small nucleolar RNAs (snoRNAs)

as well as nonribosomal proteins However, in

mam-malian systems, ribosome biogenesis is far from being

thoroughly understood due to the increased

complex-ity To date, only a few nucleolus-localized

nonribo-somal proteins have been implicated in pre-rRNA

processing in mammalian cells and include B23⁄ NO38⁄ NPM [9], C23 ⁄ nucleolin [3], fibrillarin [10,11], p120 [12], EBP1 [13], Bop1 [14] and p19Arf [15] No bona fide RNA helicase has been implicated in this process in higher eukaryotes except RNA helicase

II⁄ Gua [16,17]

RNA helicase II⁄ Gua is a multifunctional nucleolar protein with in vitro RNA-dependent ATPase activity, ATP-dependent RNA helicase activity and GTP-stimu-lated RNA foldase activity [17–19] The presence of both RNA unwinding and RNA folding activities in two distinct domains of the same protein highly sug-gests a role of Gua in rRNA biogenesis [19] Using antisense oligodeoxynucleotide and siRNA to down-regulate Gua expression in Xenopus oocytes [16] and mammalian cells [17], respectively, we demonstrated that Gua is important for 18S and 28S rRNA produc-tion in both systems In addiproduc-tion, Gua was also showed

to participate in other major cellular activities such as cell growth and differentiation [19,20], regulation of

Keywords

ribosomal protein; ribosomal RNA

biogenesis; RNA helicase; nucleolus

Correspondence

B C Valdez, Department of Pharmacology,

Baylor College of Medicine, Houston,

TX 77030, USA

Fax: +1 713 798 3145

Tel: +1 713 798 7908

E-mail: bvaldez@bcm.tmc.edu

(Received 11 April 05, revised 19 May 05,

accepted 9 June 05)

doi:10.1111/j.1742-4658.2005.04811.x

RNA helicase II⁄ Gua is a multifunctional nucleolar protein involved in ribosomal RNA processing in Xenopus laevis oocytes and mammalian cells Downregulation of Gua using small interfering RNA (siRNA) in HeLa cells resulted in 80% inhibition of both 18S and 28S rRNA production The mechanisms underlying this effect remain unclear Here we show that

in mammalian cells, Gua physically interacts with ribosomal protein L4 (RPL4), a component of 60S ribosome large subunit The ATPase activity

of Gua is important for this interaction and is also necessary for the func-tion of Gua in the producfunc-tion of both 18S and 28S rRNAs Knocking down RPL4 expression using siRNA in mouse LAP3 cells inhibits the pro-duction of 47⁄ 45S, 32S, 28S, and 18S rRNAs This inhibition is reversed

by exogenous expression of wild-type human RPL4 protein but not the mutant form lacking Gua-interacting motif These observations have sug-gested that the function of Gua in rRNA processing is at least partially dependent on its ability to interact with RPL4

Abbreviations

aa, amino acid; GST, glutathione S-transferase; Gua, RNA helicase II ⁄ Gua; HA, hemagglutinin; IPTG, isopropylthio-b- D -galactoside; NLS, nuclear localization signal; NoLS, nucleolar localization signal; RPL4, Ribosomal Protein L4; rDNA, ribosomal DNA; rRNA, ribosomal RNA; RNP, ribonucleoprotein; siRNA, small interfering RNA; snRNA, small nuclear RNA; snoRNA, small nucleolar RNA.

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c-Jun-mediated gene expressions [21] and in vitro

