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We have performed a screen for ITP-binding proteins because ITP is a deaminated product of ATP, the most abundant nucleotide, and identified RS21-C6 protein, which bound not only ITP but

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5¢-triphosphate pyrophosphohydrolase that prefers

5-iodocytosine

Mari Nonaka, Daisuke Tsuchimoto, Kunihiko Sakumi and Yusaku Nakabeppu

Division of Neurofunctional Genomics, Department of Immunobiology and Neuroscience, Medical Institute of Bioregulation, Kyushu University, Fukuoka, Japan

In living organisms, nucleotides play various roles, as

signal transmitters, molecular switches, coenzymes or

as carriers of energy, in addition to their important

role as precursors of DNA⁄ RNA synthesis For

exam-ple, ATP is a major carrier of energy, a phosphate

group donor in kinase reactions, and an extracellular

signal transmitter GTP is a molecular switch in signal

transduction pathways and an initiator complex for

translation UTP and CTP are utilized to form active

intermediates in the biosynthesis of polysaccharides or phospholipids, respectively In such roles, recognition

of nucleotides by specific proteins is very important Intracellular nucleotides, however, undergo chemical modifications caused by endogenous reactive mole-cules, such as reactive oxygen species, or by exogenous factors, such as chemicals and ionizing irradiation Chemical modification may alter the characteristics of nucleotides, including their recognition by proteins

Keywords

5-I-dCTP; CpG methylation; dCTPase;

modified nucleotide; nucleotide metabolism

Correspondence

D Tsuchimoto, Division of Neurofunctional

Genomics, Department of Immunobiology

and Neuroscience, Medical Institute of

Bioregulation, Kyushu University, Maidashi

3-1-1, Higashi-ku, Fukuoka 812-8582, Japan

Fax: +81 92 642 6804

Tel: +81 92 642 6802

E-mail: daisuke@bioreg.kyushu-u.ac.jp

(Received 2 September 2008, revised

8 January 2009, accepted 12 January 2009)

doi:10.1111/j.1742-4658.2009.06898.x

Free nucleotides in living cells play important roles in a variety of biolo-gical reactions, and often undergo chemical modifications of their base moieties As modified nucleotides may have deleterious effects on cells, they must be eliminated from intracellular nucleotide pools We have performed

a screen for ITP-binding proteins because ITP is a deaminated product of ATP, the most abundant nucleotide, and identified RS21-C6 protein, which bound not only ITP but also ATP Purified, recombinant RS21-C6 hydro-lyzed several canonical nucleoside triphosphates to the corresponding nucleoside monophosphates The pyrophosphohydrolase activity of RS21-C6 showed a preference for deoxynucleoside triphosphates and cytosine bases The kcat⁄ Km (s)1Æm)1) values were 3.11· 104, 4.49· 103 and 1.87· 103for dCTP, dATP and dTTP, respectively, and RS21-C6 did not hydrolyze dGTP Of the base-modified nucleotides analyzed, 5-I-dCTP showed an eightfold higher kcat⁄ Kmvalue compared with that of its corre-sponding unmodified nucleotide, dCTP RS21-C6 is expressed in both pro-liferating and non-propro-liferating cells, and is localized to the cytoplasm These results show that RS21-C6 produces dCMP, an upstream precursor for the de novo synthesis of dTTP, by hydrolyzing canonical dCTP More-over, RS21-C6 may also prevent inappropriate DNA methylation, DNA replication blocking or mutagenesis by hydrolyzing modified dCTP

Abbreviations

2-Cl-dATP, 2-chloro-(2¢-deoxy)adenosine 5¢-triphosphate; 2-OH-(d)ATP, 2-hydroxy-(2¢-deoxy)adenosine 5¢-triphosphate; 5-Br-dCTP, 5-bromo-2¢-deoxycytidine 5¢-triphosphate; 5-F-dUTP, 5-fluoro-2¢-5-bromo-2¢-deoxycytidine 5¢-triphosphate; 5-I-dCTP, 5-iodo-2¢-5-bromo-2¢-deoxycytidine 5¢-triphosphate;

5-Me-dCTP, 5-methyl-2¢-deoxycytidine 5¢-triphosphate; 5-OH-dCTP, 5-hydroxy-2¢-deoxycytidine 5¢-triphosphate; 8-oxo-(d)GTP, 8-oxo-(2¢-deoxy)guanosine 5¢-triphosphate; DCTD, dCMP deaminase; GAPDH, glyceraldehyde-3-phosphate dehydrogenase; NTP, nucleoside

5¢-triphosphate; RNR, ribonucleotide reductase; TS, thymidylate synthase.

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Some modified deoxynucleotides are incorporated into

DNA by DNA polymerases and accumulate in newly

synthesized DNA This may prevent DNA replication

or transcription, resulting in cell death and

degenera-tive diseases in humans [1] Normal functions of

nucle-otides, other than DNA synthesis, may also be

adversely affected by modified nucleotides Cells are

equipped with defense systems against such modified

nucleotides Some modified nucleotides in intracellular

nucleotide pools are hydrolyzed by specific enzymes

[1,2] Of these enzymes, dUTPase and MTH1 are the

best studied in human cells The former hydrolyzes

deoxyuridine triphosphate to prevent its incorporation

into DNA The latter hydrolyzes oxidized purine

nucleoside triphosphates, including

8-oxo-(deoxy)gua-nosine triphosphate [8-oxo-(d)GTP] and

2-hydroxy-(deoxy)adenosine triphosphate [2-OH-(d)ATP], to the

corresponding (deoxy)nucleoside monophosphates and

pyrophosphates to avoid their incorporation into

DNA or RNA [3] The spontaneous mutation rate in

MTH1-null mouse embryonic stem cells was twofold

higher than that in wild-type cells Further,

MTH1-null mice showed more frequent tumorigenesis in the

liver compared to wild-type mice [4]