clea-vage by PIAS1 [22] It is unclear whether the effect of

Gua on cell proliferation is due to its involvement

in ribosomal RNA production, c-Jun-mediated gene

expression or, as yet, other undiscovered mechanisms

RNA helicases are believed to function through

regulation of RNA structure rearrangement and

RNA-RNA, RNA-protein or protein–protein interactions

[23,24] To date, at least 18 putative ATP-dependent

RNA helicases have been suggested to contribute to

ribosome production in yeast S cerevisiae [25] It is

hypothesized that RNA helicases interact with other

trans-acting protein factors in preribosomal particles

during pre-rRNA processing and ribosome assembly

to modulate specific intracellular RNA structures

[26–28] To test the hypothesis that Gua may function

by interacting with other nucleolar proteins in rRNA

production, we did immunoprecipitation and identified

ribosomal protein L4 physically interacting with Gua

in an RNA-independent manner It was also

demon-strated that the interaction is necessary for the function

of Gua in 28S rRNA production in mammalian cells

Results

Ribosomal protein L4 physically interacts with

RNA helicase II⁄ Gua

In our search for Gua-interacting proteins, we used

isopropylthio-b-d-galactoside (IPTG) to induce a stable

LAP3 clone expressing FLAG epitope-tagged mouse

Gua protein Two days after induction, the cells were

lysed, treated with RNase A to rule out any protein that

may be in the complex due to binding to the same RNA,

and subjected to immunoprecipitation using anti-FLAG

Ig resin (Sigma, St Louis, MO) The immunoprecipitates

were resolved on a sodium dodecyl

sulfate–polyacryl-amide (10%) gel and analyzed by silver staining Several

stained bands were detected in the FLAG-Gua lane but

not in the control lane (Fig 1A) One of the major

bands with molecular weight of approximately 50 kDa

was recovered and sent for mass spectrometry

sequen-cing It turned out to be ribosomal protein L4 There

were other faster-migrating bands in the FLAG-Gua

lane which were of greater abundance than those in

the control lane (Fig 1A) We did not sequence these

bands, but we suspect they represent other ribosomal

proteins and⁄ or trans-acting factors of a large nucleolar

complex essential for ribosome biogenesis

The yeast two-hybrid system was used to prove the

direct interaction between Gua and RPL4 We

sub-cloned human Gua and RPL4 into pGBKT7 and

pGADT7 yeast expression vectors, respectively The

growth of yeast cells containing both Gua and RPL4

in a triple drop-out medium that lacks tryptophan, leucine, and histidine indicates interaction of the two proteins (Fig 1B, right) The specificity of Gua–RPL4 interaction is shown by the inability of the yeast clones that harbor RPL4 and p68, a DEAD-box helicase implicated in RNA splicing and export [29], or RPL4 and p53, to grow in a triple drop-out medium (Fig 1B, right) The growth of yeast cells containing the above expression constructs in a double drop-out medium that lacks tryptophan and leucine indicates that these constructs were expressed (Fig 1B, left) Because protein–protein interactions shown by yeast two-hybrid system are not always direct, we performed

an in vitro pull down assay using bacterially expressed GST-RPL4 and untagged Gua mixed together and pulled down with GSH-resin Gua was pulled down with GST-RPL4, which further supported the direct interaction between Gua and RPL4 (Fig 1C)

The in vivo association of Gua with RPL4 was shown in both human HeLa cells and mouse LAP3 cells We cotransfected either FLAG-tagged Gua and protein A-tagged RPL4, or FLAG-tagged RPL4 and protein A-tagged Gua into HeLa cells and did immu-noprecipitation using anti-FLAG resin Figure 1D shows that both overexpressed (proA-RPL4) and endogenous RPL4 interact with Gua Figure 1E shows that both overexpressed (proA-Gua) and endogenous Gua interact with RPL4 We tried using either anti-Gua or anti-RPL4 Ig to do similar experiments to show an association of endogenous Gua and endo-genous RPL4 However, neither antibody worked for immunoprecipitation although they performed well in western blot analyses

To determine the expression and cellular localization

of RPL4 protein, anti-FLAG Ig was used for indirect-immunofluorescence of HeLa cells transfected with FLAG-tagged RPL4 GFP-tagged Gua was cotrans-fected as a control to show the positions of nucleoli The localization of RPL4 to the nucleolus further indi-cates the role it may play in rRNA processing and ribosome assembly (Fig 1F) RPL4 is a component of 60S ribosome large subunit with proposed cytoplasmic localization However, we did not observe strong cyto-plasmic fluorescent signal for FLAG-RPL4 This observation has been shown with other reported ribo-somal large subunit proteins such as L23 [30]

A DEVD (Asp-Glu-Val-Asp) mutant of RNA helicase II⁄ Gua does not interact with RPL4 The DEVD motif of RNA helicases is critical for their ATPase activity which is necessary for RNA

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helicase-mediated reorganization of RNA⁄ protein structure

[23] We previously showed that the DEVD motif of

Gua is important to 18S and 28S rRNA production

[17] Since we surmised that Gua–RPL4 interaction is

necessary for Gua to function in pre-rRNA processing,

we sought to determine if the DEVD motif is also

important to the Gua–RPL4 interaction DEVD was

mutated to ASVD with reported abolishment of both

ATPase and helicase activities [16] An SAT mutant, in

which the SAT motif was mutated to LET, was used

as a control This mutant still retains ATPase activity but not RNA helicase activity [19] We cotransfected FLAG-tagged human RPL4 with hemagglutinin (HA)-tagged wild-type, DEVD mutant or SAT mutant form

of human Gua into HeLa cells and did immunopreci-pitation with anti-FLAG resin Figure 2A shows that FLAG-RPL4 was pulled-down efficiently in all three precipitates However, only wild-type Gua was also present in the precipitate, suggesting the relevance of the DEVD and SAT motifs in its association with

F D

C A

B

E

Supernatant

WB: anti-FLAG FLAG-Guα

Gu α

FLAG-RPL4 ProA-Gu α ProA-RPL4

RPL4 WB: anti-Guα

WB: anti-FLAG

Input Sup’

IP Input Sup’

WB: anti-RPL4

Pellet

Mock GST

GST Mock GST

Gu α

Gu α

RPL4

Other proteins

Double drop-out

(No Trp, No Leu)

Triple drop-out (No Trp, No Leu, No His)

IgG-H

Fig 1 Gua interacts with RPL4 (A) Stable LAP3 clones were induced with 2 m M IPTG for 48 h to express FLAG-tagged mouse Gua RNase A-treated lysates were used in immunoprecipitation using anti-FLAG resin Silver staining shows the precipitation of 50-kDa protein (RPL4)

in cells expressing mouse Gua but not in cells expressing vector alone (B) Yeast two-hybrid analysis showing the interaction of human RPL4 with human Gua and Gub Yeast clones were grown on selection media Growth in the absence of tryptophan and leucine would indi-cate presence of the appropriate vectors used to clone RPL4 and its candidate partner Presence of colonies in the triple drop-out medium (no tryptophan, no leucine, no histidine) would indicate interaction between RPL4 and the other protein (C) In vitro interaction of RPL4 with Gua Purified GST, GST-RPL4 or blank control was mixed with purified untagged Gua in a binding buffer prior to addition of GSH-resin Cen-trifugation separated the supernatant from the resin Both the supernatant and resin were analyzed by western blot analysis using anti-Gua