In addition to oxidization, deamination of the amino

group is another major chemical modification that

occurs in purine nucleotides Deamination of the

amino group at C6 of adenine or C2 of guanine

gener-ates hypoxanthine or xanthine, respectively Thus,

(d)ITP and (d)XTP are generated from (d)ATP and

(d)GTP, respectively Incorporation of these modified

nucleotides into DNA during DNA replication or into

RNA during transcription results in gene mutations or

the synthesis of abnormal proteins because

hypo-xanthine and hypo-xanthine can mis-pair with cytosine or

thymine, respectively Recently, mammalian inosine

triphosphate pyrophosphohydrolases (ITPases) have

been reported to hydrolyze deaminated purine

nucleo-side triphosphates to the corresponding nucleonucleo-side

monophosphates and pyrophosphates [5,6]

ITPase-null mice, in which accumulation of ITP was observed,

showed abnormal development and died within

14 days after birth (M Behmanesh, K Sakumi,

S Toyokuni, S Oka, Y Ohnishi, D Tsuchimoto &

Y Nakabeppu, unpublished results) The ITP that

accumulates in these mice may have deleterious effects

on cell functions, for example via DNA⁄ RNA

synthe-sis, or, because of its structural similarity to ATP, by

interaction with ATP-related proteins

In the present study, we prepared ITP-agarose and

purified ITP-binding proteins to identify additional

ITP-hydrolyzing enzymes or target proteins whose

function can be inhibited by ITP As a result, we

iden-tified RS21-C6, which was previously reported to be a thymocyte development-related molecule [7], as well as ITPase, as ITP-binding proteins Because RS21-C6 contains a typical MazG domain conserved in the bac-terial NTP pyrophosphatase MazG, it has been described as a member of the all-a NTP pyrophospho-hydrolase superfamily with all-a helix structures [8] A preliminary structure of RS21-C6 without substrate has been initially determined [9] Recently, it was shown that RS21-C6 hydrolyzes 5-methyl-dCTP, and the crystal structure of truncated RS21-C6 complexed with 5-methyl-dCTP indicated that tetramer formation

is required for substrate binding [10] We examined the NTP pyrophosphohydrolase activity of purified recom-binant RS21-C6 protein towards various nucleotides, and found that it hydrolyzes some deoxynucleotides, particularly dCTP, but not dITP or ITP Furthermore,

we found that iodination at C5 of cytosine significantly increases the kcat⁄ Kmvalue of RS21-C6

Results

Preparation of ITP-agarose

We prepared ITP-agarose from ATP-agarose as described in Experimental procedures Analysis of bases excised from agarose beads revealed that most adenine bases on the agarose were converted to hypo-xanthine after deamination (Fig 1) We also confirmed that most free ATP was converted to ITP after the same treatment (data not shown), demonstrating that the nucleotides on the treated agarose were ITP Quan-tification of released bases indicated that the amounts

of nucleotide on ATP- and ITP-agarose were 17.3 and 2.6 nmol per 25 lL bed volume, respectively

ITP-binding proteins ITP-binding proteins were purified from mouse thymo-cyte extract by a pulldown method using ITP-agarose Proteins were then fractionated by SDS–PAGE (Fig 2A) After staining the acrylamide gel, we chose ten ITP-specific bands, and, from these, identified 11 proteins by LC-MS⁄ MS analysis (Table 1) We detected ten peptides of ITPase and four peptides of RS21-C6 in bands 4 and 5, respectively Specific binding of ITPase to ITP-agarose was confirmed by western blot analysis of pulldown samples using anti-ITPase serum [5] (Fig 2B, upper panel) To analyze the interaction of RS21-C6 with ITP in detail, RS21-C6 cDNA and recombinant RS21-C6 protein were pre-pared as described in Experimental procedures We performed pulldown experiments, using ITP-agarose

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from cell extracts of Escherichia coli BL21-CodonPlus (DE3)-RIL that had been transformed with pET3a: RS21-C6 and induced for RS21-C6 expression Recom-binant RS21-C6 protein in bacterial cell extracts also bound to both ITP- and ATP-agarose (Fig 2C)

0.00

0.02

0.04

0.06

0

Before deamination

0.00

0.02

0.04

0.06

0

Retention time (min)

*

After deamination

Fig 1 Preparation of ITP-agarose ATP-agarose were incubated in

1 M HCl before (upper graph) or after (lower graph) deamination,

and the bases released were analyzed by HPLC The peaks

indi-cated by an open arrowhead and a closed arrowhead were

com-pared with peaks of standard samples, and were identified as

adenine base and hypoxanthine base, respectively The peak

indi-cated by an asterisk was also observed in a sample released from

agarose without any nucleotide, suggesting that it was derived

from the carrier agarose (data not shown).

25 7

17 4

(kDa )

ITPase 25.7

ITP-agarose ATP-agarose Agarose

100

75

37

25

20

15

50 (kDa)

Recombinant RS21-C6

20

15

25 (kDa) ATP-agarose ITP-agarose Agarose

Recombinant RS21-C6

1

2

3

4

6

7

8

9

10

(kDa)

25.7

17.4

31.6 47.3

84.7

114

D

B

C

A

Fig 2 Purification of ITP-binding proteins (A) Pulldown of ITP-specific proteins from mouse thymocyte extract Proteins were pulled down using ATP- or ITP-agarose and then separated by SDS–PAGE The lanes were loaded with samples pulled down from 5.0 · 10 7 cells The gel was subjected to silver staining Arrowheads indicate the numbered ITP-specific bands that were recovered and subjected to a mass spectrometry (B) Western blot of pulled down samples with anti-ITPase serum and anti-RS21-C6 Ig Samples pulled down from 2.5 · 10 7

cells were loaded on the gel as described in (A) (C) Pulldown of recombinant RS21-C6 Recombinant RS21-C6 protein was expressed in

E coli Binding proteins were pulled down using agarose beads, separated by SDS–PAGE and stained by silver staining (D) Purification of recombinant RS21-C6 protein Recombinant RS21-C6 protein, expressed in E coli, was purified as described in Experimental procedures The purified protein (100 ng) was separated by SDS–PAGE and stained by silver staining.