Ig (D) Overexpressed Gua interacts with both endogenous and overexpressed RPL4 Extracts from HeLa cells cotransfected with FLAG-tagged Gua and protein A-FLAG-tagged RPL4 were immunoprecipitated using anti-FLAG resin and probed with the indicated antibodies (E) Over-expressed RPL4 interacts with both endogenous and overOver-expressed Gua in HeLa cells Extracts from HeLa cells cotransfected with FLAG-tagged RPL4 and protein A-tagged Gua were immunoprecipitated with anti-FLAG resin and probed with indicated antibodies (F) HeLa cells transfected with FLAG-tagged human RPL4 were stained by indirect immunofluorescence using anti-FLAG Ig Anti-mouse IgG coupled

to rhodamine was used as secondary antibody GFP-tagged human Gua was cotransfected and visualized directly under microscope, as a control showing the position of nucleoli.

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RPL4 This experiment further proved the specificity

of Gua–RPL4 interaction since B23, an abundant

nucleolar phosphoprotein which is also implicated in

ribosomal RNA processing [31], was not pulled-down

by RPL4 (Fig 2A, bottom)

In a similar experiment using mouse cell line,

Xpress-tagged mouse RPL4 was transfected into a

sta-ble LAP3 clone which expressed either IPTG-induced

FLAG-tagged wild-type, DEVD mutant or SAT

mutant form of mouse Gua Immunoprecipitation was

again carried out using anti-FLAG resin and the

pre-cipitate was analyzed by western blot analysis

Figure 2B shows that all three forms of Gua

pulled-down Xpress-tagged mouse RPL4 but with different

efficiencies Wild-type Gua interacted with RPL4 with

the highest efficiency, while the DEVD mutant showed

the least This is demonstrated by comparing the signal

intensities of the precipitates with those of the original

inputs (Fig 2B) The ratio (input : IP) is

approxi-mately 1 : 5 for wild-type, 1 : 1 for SAT mutant and

5 : 1 for DEVD mutant The observed difference in the sensitivity of the two experiments (Fig 2A,B) might be attributed to difference in the levels of expression of FLAG-Gua and FLAG-RPL4 The sig-nal intensity of the FLAG-Gua (Fig 2B) is greater than FLAG-RPL4 (Fig 2A), which is possibly due to higher expression level of FLAG-Gua in the LAP3 stable cell line that was induced with IPTG compared

to FLAG-RPL4 that was expressed by transient trans-fection

The DEVD motif is important for the function of Gua in both 18S and 28S rRNA production

We have demonstrated that in Xenopus oocytes, wild-type Gua can reverse the aberrant rRNA processing pattern while the DEVD mutant cannot [16], highlight-ing the importance of the DEVD motif to the function

of Gua in both 18S and 28S rRNA production in Xenopus In the mammalian system, we were able to demonstrate that an SAT mutant, which lacks helicase activity, can restore 28S but not 18S rRNA production

in mouse LAP3 cells [17], which suggests that the SAT motif is important in 18S but not 28S rRNA produc-tion Because the helicase activity is dependent on the presence of the ATPase activity of Gua [18,19], it is reasonable to expect that mutation of the DEVD motif would consequently result in defects of 18S matur-ation However, whether or not the DEVD motif is necessary for 28S production in mammalian cells is unknown Here, a rescue experiment was performed exactly as described [17] to address this issue Briefly, a stable LAP3 clone was induced with IPTG to over-express a DEVD mutant form of the human Gua, after which the cells were treated with si935, an effect-ive siRNA that specifically targets mouse Gua mRNA but not human Gua mRNA Figure 3 shows that treat-ment of the cells with si935 effectively inhibited the production of both 18S and 28S rRNAs (lane 3, com-pared with lanes 1 and 2), which conforms to our pre-vious results [17] However, in this experiment the expression of a DEVD mutant form of human Gua protein did not restore 18S nor 28S rRNA (Fig 3 lane 4) as the wild-type did [17] Thus, we conclude that the DEVD motif is indispensable for the function of human Gua in both 18S and 28S rRNA production, consistent with our results in the Xenopus oocyte [16]

Amino acids 264–333 of human RPL4 is important

to its interaction with Gua Human RPL4 has not been extensively studied after its cloning [32] Human and mouse RPL4 are 90%

A

B

Input Sup’

Input IP Input IP Input IP

Input Sup’

IP Input Sup’

WB: anti-FLAG

FLAG-RPL4

HA-Gu (WT)

FLAG-RPL4 HA-Gu (SAT-M)

FLAG-RPL4 HA-Gu (DEVD-M) IP: anti-FLAG

IP: anti-FLAG

WB: anti-HA

WB: anti-B23

WB: anti-FLAG

WB: anti-Xpress

WB: anti-B23

FLAG-Guα

Xpress-RPL4

Xpress-RPL4

FLAG-Guα (WT) FLAG-Guα (SAT-M)Xpress-RPL4 FLAG-Gu (DEVD-M)Xpress-RPL4

B23

FLAG-RPL4

HA-Guα B23

Fig 2 The DEVD motif of Gua is important to Gua–RPL4

interac-tion (A) HeLa cells were cotransfected with FLAG-tagged

human-RPL4 and plasmids encoding HA-tagged wild-type (WT), SAT

mutant (SAT-M) or DEVD mutant (DEVD-M) form of human Gua.