Table 1 ITP-binding proteins identified by LC-MS ⁄ MS analysis Band

no Protein description

NCBInr accession no.

1 Acetyl coenzyme A acetyltransferase

1 precursor

gi|21450129

2 Glyceraldehyde-3-phosphate

dehydrogenase

gi|50233866

Similar to ISOC2 protein gi|20818892

Phospholipid hydroperoxide glutatione peroxidase

gi|2522259

Divalent cation tolerant protein CUTA isoform 1

gi|62198210

7 Unidentified

8 Isochorismatase domain-containing 1 gi|31541909

10 Unidentified

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TrxA-RS21-C6 protein was expressed from

pET32a(+):RS21-C6 and was used as an antigen to

prepare anti-RS21-C6 rabbit serum Western blot

anal-ysis, using affinity-purified anti-RS21-C6 Ig, showed

that endogenous RS21-C6 also binds to both ITP- and

ATP-agarose (Fig 2B, lower panel)

Glyceraldehyde-3-phosphate dehydrogenase (GAPDH), detected in

band 2, bound to the negative control deaminated

agarose, as well as to ITP-agarose, as shown by western

blot analysis with anti-GAPDH Ig, suggesting that the

binding of GAPDH was not nucleotide-specific (data

not shown)

The non-tagged recombinant RS21-C6 protein was

purified to a nearly homogeneous state by SDS–

PAGE to analyze its enzyme activity (Fig 2D) Its

molecular mass was estimated, based on its SDS–

PAGE mobility, as approximately 19 kDa, which is

almost identical to the calculated molecular weight of

18 783

Expression of endogenous RS21-C6 Western blot analysis using anti-RS21-C6 Ig and whole-cell extracts from several mouse cell lines pro-duced an intense band corresponding to a polypeptide with a molecular mass of about 19 kDa in each lane (Fig 3A), although additional, non-specific bands were detected in some lanes We then transfected the mouse

B cell lymphoma line A20 individually with two plas-mids to express non-tagged recombinant RS21-C6, and independently transfected vector controls without inserts After incubation for 24 h, whole-cell extracts were prepared and subjected to western blot analysis using anti-RS21-C6 We detected a band with a very intense signal that corresponded to a size of approxi-mately 19 kDa in each of the samples overexpressing RS21-C6 We detected a band with identical mobility but a weak signal in each of the vector control sam-ples, indicating that anti-RS21-C6 specifically reacts

25.7

17.4

(kDa) 20 pcDNA pcDNA :RS21-C6 pIRES2-EGFP pIRES2-EGFP :RS21-C6

15

(kDa)

RS21-C6

GAPDH

RS21-C6

GAPDH

31.3 47.3

84.7

114

W N Mt Cy t

RS21-C6 Lamin B HSP60

GAPDH

0

5

10

15

20

Cerebrum Cerebellum

Eye Spinal cord

Heart Kidney Liver Lung

Esophagus Lymph node Spleen Bone marrow

Thymus Testis Uterus

Stomach Salivary gland Ovary

Skeletal muscle Thyroid gland

C

D

Fig 3 Expression of endogenous RS21-C6.

(A) Western blot analysis of RS21-C6

pro-tein in various mouse cell lines A whole-cell

extract from 1.0 · 10 5 cells of each cell line

was loaded in each lane Proteins were

sep-arated by SDS–PAGE, and then transferred

to a poly(vinylidene difluoride) membrane.

Signals for RS21-C6 protein and of GAPDH

were detected using RS21-C6 and

anti-GAPDH Ig, respectively (B) Western blot

analysis of endogenous or recombinant

RS21-C6 proteins in A20 cells A20 cells

were transfected with plasmids expressing

recombinant RS21-C6 or with control

vec-tors by electroporation The cells were

incu-bated for 24 h, and whole-cell extracts from

1.0 · 10 5 cells were loaded on each lane.

(C) Intracellular localization of RS21-C6

pro-tein Aliquots (20 lg protein) from whole-cell

extract (W) or each cell fraction were loaded

into each lane Lamin B, HSP60 or GAPDH

were detected as nuclear (N), mitochondrial

(Mt) or cytoplasmic (Cyt) markers,

respec-tively (D) Real-time quantitative PCR

analy-sis of RS21-C6 expression in various mouse

tissues The mRNA expression levels of

RS21-C6 were normalized to those of 18S

rRNA Error bars represent SD (n = 3) The

expression level of RS21-C6 in spleen was

arbitrarily set as 1.0, and the expression

levels in the other tissues are expressed

relative to that in spleen.

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with both recombinant RS21-C6 and mouse RS21-C6 that is endogenous to A20 cells (Fig 3B) We then analyzed the intracellular localization of the RS21-C6 protein Nuclear, mitochondrial and cytosolic fractions were prepared from mouse liver Western blot analysis

of each fraction revealed that RS21-C6 is exclusively located in the cytosol (Fig 3C) Finally, we examined the expression levels of RS21-C6 mRNA by real-time quantitative PCR, and found that RS21-C6 is ubiqui-tously expressed and that expression was highest in the liver and heart, and to a lesser extent the salivary gland (Fig 3D)

Nucleoside triphosphate pyrophosphohydrolase activity of RS21-C6 protein

In a preliminary analysis, using canonical nucleotides, purified RS21-C6 protein showed strong pyrophospho-hydrolase activity on dCTP, producing dCMP (Fig 4A, middle panel) We also analyzed the reaction product using BIOMOL GREEN reagent (Enzo Bio-chem, Inc., New York, NY, USA) and detected no free phosphate, indicating that RS21-C6 hydrolyzes dCTP

to dCMP and pyrophosphate (data not shown) Next,

we analyzed the optimal conditions for the pyrophos-phohydrolase activity of RS21-C6 using dCTP as a substrate RS21-C6 showed a temperature-dependent increase of activity up to 60C (Fig 4B) RS21-C6 demonstrated strongest activity at pH 9.5, the highest

pH analyzed here (Fig 4C) The divalent metal cation requirements of RS21-C6 were tested using MgCl2and MnCl2 No activity was detected in reactions without added metals, and maximum activity was measured in reactions containing 100 mm MgCl2 At 100 mm, MnCl2did not support full activity (Fig 4D) RS21-C6 showed the same activity with various concentrations

of KCl between 0 and 1000 mm (Fig 4E) NaCl mod-erately reduced RS21-C6 activity Based on these results and in view of physiological conditions, we

0.08

0.04

0.00

0.08

0.04

0.00

0.08

0.04

0.00

Retention time (min)

dCTP + buffer

dCTP + RS21-C6

dCMP standard

0

20

40

60

80

100

Temperature (°C)

0

20

40

60

80

100

pH

PIPES-Na

Tris-HCl

AMPD-HCl

0

20

40

60

80

100

100

Mg 2+

Mn 2+

0

20

40

60

80

100

Salt concentration (m M )

Metal ion concentration (m M )

NaCl KCl

A

B

C

D

E

Fig 4 dCTP pyrophosphohydrolase activity of RS21-C6 protein (A) Hydrolysis of dCTP to dCMP by RS21-C6 Substrate dCTP (300 l M ) was incubated for 20 min in reaction buffer supplemented with

50 n M of purified RS21-C6 protein Reaction products were ana-lyzed by HPLC (middle panel), and were compared with substrate dCTP incubated in the reaction buffer without RS21-C6 (upper panel), and with standard dCMP prepared in the reaction buffer (lower panel) The dependency of RS21-C6 activity on temperature (B), buffer pH (C), divalent metal cations (D) and salts (E) was ana-lyzed The amounts of dCMP produced were measured and are shown as percentages of the highest value Each point and error bar indicates the mean and standard deviation of three reactions.

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performed further analysis of RS21-C6 activity under

the conditions described in Experimental procedures

The substrate concentration was set at 10, 30, 100 or

1000 lm, except for that of 5-I-dCTP, which was set at

2, 3.9, 7.8, 15.6, 31.3, 62.5, 125, 250, 500 or 1000 lm, because the Km value for 5-I-dCTP is < 10 lm The

Km and kcat values at 50 nm RS21-C6 for various nucleotides were determined from Lineweaver–Burk plots, and are shown in Table 2 Except for CTP, RS21-C6 did not hydrolyze the analyzed ribo-nucleotides, even ITP For the analyzed deoxynu-cleotides, the kcat⁄ Km values for dCTP, dATP and dTTP were 3.11· 104, 4.49· 103 and 1.87· 103, and RS21-C6 did not hydrolyze dGTP and dITP Thus, RS21-C6 shows a preference for cytosine base and deoxyribose Among the base-modified nucleo-tides, 5-I-dCTP had an eightfold higher kcat⁄ Km value compared to its corresponding unmodified deoxy-nucleotide, dCTP We analyzed the hydrolysis of dCTP by RS21-C6 in the presence of ITP, and found that ITP did not inhibit it even at 500 lm (data not shown)

Enzyme reaction curves, a Michaelis–Menten plot and a Lineweaver–Burk plot for 5-I-dCTP are shown

in Fig 5 The oligomeric structure of RS21-C6, together with the conformational flexibility of its active sites, suggests cooperativity between the sites and allo-steric regulation However, we obtained a typical Michaelis–Menten-type saturation curve rather than the sigmoid curve typical of non-Michaelis–Menten-type reactions This result indicates that the RS21-C6 tetramer does not show any cooporative binding of substrates to the multiple active sites under the present conditions

Table 2 Kinetic parameters for nucleoside triphosphate

pyrophos-phohydrolase activity of RS21-C6 protein.

Substrate K m (l M ) k cat (s)1) k cat ⁄ K m (s)1Æ M )1)

2-OH-ATP No activity

8-oxo-dGTP No activity

0 10 20 30 40

50

60 70 [Substrate] (µ M )

0 1 2 3

–1 )

2 µ M

3.8 µ M

7.8 µ M

15.6 µ M

31.3 µ M

62.5 µ M

125 µ M

250 µ M

500 µ M

1000 µ M

0 5 10

30

0

60

A

B

[Substrate] –1 (µ M–1)

0.4 0.2

50

25

C

Fig 5 Kinetic analysis of the reaction of

RS21-C6 with 5-I-dCTP The concentration

of 5-I-dCTP substrate was set at 2, 3.9, 7.8,

15.6, 31.3, 62.5, 125, 250, 500 or 1000 l M

(A) Enzyme reaction curves (B) Michaelis–

Menten plot using the results at substrate

concentrations from 0 to 62.5 l M , showing

the saturation pattern (C) A Lineweaver–

Burk plot prepared using all the results.