Whole cell extracts were immunoprecipitated using anti-FLAG resin

and probed with anti-FLAG, anti-HA or anti-B23 Ig (B) LAP3 cells

were transfected with Xpress-tagged mouse RPL4 and induced

with 2 m M IPTG for 48 h to express FLAG-tagged wild-type, SAT

mutant or DEVD mutant of mouse Gua Whole cell extracts were

immunoprecipitated using FLAG resin and probed with

anti-FLAG, anti-Xpress or anti-B23 Ig.

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homologous in their cDNA-derived amino acid

sequences The 98 amino acid C-terminal of human

RPL4 protein has little homology with its mouse

homologue However, their N-termini (amino acids 1–

333) are 99% identical with differences in only three

amino acid residues In higher eukaryotes, other than

the proposed involvement in ribosome assembly, RPL4

has been implicated in cell proliferation and

differenti-ation during rat neurogenesis [33] with unknown

mech-anism In yeast, ribosomal protein RPL2 is most

homologous to human RPL4 The yeast RPL2 has

two copies, RPL2A and RPL2B [34] A decrease in the

expression of RPL2A leads to reduced production of

60S large subunits and mature ribosomes, which

conse-quently results in slower growth rates [34] These data

suggest the relevance of RPL4 in lower and higher

eukaryotes We hypothesize that the function of RPL4

is partly regulated by protein–protein interactions

To determine the RPL4 domains involved in Gua

interaction, we generated FLAG-tagged human RPL4

deletion mutants (Fig 4A,D), expressed them in HeLa cells and tested their ability to bind with Gua via immunoprecipitation Analysis of the overexpressed proteins by indirect immunofluorescence showed that wild-type RPL4 (amino acids 1–428), N1 mutant (amino acids 1–264) and C1 mutant (amino acids 131– 428) predominantly localize to the nucleolus but the C2 mutant (amino acids 264–428) is dispersed within the nucleus but not in the nucleolus (Fig 4B), indica-ting the region of amino acids 131–264 probably con-tains both the nuclear (NLS) and nucleolar (NoLS) localization signals while amino acids 264–428 may harbor another NLS but no NoLS

Figure 4C reveals that RPL4 C1 and C2 mutants, but not its N1 mutant form, coimmunoprecipitate with Gua, suggesting the Gua-interacting domain resides in amino acids 264–428 of RPL4 We speculated that if Gua–RPL4 interaction is important to cellular func-tions, then the chance should be high that the Gua-interacting domain in RPL4 would be in a conserved region As the region of amino acids 333–428 is not highly conserved among different species, we focused

on amino acids 264–333 as a possible Gua-interacting motif in RPL4 This hypothesis was proved to be cor-rect by coimmunoprecipitation of three mutants har-boring amino acids 264–333 (Fig 4F, M3, M5, M6) The other three RPL4 mutants that lack amino acids 264–333 did not coimmunoprecipitate with Gua (Fig 4F, M1, M2, M4) We observed that two bands are recognized by the anti-FLAG Ig in mutant M1 The lower band should be the correct deletion mutant expression product according to its expected molecular size The identity of the upper band remains to be determined

Localizations of M2 (amino acids 131–264) and M3 (amino acids 131–333) mutants to the nucleolus are in accordance with the finding that both NLS and NoLS are within amino acids 131–264 Mutant M1 (amino acids 131–196) is dispersed within the whole cell but with stronger signal intensity in the cytoplasm than in the nucleus, suggesting that both the NLS and NoLS should be in the region of amino acids 196–264 Because both M4 (amino acids 204–264) and M5 (amino acids 204–333) mutants localize to the nucleo-plasm but not to the nucleolus, it would follow that the major NoLS for human RPL4 is within amino acids 196–204 For mutant M4, the fluorescent signal

is mainly in the nucleoplasm, however, a significant proportion was also found in the cytoplasm The locali-zations of M5 and M6 mutants, consistent with that of C2 mutant, are predominantly in the nucleus excluding the nucleolar region (Fig 4E) Thus, we suspect that

a strong NLS is within amino acids 264–333 while a

IPTG

si935

47S/45S

32S

28S

28S

18S

18S

+ –

– +

+ +

Fig 3 The DEVD motif of Gua is important to both 18S and 28S

rRNA production LAP3 cells were induced with 2 m M IPTG to

express DEVD mutant of human Gua Cells were then treated with

si935 for 48 h followed by pulse-labeling with [ 32 P]orthophosphate

for 1.5 h and a chase for 3 h with normal growth medium Total

RNAs were extracted, resolved on a 1.2% agarose-formaldehyde

gel and blotted onto a membrane for phosphorimager analysis.

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weak NLS may be within amino acids 196–264

Com-bined with the NoLS, this weak NLS is capable to

cause most RPL4 molecules to enter the nucleolus It

is not uncommon to have more than one nuclear

local-ization signal within a protein [35,36]

Downregulation of RPL4 inhibits rRNA production

in mouse LAP3 cell line

RPL4 is a component of the 60S ribosome large

sub-unit To date, there is no report showing a direct

involvement of RPL4 in pre-rRNA processing

Ribo-somal proteins are always produced in the cytoplasm,

and then imported into the nucleoli to participate in

preribosome assembly The ribosomes are then

expor-ted back into the cytoplasm where they direct protein

production [37] As we hypothesize the interaction between RPL4 and Gua is important to the function of Gua in rRNA production, it will aid to our hypothesis

if we could determine whether downregulation of RPL4 has any effect on this process A sequence near the 3¢ end of mouse RPL4 was used to design a small interfering RNA (si-L4-M1), which targets mouse but not human RPL4 mRNA (Fig 5A) Downregulation effects were examined at both mRNA and protein lev-els, using RT-PCR and western blot analysis, respect-ively Treatment of LAP3 cells with 100 nm si-L4-M1 for 48 h resulted in a decrease of the mouse RPL4 mRNA level by 70% (Fig 5B, lanes 7 and 8) This decrease was dose-dependent When 5 nm or 10 nm si-L4-M1 was used, the mRNA level decreased by about 42% or 55%, respectively (Fig 5B, lanes 3–6)

C

F

E

D A

B

WT 1

1

131

Constructs Guα-binding Localization

264

WT

anti-FLAG

IP: anti-FLAG

WB: anti-FLAG

FLAG-C1 FLAG-N1

FLAG-C2

HA-Guα

FLAG-M3

FLAG-M5 FLAG-M2

FLAG-M4

HA-Guα

WB: anti-HA

WB: anti-FLAG

WB: anti-HA

IP: anti-FLAG

Hoechst

Phase

anti-FLAG

Hoechst

Phase

M1 M2 M3 M4 M5 M6

264

333

428

131 131 131

196 264 333 264 204 204 264

– – + – + +

Nucleoplasm and cytoplasm Nucleoli Nucleoli

Nucleoplasm and cytoplasm Nucleoplasm

Nucleoplasm 333

333

428 428

+ – + +

Nucleoli Nucleoli Nucleoli Nucleoplasm

N1

C1

C2

M1 M2 M3

M6 M5 M4

Constructs Guα-binding Localization

Fig 4 Mapping of Gua-binding domain in human RPL4 (A) Schematic representation of wild-type and mutant forms of human RPL4 The open bar represents regions conserved between human and mouse RPL4 The shaded bar represents nonconserved regions (B) Cellular localization of human RPL4 mutants HeLa cells transfected with FLAG-tagged human RPL4 and various mutants were stained by indirect immunofluorescence using anti-FLAG Ig Anti-mouse IgG coupled to FITC was used as secondary antibody Nuclei were visualized by Hoe-chst stain The phase images show dark phase nucleoli (C) Whole cell extracts from HeLa cells cotransfected with HA-tagged human Gua and FLAG-tagged human RPL4 deletion mutants were immunoprecipitated using anti-FLAG resin and blotted as indicated (D) Schematic rep-resentations of human RPL4 mutants M1 to M6 and their (E) cellular localization (F) Whole cell extracts from HeLa cells cotransfected with HA-tagged human Gua and FLAG-tagged human RPL4 deletion mutant shown in (D) were immunoprecipitated using anti-FLAG resin and blotted as indicated.

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Lacking an antibody against mouse RPL4 protein,

we instead used an indirect way to determine the effect

of si-L4-M1 on the protein level of mouse RPL4 We

cotransfected HeLa cells with si-L4-M1 and an

Xpress-tagged mouse RPL4 construct In this

experi-ment, the remaining exogenously expressed mouse

RPL4 protein level after si-L4-M1 treatment could be

measured with anti-Xpress Ig Figure 5C shows that

mouse RPL4 mRNA level was decreased by 83% after

treatment of the cell with 100 nm si-L4-M1 for 48 h

while the exogenously expressed mouse RPL4 protein

level was downregulated by 72% The siRNA did not

have significant influence on mRNA levels of human

RPL4 and U1C, and the protein levels of human

RPL4 and B23

We hypothesized that downregulation of RPL4

would lead to an aberrant rRNA processing pattern if

Gua–RPL4 interaction is necessary for the function of

Gua in rRNA production After 48 h of si-L4-M1

treatment, LAP3 cells were pulse-labeled with [32

P]-orthophosphate and chased with growth medium for

3 h As a negative control, a nonrelated siRNA

(si934Scr) was included [17] Total RNA was extracted,

resolved on a 1.2% agarose-formaldehyde gel and

transferred to a hybond-N nitrocellulose filter for

phos-phorimager analysis We found that all four main

visible species of rRNA (47⁄ 45S, 32S, 28S and 18S) were dramatically decreased in samples treated with si-L4-M1 (Fig 5D) However, the decreases in 47⁄ 45S rRNAs were not as great as those in mature 28S rRNA (Fig 5D), indicating that only part of the decrease in 28S was due to less precursors while the remaining changes resulted from the influence by downregulation

of RPL4 on other pathways involved in rRNA produc-tion Ethidium bromide-stained gel (Fig 5D, bottom)

is shown to indicate equal loading of the RNA

Wild-type RPL4 but not its mutant form which lacks the Gua-interacting domain reverses inhibition of rRNA production

We constructed a deletion mutant of human RPL4, D264-333, which lacks the Gua-interacting domain amino acids 264–333 and another mutant D204-264 as

a control (Fig 6A) We had already localized the NoLS of RPL4 to amino acids 196–204, which was supported by the nucleolar localization of both mutants (Fig 6B) The immunoprecipitation experi-ment shows their Gua-binding activity (Fig 6C), and supports our earlier findings (Fig 4F) Because the region including amino acids 264–333 seems to be the RPL4-Gua-interacting domain, the D204-264 mutant,