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We have identified ITPase, a known ITP-hydrolyzing

enzyme, as an ITP-binding protein, using ITP-agarose

This indicates that our screening technology is a valid

approach for identification of novel proteins that

bind various modified nucleotides RS21-C6, another

protein identified in this study, was an ITP-binding

protein which can also bind ATP Because of its

homology in amino acid sequence to known nucleoside

triphosphate (NTP) pyrophosphohydrolases, including

bacterial MazG proteins [8], we analyzed its catalytic

activity for hydrolyzing canonical nucleotides We

showed that RS21-C6 is a deoxynucleoside

triphos-phate pyrophosphohydrolase that prefers dCTP In

mammalian cells, no other protein has been reported

as a pyrimidine dNTP-specific pyrophosphohydrolase,

although Orf135 and iMazG, a novel bacterial MazG

protein, have been reported as bacterial dCTPases

[11,12] The likely biological unit of RS21-C6 is a

tet-ramer [10], suggesting that the multivalency of the

RS21-C6 tetramer may stabilize its interaction with

ITP immobilized on agarose beads This may explain

why RS21-C6, which is not an ITP-specific protein,

has been identified as an ITP-binding protein

It has been shown that the substrate-binding pocket

of RS21-C6 comprises several residues, including

His38, Trp47, Trp73, Tyr102, Glu63, Glu66, Glu95

and Asn98, that the nitrogenous base and deoxyribose

of 5-methyl-dCTP are located in a hydrophobic cavity,

and that the phosphate groups interact with the four

electronegative amino acid residues [10] Among the

known all-a-NTP pyrophosphohydrolases, we found

that residue Asn125 in RS21-C6 is conserved in

vari-ous dUTPases [Campylobacter jejuni dUTPase

(CjdUTPase), Leishmania major dUTPase,

Trypanoso-ma cruzi dUTPase (TcdUTPase)], dCTPases

(entero-bacteria phage T2 dCTPase, entero(entero-bacteria phage T4

dCTPase, bacteriophage RB15 dCTPase), and iMazG

[8,12] Moroz et al and Harkiolaki et al analyzed the

crystal structures of CjdUTPase [13] and TcdUTPase

[14], respectively, with their substrates They showed

that Asn179 of CjdUTPase and Asn201 of

TcdUT-Pase, the residues corresponding to Asn125 of

RS21-C6, bind to the 2¢-deoxyribose moiety of substrates

This residue is not conserved in either the HisE family,

which has phosphoribosyl-ATP pyrophosphatase

activ-ity (E coli HisIE, Corynebacterium glutamicam HisE,

Pyrococcus furiosus HisIE, Saccharomyces cerevisiae

HIS4, Arabidopsis thaliana HisIE), or the MazG family

(SSO12199, E coli MazG, Thermotoga maritime

MazG, Bacillus subtilis YABN, Streptomyces cacaoi

YBL1) Because enzymes in the HisE and MazG

fami-lies but not those of the dUTPase, dCTPase, iMazG and RS21-C6 families hydrolyze ribonucleotides, we suggest that the Asn125 residue in RS21-C6 may be involved in the preference for deoxyribose sugar

Wu et al [10] did not mention an interaction between Asn125 and the substrate 5-methyl-dCTP in their anal-ysis of the crystal structure of RS21-C6 with the sub-strate They used the core fragment of RS21-C6 (RSCUT: residues 21–126) in which the Asn125 resi-due is located very close to the C-terminal end There-fore, Asn125 might not be appropriately located in the truncated molecule

Recognition of the cytosine base by RS21-C6 appears to be supported by the His38 residue in helix 1, which forms a hydrogen bond with the O2 of the cytosine base [10] This corresponds to the gluta-mine residues in the first helix of dUTPases (Gln14 of CjdUTPase or Gln22 of TcdUTPase), which form a hydrogen bond with the O2 of a uracil base [8] It has been shown that His58 of CjdUTPase [13] and Trp61

of TcdUTPase [14] form a hydrogen bond with O4 of the uracil base, and this appears to be involved in their discrimination of uracil from cytosine These residues are not conserved in RS21-C6, supporting its lower affinity for dUTP in comparison to dCTP as revealed

in the present study

RS21-C6 has essentially similar affinities towards both dCTP and 5-methyl-dCTP, indicating that there may not be a specific residue that recognizes the methyl group at the C5 position However, halogena-tion at the C5 posihalogena-tion, particularly iodinahalogena-tion, signifi-cantly increased its affinity to RS21-C6 It is possible that one of the residues that form the substrate-bind-ing pocket may recognize the iodine at the C5 posi-tion An analysis of the crystal structures of RS21-C6 complexed with 5-I-dCTP will help to delineate the structural basis for its substrate recognition

What is the biological role of dCTPase in mamma-lian cells? RS21-C6 hydrolyzes dCTP and produces dCMP In mammalian cells, deamination of dCMP by dCMP deaminase is the most important pathway for dUMP production dUMP is then converted to dTMP

by thymidylate synthase [15–18], and dTMP is con-verted to dTTP by a two-step phosphorylation reac-tion The activity of dCMP deaminase is positively regulated by dCTP, and negatively by dTTP [19] Hence, the conversion of dCTP to dTTP appears to be

a key pathway for regulating the ratio of dCTP⁄ dTTP

in the nucleotide pool Based on pulse-chase experi-ments using [5-3H]cytidine, Bianchi et al [20] estimated that dCTP was incorporated into DNA at a rate of

16 pmolÆmin)1 in rapidly growing mouse 3T6 cells, and that dTMP was formed from free dCMP at a rate

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of 10 pmolÆmin)1 A defect in dCMP deaminase in

hamster fibroblasts was reported to cause an

imbal-anced dCTP⁄ dTTP ratio, and to mildly affect the

fidelity of DNA replication [21] dCTP is not an

effector molecule that allosterically controls

ribonucle-otide reductase (RNR) Therefore, cells must have

other mechanisms to regulate the cytosolic dCTP

concentration In this regard, dCTP

pyrophosphohy-drolase plays an important role in avoiding

accumu-lation of excess dCTP and supplying sufficient levels

of dCMP, an upstream precursor of de novo synthesis

of dTTP

Expression of RS21-C6 mRNA was detected in all

tissues examined in this study, and was particularly high

in the liver and heart These data suggest that RS21-C6

plays a role in both proliferating and non-proliferating

cells Additionally, we found that RS21-C6 was

local-ized to the cytosol In mammalian cells, two types of

RNR, R1⁄ R2 RNR and R1 ⁄ p53R2 RNR, regulate the

synthesis of dNTPs for DNA replication [22,23] In

proliferating cells, the R1⁄ R2 RNR complex, which

consists of R1 and R2 subunits, is localized to the

cyto-plasm and supplies deoxynucleotides for nuclear DNA

synthesis [22] Even in non-proliferating cells, dNTPs

are necessary for mitochondria DNA replication These

cells express the p53R2 subunit instead of the R2

subunit in the cytoplasm Mitochondrial DNA

deple-tion caused by mutadeple-tions in the RRM2B gene, which

encodes the p53R2 subunit, demonstrates that the

R1⁄ p53R2 RNR complex plays a critical role in dNTP

supply for mitochondrial DNA replication [24]

Simi-larly to the RS21-C6 gene, expression of the RRM2B

gene is found in many tissues, in contrast to the R2

subunit, which is undetectable in the heart, brain and

muscle [25] dTMP and thymidine synthesized in the

cytoplasm are imported into the mitochondria,

phos-phorylated by mitochondrial enzymes and used for

mitochondrial DNA replication [26] On the other

hand, it has been reported that dCTP transport activity

exists in human mitochondria [27] These reports raise

the possibility that the cytosolic concentration of dCTP

and dTMP influences the balance of mitochondrial

deoxypyrimidine nucleotide pools (Fig 6A)

Several members of the NTP pyrophosphohydrolase

family have also been shown to eliminate

non-can-onical nucleotides from the intracellular NTP pool

Here, we have shown that RS21-C6 has the highest

kcat⁄ Km value for the modified deoxynucleotide

5-I-dCTP of the various nucleotides examined, including

its corresponding canonical deoxynucleotide, dCTP

Our data indicate that 5-I-dCTP or its analogs might

be true substrates of RS21-C6 It is unlikely that the

intracellular level of ITP prevents RS21-C6 activity,

because 500 lm ITP did not inhibit hydrolysis of dCTP by RS21-C6 in our preliminary experiment 5-I-dCTP is a derivative molecule of dCTP, in which C5 of the cytosine base is iodinated Halogenation of cytosine at C5, including chlorination and bromina-tion, has been shown to occur under physiological con-ditions Such halogenation occurs in the presence of either myeloperoxidase or eosinophil peroxidase, which are produced by phagocytic cells [28,29] In these reac-tions, hypohalous acids, inter-halogen and haloamines are candidate intermediate molecules that can diffuse across plasma membranes into the cytoplasm and halogenate cytosine in cells Kawai et al [30] have pre-viously detected halogenated cytosine in DNA in inflamed tissues Their results indicate in vivo haloge-nation of cytosine, but do not provide direct evidence for intra-cellular halogenation Valinluck et al reported that 5-iodocytosine, 5-bromocytosine and 5-chlorocytosine, at a CpG site of DNA, can mimic 5-methylcytosine and induce inappropriate DNA meth-yltransferase 1-dependent methylation within the CpG sequence [31,32] The induction effect of 5-iodocytosine was the greatest among the 5-halogenated cytosines, and was even better than that of 5-methylcytosine 5-halogenated cytosine, at a CpG site, may enhance the binding of methyl-CpG-binding protein 2 [33] These reports suggest that the 5-halogenated dCTP generated in chronic inflamed tissues might be incorpo-rated into promoter regions of important genes, such

as tumor-suppressor genes, and induce their silencing through inappropriate CpG methylation of the promoter regions or by binding of methyl-CpG-bind-ing protein 2, resultmethyl-CpG-bind-ing in tumorigenesis RS21-C6 may

Fig 6 Models of two biological roles of RS21-C6 protein (A) RS21-C6 may supply dCMP as an upstream precursor of de novo synthesis of dTTP CDP is reduced to dCDP by ribonucleotide reductase (RNR) dCTP is synthesized by phosphorylation of dCDP Excess dCTP is hydrolyzed to dCMP by RS21-C6 dCMP is converted to dTMP by dCMP deaminase (DCTD) and thymidylate synthase (TS) dTMP is converted to dTTP by two steps of phosphorylation RS21-C6 plays a role in regulation of the dCTP ⁄ dTTP ratio in dNTP pools for nuclear and mitochondrial DNA synthesis (B) RS21-C6 may hydrolyze 5-I-dCTP or its structurally related nucleotides to prevent inappropriate CpG methylation.