A

D

Human 1120

Mouse 1129

mRPL4

mU1C

si-L4-M1

47S/45S

32S 28S

18S

18S 28S

RT-PCR Western

hU1C hRPL4

hRPL4

hB23

Fig 5 Downregulation of mouse RPL4 using si-L4-M1 resulted in aberrant rRNA processing (A) Comparison of human and mouse RPL4 partial cDNA sequences containing the si-L4-M1 region Underscored nucleotides differ from human to mouse (B) siRNA-mediated down-regulation of mouse RPL4 mRNA LAP3 cells were transfected with increasing concentrations of si-L4-M1 Total RNA was isolated after

48 h and analyzed by RT-PCR to determine the mRNA levels of mouse RPL4 and mouse U1C (C) siRNA-mediated downregulation of mouse RPL4 protein HeLa cells were cotransfected with Xpress-tagged mouse RPL4 and si-L4-M1 Total RNA was isolated after 72 h using TRIzol Reagent (Invitrogen) and analyzed by RT-PCR to determine the mRNA levels of mouse RPL4, human RPL4 and human U1C (left panel) A parallel experiment was done to analyze changes in the protein levels of mouse RPL4, human RPL4 and human B23 after si-L4-M1 treat-ment (D) LAP3 cells were treated with 100 n M si-L4-M1 for 48 h Total 32 P-labeled RNA was analyzed as described in the legend to Fig 3 Ethidium bromide staining of both 18S and 28S rRNA is shown at the bottom.

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but not the D264-333 mutant form,

coimmunoprecipi-tated with Gua (Fig 6C)

To determine the significance of Gua–RPL4

inter-action in rRNA biogenesis, we first used si-L4-M1 to

downregulate endogenous mouse RPL4 expression and

then exogenously expressed the human orthologue and

looked for reversal of inhibition of rRNA production

Figure 6(D) (lanes 1 and 2) shows that si-L4-M1

effectively inhibited production of all four species of

rRNAs, which is consistent with the results in

Fig 5(D) This effect was reversed by the exogenous

expression of human wild-type RPL4, suggesting that

the human orthologue can functionally replace mouse

RPL4 (Fig 6D, lane 3) However, the expression of

mutant human RPL4 lacking the Gua-interacting

domain could not reverse the aberrant rRNA

process-ing pattern as effectively as the wild-type while the

mutant lacking amino acids 204–264 had a similar

effect to that of the wild-type, indicating that the

Gua–RPL4 interaction is important to the function of

Gua in rRNA processing (Fig 6D, lanes 4 and 5)

Human RPL4 associates with 28S but not 18S

rRNA

To determine if RPL4 associates with 18S or 28S

rRNA, we performed RNA immunoprecipitation using

HeLa cells transiently transfected with either FLAG vector or FLAG-tagged human RPL4 RNA–RPL4 complexes in the nucleolar extracts were immunopre-cipitated with anti-FLAG resin, and the RNA compo-nents were resolved on a 1.2% agarose-formaldehyde gel, blotted onto a nitrocellulose membrane and sub-jected to northern blot analysis Figure 7 shows that

A

D

∆204-264

∆204-264

anti-FLAG

Hoechst

Phase

IP: anti-FLAG

WB: anti-FLAG

WB: anti-FLAG

1 st transfection si934Scr

FLAG FLAG FLAG-RPL4

(WT) FLAG-RPL4 (∆204-264) FLAG-RPL4(∆264-333) si-L4-M1 si-L4-M1 si-L4-M1 si-L4-M1

2 nd transfection

18S 28S 18S 28S 32S 47S/45S

54 ±5 84±1084 ±10 73±683 ±5 42±747 ±6

100 Total±SE

WB: anti-HA

204 264

428

428

333

264 333

∆264-333

∆264-333

∆204-264 ∆264-333

FLAG-∆204-264

FLAG-RPL4 (WT) FLAG-RPL4 (∆204-264) FLAG-RPL4 (∆264-333)

FLAG-∆264-333 HA-Guα

1

1

Fig 6 Reversal of inhibition of rRNA production (A) Schematic representation of human RPL4 deletion mutants D204-264 and D264-333 The open bar represents regions conserved between human and mouse RPL4 The shaded bar represents nonconserved regions (B) Indi-rect immunofluorescence showing both D204-264 and D264-333 are localized to nucleoli (C) Whole cell extracts from HeLa cells

cotransfect-ed with HA-taggcotransfect-ed human Gua and FLAG-taggcotransfect-ed human RPL4 deletion mutant were immunoprecipitatcotransfect-ed using anti-FLAG resin and blottcotransfect-ed

as indicated (D) LAP3 cells were transfected with either 100 n M si934Scr or 100 n M si-L4-M1 as indicated After 48 h, cells were next trans-fected with FLAG-vector, FLAG-tagged wild-type human RPL4 or FLAG-tagged human RPL4 mutants as indicated After an additional 48 h, cells were pulse-labeled with [ 32 P]orthophosphate for 1.5 h and chased with cold medium for 3 h Total RNA was extracted and analyzed as described in Fig 3 Ethidium bromide staining for 18S and 28S rRNAs is shown in the middle panel The lowest panel shows expression of the FLAG-tagged human wild-type RPL4 (WT) and its mutant forms (D204-264 and D264-333) by western blot analysis using anti-FLAG Ig The numbers below the upper panel correspond to the amount of 28S rRNA or total rRNA ± standard error relative to samples in lane 1 (set

at 100) calculated with IMAGE - QUANT software Results were average of three independent experiments ± SE.

28S probe

28S rRNA

18S rRNA 18S probe

Fig 7 Human RPL4 associates with 28S but not 18S rRNA HeLa cells were transfected with either vector only or FLAG-tagged human RPL4 After 48 h, cells were collected and RNA-RPL4 complexes were immunoprecipitated from nucleolar extracts using anti-FLAG resin as described under Experimental procedures RNA components were isolated and resolved in a 1.2% agarose-formaldehyde gel and blotted onto a nitrocellulose membrane, which was subjected to northern blot analysis as described under Experimental procedures.

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overexpressed RPL4 pulled-down 28S but not 18S

rRNA in a dose-dependent manner (lanes 5 and 6)

We did not observe any signal from 47⁄ 45S, 36S and

32S pre-rRNAs, the precursors of 28S rRNA Based

on our previous experience [16], a single

oligodeoxy-nucleotide probe we used to detect 28S could not

detect higher molecular weight pre-rRNAs under our

hybridization conditions for unknown reasons As we

used nucleolar extract as the starting material for

the immunoprecipitation experiment, the 28S rRNA

pulled down by RPL4 should be newly produced

Discussion

RNA helicase II⁄ Gua is the first nucleolar RNA

heli-case shown to be directly involved in rRNA processing

in both the metazoan and mammalian systems [16,17]

There are several other nonhelicase nucleolar proteins

which have been demonstrated to also function in

rRNA processing in higher eukaryotes including

B23⁄ nucleophosmin, C23 ⁄ nucleolin, Bop1, p120 and

p19Arf Each of these proteins was found to function

at least partially through RNA–protein or protein–

protein interactions [12,14,38–42] Among the 18 RNA

helicases which have been directly implicated in yeast

ribosome biogenesis, at least nine were demonstrated

to functionally interact with other protein factors

[25,37,43] Based on the bona fide helicase activity of

Gua and its demonstrated role in rRNA processing, it

is conceivable that Gua will be shown to have partners

that facilitate its function in the ribosome biogenesis

pathway

In this paper, we report the identification of

ribo-somal protein L4 as a Gua-interacting partner through

immunoprecipitation in mouse LAP3 cells (Fig 1A)

We noticed that several other fast migrating bands

were also pulled down by anti-FLAG resin (Fig 1A,

other proteins), which we suspect to be proteins

associ-ated with either Gua or RPL4 The high concentration

of RNase A (200 lgÆmL)1), which was used in

previ-ous reports to isolate specific target-associated proteins

[31,44], suggests that these additional interactions

might not be RNA-mediated The Gua–RPL4

inter-action was further confirmed by immunoprecipitation

from HeLa cells (Fig 1D,E), yeast two-hybrid analysis

(Fig 1B) and in vitro binding assay (Fig 1C) It is

noteworthy that Gub also interacts with RPL4 as

shown by the two-hybrid analysis (Fig 1B, lower

right) As a paralogue of Gua, Gub also possesses

in vitro ATPase and helicase activities, but no RNA

foldase activity [45] The current data suggest that

both paralogues arose through gene duplication but

the resulting genes are differentially regulated and

might possess different functions [46] Overexpression

of Gub in mouse LAP3 cells leads to inhibition of total rRNA production, suggesting contrasting roles for Gub and Gua [17] It would be valuable to deter-mine whether the inhibitory effect of Gub on rRNA biogenesis is through its competitive interaction with RPL4 Indirect immunofluorescence showed a predom-inant localization of newly produced FLAG-tagged RPL4 protein to the nucleolus (Fig 1F) which is con-sistent with the published report that most newly formed ribosomal proteins are highly concentrated in the nucleolus [47] Burial of FLAG epitope within the highly structured mature ribosome subunit might account for the absence of strong fluorescent signal in the cytoplasm The distribution of RPL4 in the nucleo-lus seems more localized compared with the more dis-persed localization of Gua throughout the entire nucleolus (Fig 1F) This subtle discrepancy between the localizations of the two proteins may indicate that Gua interacts with other partners in different sub-nucleolar regions, which is consistent with the presence

of other additional bands shown in Fig 1A Moreover, our previous immunoelectron microscopy experiments showed that rat Gua is localized to the dense fibrillar component (DFC) and granular component (GC) within the nucleolus [16]

An interesting finding was the importance of the DEVD motif in Gua–RPL4 interaction (Fig 2) We previously showed that in Xenopus, the DEVD motif

of Gua was important for both 18S and 28S rRNA production [16] However, in mammalian cells, we were only able to prove that SAT motif is necessary for 18S maturation [17] As the unwinding activity is dependent on the ATPase activity, we speculated that the DEVD motif of Gua is also necessary for 18S pro-duction in mammalian cells In this report, we showed the conserved importance of the DEVD motif of Gua

to 28S maturation in mouse LAP3 cells (Fig 3) Because DEVD is important for 28S production as well as Gua–RPL4 interaction, and because RPL4 is a component of the ribosome large subunit which con-tains 28S but not 18S rRNA, it is reasonable to sus-pect that RPL4 might be involved in the function of Gua in 28S rRNA production