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prevent such deleterious effects by hydrolyzing

modi-fied deoxynucleotides including 5-I-dCTP or its

struc-turally related molecules (Fig 6B)

5-Methyl-dCTP is also a potential inducer of

inap-propriate CpG methylation by DNA

methyltransfer-ase 1 when it is incorporated in a CpG site Although

5-methyl-dCMP is a poor substrate for mammalian

nucleoside monophosphate kinases [34,35],

5-methyl-dCMP has been shown to be incorporated into the

DNA of Chinese hamster ovary cells with low dCMP

deaminase activity [36] Using preliminary

compara-tive modeling, Moroz et al [8] found that

5-methyl-dCTP is a potential substrate candidate of RS21-C6,

and 5-methyl-dCTP hydrolyzing activity of RS21-C6

was recently reported by Wu et al [10] In the present

study, we show that the kcat⁄ Km value of RS21-C6

for 5-methyl-dCTP is almost the same as that for

dCTP More detailed comparisons of the enzyme

kinetics for dCTP and 5-methyl-dCTP are necessary

to establish the physiological role of RS21-C6 for

5-methyl-dCTP

XTP3TPA (gi|13129100) is a human homolog of

RS21-C6 Our study demonstrated the substrate

pref-erence of RS21-C6 for deoxynucleotides, cytosine bases

and iodination at C5 of cytosine These data suggest

that a defect of human XTP3TPA might cause a

nuclear DNA replication block or mitochondrial DNA

depletion, as a result of an imbalanced dCTP⁄ dTTP

ratio Moreover, XTP3TPA might be involved in

tumorigenesis in chronically inflamed tissues, as a

result of accumulation of modified deoxynucleotides

Experimental procedures

Synthetic oligonucleotides

The synthetic oligonucleotides listed below, used as PCR

primers, were purchased from Genenet Co Ltd (Fukuoka,

Japan), Sigma-Aldrich Japan (Tokyo, Japan) and Takara

Bio Inc (Ohtsu, Japan): 5¢Nde-mMAZG, 5¢-ATACATATG

TCCACGGCTGGTGACGGTGAGCG-3¢; 5¢Nco-mMAZG,

5¢-ATACCATGGCCTCCACGGCTGGTGACGGTGAGC-3¢; 3¢mMAZG-BamHI, 5¢-ATAGGATCCTTATGTGGAAG

CCTGGTCTCTC-3¢; RS21-C6 forward, 5¢-GCGAGCTGGC

AGAACTCTTC-3¢; RS21-C6 reverse, 5¢-TTTGGTGGCCA

TGCTTGA-3¢; 18S rRNA forward, 5¢-AGGATGTGAAGG

ATGGGAAG-3¢; 18S rRNA reverse, 5¢-ACGAAGGCCCC

AAAAGTG-3¢

Nucleotides

The nucleotides used as substrates for RS21-C6 were

pur-chased from Sigma-Aldrich (St Louis, MO, USA), TriLink

Biotechnologies Inc (San Diego, CA, USA) or Jena Biosci-ence (Jena, Germany)

Preparation of mouse thymocyte extract Five-week-old C57BL⁄ 6J male mice (Clea Japan, Tokyo, Japan) were dissected under pentobarbital anesthesia (75 mgÆkg)1, intraperitoneally), and killed by blood drai-nage from abdominal vessels The thymus was removed and ground between glass slides to prepare thymocyte sus-pensions Thymocytes (6· 108

) were suspended in 3 mL of lysis buffer {25 mm 2-[4-(2-Hydroxyethyl)-1-piperazinyl] ethanesulfonate-Na pH 7.2, 150 mm NaCl, 60 mm MgCl2, 0.05% Nonidet P-40 (Nacalai tesque, Kyoto, Japan), 1 mm dithiothreitol, 1% protease inhibitor cocktail (Nacalai Tesque)}, and were disrupted by sonication Cell lysates were then centrifuged at 100 000 g for 30 min The super-natant was collected as the thymocyte extract Handling and killing of all animals used in this study were in accor-dance with the national prescribed guidelines, and ethical approval for the studies was granted by the Animal Experi-ment Committee of Kyushu University (Fukuoka, Japan)

Preparation of ITP-agarose ATP-agarose (adenosine 5¢-triphosphate agarose, Sigma-Aldrich; 25 lL bed volume) or 25 lL agarose carrier matrix were washed for 2 min twice in 1 mL 3 m sodium acetate buffer (pH 3.2), and then suspended in 150 lL of deamina-tion buffer [100 mm sodium nitrite (NaNO2), 500 mm sodium thiocyanate (NaSCN), 3 m sodium acetate (NaCH

3-COO) pH 3.2], and incubated at 37C for 60 min Then each agarose aliquot was washed for 2 min twice in 1 mL

of water and used as ITP-agarose or as deaminated aga-rose, respectively To confirm the nucleotide immobilized

on each agarose, the base moiety was excised by incubation

in 1 m HCl at 100C for 1 h, and analyzed by HPLC after neutralization and filtration

Purification and identification of ITP-binding proteins

ITP-agarose was resuspended in 450 lL thymocyte extract

in a microtube, and mixed by vertical rotation for

30 min Agarose that had been subjected to deamination was used as the negative control Each agarose sample was then washed for 1 min three times in 1 mL of lysis buffer without protease inhibitor All procedures were per-formed at 4C Each agarose was then resuspended in

40 lL of 2· SDS sampling buffer (Sigma-Aldrich), and incubated at 95C for 5 min The supernatant was col-lected after centrifugation at 140 g for 5 s at room temperature Proteins in each sample were separated by SDS–PAGE and analyzed by LC-MS⁄ MS as described

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previously [37] Collision-induced dissociation spectra were

acquired and compared with those in the International

Protein Index (IPI version 3.16; European Bioinformatics

Institute Hinxton, UK) using the MASCOT search engine

(Matrix Science, Boston, MA, USA) The high-scoring

peptide sequences (MASCOT score > 45) assigned by

MASCOT were manually confirmed by comparison with

the corresponding collision-induced dissociation spectra

Finally we selected as candidate proteins those proteins for

which multiple peptides were identified in this analysis

Isolation of RS21-C6 cDNA

RS21-C6 cDNA fragments, RS21-C6(NdeI/BamHI) and

RS21-C6(NcoI/BamHI) were amplified by PCR from a

mouse fibroblast cell line, NIH/3T3, prepared as described

previously [38], using primer sets 5¢Nde-mMAZG/

3¢mMAZG-BamHI and

5¢Nco-mMAZG/3¢mMAZG-BamHI Amplified fragments were subcloned into pT7Blue-2

T-vector (Novagen, Madison, WI, USA) to generate

the plasmids pT7Blue2T:RS21-C6(NdeI⁄ BamHI) and

pT7Blue2T:RS21-C6(NcoI⁄ BamHI), respectively

Construction of expression plasmids

Plasmids pET3a:RS21-C6, pET32a(+):RS21-C6,

pcDNA3.1-hyg(+):RS21-C6 and pIRES2-EGFP:RS21-C6 were

prepared by inserting DNA fragments containing the

ORF of RS21-C6 cDNA into the NdeI⁄ BamHI site of

pET3a (Novagen), the NcoI⁄ BamHI site of pET32a(+)

(Novagen) or the XhoI⁄ BamHI site of pcDNA3.1hyg(+)

(Invitrogen, Carlsbad, CA, USA) or into the XhoI/BamHI

site of pIRES2-EGFP (Clontech Laboratories Inc.,

Moun-tain View, CA, USA), respectively

Expression and purification of recombinant

RS21-C6 protein

Expression of recombinant RS21-C6, without any tag

sequence, was induced in E coli

BL21-CodonPlus(DE3)-RIL cells (Stratagene, La Jolla, CA, USA) transformed

with pET3a:RS21-C6, as described previously [39] Cells

were suspended in buffer A (50 mm Tris⁄ HCl pH 8.0,

100 mm NaCl, 5 mm EDTA, 5 mm 2-mercaptoethanol,

1 mm phenylmethanesulfonyl fluoride, 1 lgÆmL)1

pepsta-tin A, 1 lgÆmL)1 chymostatin, 1 lgÆmL)1 leupeptin),

dis-rupted by sonication, and clarified by centrifugation at

20 000 g for 30 min at 4C Proteins in the supernatants

were precipitated using ammonium sulfate (40–50%

satura-tion), and re-dissolved in buffer B (50 mm Tris⁄ HCl

pH 8.0, 100 mm NaCl, 5% glycerol, 5 mm MgCl2, 5 mm

2-mercaptoehanol) Dissolved samples were dialyzed three

times against 1 L of buffer B and loaded onto HiTrap-Q

HP anion exchange columns (GE Healthcare, Chalfont

St Giles, UK) equilibrated with buffer C (50 mm Tris⁄ HCl

pH 8.0, 50 mm NaCl, 5% glycerol, 5 mm MgCl2, 5 mm 2-mercaptoehanol) Binding proteins were eluted using a linear gradient of NaCl (50–1000 mm) Fractions containing RS21-C6 protein were applied onto Superdex 75 HR10⁄ 30 size exclusion columns (Sigma-Aldrich) equilibrated with buffer B Fractions containing RS21-C6 were then loaded onto a MonoQ HR5⁄ 5 anion exchange column (GE Health-care) equilibrated with buffer C, and eluted using a linear gradient of NaCl (50–1000 mm) Fractions containing RS21-C6 were loaded sequentially onto HiTrap-S HP and HiTrap heparin columns (GE Healthcare), and flow-through fractions were collected RS21-C6 protein was concentrated using HiTrap-Q columns, dialyzed against buffer D (50 mm Tris⁄ HCl pH 8.0, 100 mm NaCl, 50% glycerol, 5 mm MgCl2, 1 mm dithiothreitol), and stored at)30 C as puri-fied RS21-C6 protein

Nucleotide-hydrolyzing assay with RS21-C6 protein

Substrate nucleotides were incubated in 18 lL of reaction buffer [50 mm Tris⁄ HCl pH 8.0, 100 mm KCl, 5 mm MgCl2, 100 lgÆmL)1 BSA (New England Biolabs Inc., Ipswich, MA, USA), 1 mm dithiothreitol] at 30C for

10 min Then, 2 lL of 500 nm RS21-C6 protein, in reac-tion buffer, was added to the reacreac-tion and further incu-bated at 30C for 0–30 min Sample solutions were mixed with 10 lL of ice-cold 50 mm EDTA to stop the reactions, clarified by centrifugation at 9000 g for 10 min

at 4C, and separated on SunFire C18 5 lm 4.6 ·

250 mm columns (Waters, Milford, MA, USA) or TSK gel DEAE-2SW columns (Tohso, Tokyo, Japan) using an Alliance photodiode array HPLC system (Waters), at a flow rate of 1 mLÆmin)1 with HPLC buffer 1 (0.1 m potassium phosphate pH 6.0, 5% methanol) or HPLC buffer 2 (75 mm sodium phosphate pH 6.0, 20% acetoni-trile, 0.4 mm EDTA) The amounts of nucleotide were quantified by UV absorption

Anti-RS21-C6 Ig

An antigen, TrxA-RS21-C6 protein, was expressed in

E coli BL21-CodonPlus (DE3)-RIL cells transformed with pET32a(+):RS21-C6, and purified by metal affinity chro-matography with TALON beads (Clontech) Preparation of rabbit anti-TrxA-RS21-C6 serum and affinity purification

of anti-RS21-C6 Ig were performed as described previously [39,40]

Western blot Western blot analysis using antibodies against anti-RS21-C6, Lamin B (Santa Cruz Biotechnology Inc., Santa Cruz,

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