Through a series of deletion mutants of RPL4 used

in the immunoprecipitation and indirect immunofluo-rescence experiments, we identified the NLS and NoLS

as well as the Gua-interacting domains in RPL4 (Figs 4 and 5) However, it is worth mentioning that the use of deletion mutants may not accurately reflect the exact functional states of protein inter-actions since the possibility exists that the shortened proteins may be unfolded and thus nonfunctional

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Subtle point mutations in the identified Gua-interacting

domain might lend more support to our conclusions

Downregulation of RPL4 via si-L4-M1 resulted in the

inhibition of production of all four rRNA species

(Figs 5D and 6D lane 2), strongly suggesting a general

mechanism whereby RPL4 modulates rRNA

biogen-esis through rDNA transcription, rRNA turn over,

ribosome production rate, ribosome stability or rRNA

degradation It is possible that the amount of RPL4 in

the cell correlates with the assembly or stabilization of

pre-rRNA processing machineries or preribosomal

par-ticles Perhaps RPL4 is actually a component of the

pre-rRNA processing machinery If so, a dramatic

change in the amount of RPL4 protein level might

lead to disassembly of the specific machineries or

parti-cles, which would send feedback signals to RNA

polymerase I to advance more slowly or even to

rRNA-degrading complexes to degrade the unincorporated

mature rRNA [17] This hypothesis might help to

interpret the involvement of other nucleolar proteins in

pre-rRNA production such as p19Arf[15] It might also

explain why there is a decrease of 18S rRNA

More-over, inhibition of the 28S pathway might

concomit-antly result in the reduction of 18S rRNA through an

unidentified mechanism For example, many yeast

mutants with 25S rRNA production defects also show

an inhibition in 35S pre-rRNA cleavages which lead to

decrease in 18S biogenesis [48]

Other than the proposed general function of RPL4

in overall rRNA production, we also hypothesize that

RPL4 plays a direct role in 28S production through its

interaction with Gua Several arguments and lines of

evidence support this: (a) our rescue experiment

showed that wild-type human RPL4 reversed the

aber-rant rRNA processing pattern (Fig 6D, compare lanes

2 and 3) but the mutant lacking the Gua-interacting

domain had no effect (Fig 6, compare lanes 2 and 5);

(b) inhibition of 28S rRNA production is more

signifi-cant than that of 47⁄ 45S when RPL4 is downregulated

(Fig 5D, lanes 1 and 2); (c) RPL4 is an important

component of the 60S ribosome subunit which

con-tains the 28S but not the 18S rRNA; (d) RNA

immu-noprecipitation revealed coprecipitation of RPL4 with

28S but not with 18S rRNA (Fig 7); (e) ATPase

activ-ity of Gua is important for both 28S production and

Gua–RPL4 interaction These five lines of evidence

support a more direct role for the Gua–RPL4

inter-action in 28S production than a possible general

mechan-ism, although it is likely that both mechanisms coexist

It is not uncommon for a nucleolar protein to function

in different pathways For example, the function of

C23 in ribosome biogenesis is reflected in almost all

steps of the process including rDNA transcription,

pre-rRNA processing, preribosome assembly and nucleocytoplasmic transport [39]

What then could be a mechanism whereby the Gua– RPL4 interaction facilitates 28S rRNA biogenesis? The fact that Gua and RPL4 have been identified in ribo-nucleoprotein (RNP) particles [31,49] indicates that their interaction might cause them to be localized into pre-rRNA processing machineries essential for pre60S ribosome particles It is known that interruption of early assembly steps results in disassembly of the parti-cles and destabilization of pre-rRNAs [43] Moreover,

we did observe several other bands in the immunopre-cipitation assay (Fig 1A) coimmunoprecipitating with Gua, which may represent other proteins in the same processing machinery as Gua Once Gua has been incorporated into the RNP particle, it might function

in early rRNA processing steps such as regulating interactions between guide snoRNAs and pre-rRNAs, helping the endo- and exo-nucleases in removing inter-nal or exterinter-nal transcribed spacer sequences as well as modulating the numerous trans-acting factors and ribosomal proteins in the pre60S particles through regulation of RNA-RNA, RNA–protein and protein– protein interactions [43] In yeast S cerevisiae, involve-ment of ATP-dependent RNA helicase has been implicated in each of these possible roles [50–54] The functional diversity of an RNA molecule is based on its extreme flexibility With the help of proteins, RNA retains or loses its active configuration in response to various different cellular signals [55] RNA helicase is

a candidate to function in an energy-dependent manner in this process In addition, Gua has another activity, GTP-stimulated RNA folding activity which resides within a domain separate from the ATPase⁄ helicase activity Proteins utilizing GTP as an energy source have recently been found to participate in ribo-some biogenesis [56,57] In addition, it was recently reported that GTP-binding state might influence the nucleolar targeting of nucleostamin, a nucleolar pro-tein which shuttles between nucleoplasm and nucleolus with suspected roles in cell cycle and cell proliferation regulation [58–60] Interestingly, the C-terminal FRGQR-containing region of Gua has also been reported to be critical for both GTP-stimulated RNA foldase activity and nucleolar localization [20,61] It remains to be identified if the FRGQR region of Gua

is relevant to GTP–binding or RPL4 interaction It would not be surprising if the Gua–RPL4 interaction

is found to be important in all three roles mentioned considering the complexity of ribosome assembly, which highly demands versatile energy producers and consumers The multifunctional property of Gua makes it a good candidate

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