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In this article, it is shown that, under physiological conditions, ADP may be formed in mammalian tissues by the disproportionation of AMP, consistent with an AMP–AMP phosphotransferase

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nucleotide metabolism

Daniela Vannoni1,*, Roberto Leoncini1,*, Stefania Giglioni1, Neri Niccolai2, Ottavia Spiga2, Emilia Aceto1and Enrico Marinello1

1 Department of Internal Medicine, Endocrine-Metabolic Sciences and Biochemistry, University of Siena, Italy

2 Department of Molecular Biology, University of Siena, Italy

Purine nucleotides are precursors of nucleic acids and

participate in many metabolic pathways as substrates,

coenzymes and energy sources Much attention has

been focused on the roles and intracellular levels of

AMP, ADP and ATP in various tissues under normal

and pathological conditions [1–3] Their relationships

to genetic diseases, blood disorders, drugs, tumours

and other pathologies have been studied extensively

[4–6]

Although ATP formation occurs via several

well-known mechanisms, ADP is thought to be formed

only from ATP, either by the adenylate kinase reaction

or by ATPase-mediated hydrolysis The possibility that

ADP can be formed by de novo synthesis from

low-energy precursors has never been investigated fully Even less studied is the possibility that ADP might be synthesized from low-energy precursors under special conditions, such as ischaemia and hypoxia, in which massive depletion of ATP and elevation of AMP are known to occur [7–10]

In this article, it is shown that, under physiological conditions, ADP may be formed in mammalian tissues

by the disproportionation of AMP, consistent with an AMP–AMP phosphotransferase reaction This reaction

is carried out by enzymes of purine metabolism which, under specific cellular conditions, associate in a biolog-ical network and cooperate in a reaction not reported previously

Keywords

adenosine deaminase; adenosine kinase;

adenylate kinase; ADP; ATP

Correspondence

R Leoncini, Department of Internal

Medicine, Endocrine-Metabolic Sciences

and Biochemistry, Via A Moro 2,

53100 Siena, Italy

Fax: +39 0577 234285

Tel: +39 0577 234287

E-mail: leoncini@unisi.it

*These authors contributed equally to this

work

(Received 16 July 2008, revised 24

September 2008, accepted 5 November

2008)

doi:10.1111/j.1742-4658.2008.06779.x

Crude rat liver extract showed AMP–AMP phosphotransferase activity which, on purification, was ascribed to a novel interaction between adeny-late kinase, also known as myokinase (EC 2.7.4.3), and adenosine kinase (EC 2.7.1.20) The activity was duplicated using the same enzymes purified from recombinant sources The reaction requires physical contact between myokinase and adenosine kinase, and the net reaction is aided by the pres-ence of adenosine deaminase (EC 3.5.4.4), which fills the gap in the energy balance of the phosphoryl transfer and shifts the equilibrium towards ADP and inosine synthesis The proposed mechanism involves the association of adenosine kinase and myokinase through non-covalent, transient interac-tions that induce slight conformational changes in the active site of myokinase, bringing two already bound molecules of AMP together for phosphoryl transfer to form ADP The proposed mechanism suggests a physiological role for the enzymes and for the AMP–AMP phosphotrans-ferase reaction under conditions of extreme energy drain (such as hypoxia

or temporary anoxia, as in cancer tissues) when the enzymes cannot display their conventional activity because of substrate deficiency

Abbreviations

ADA, adenosine deaminase; AdK, adenosine kinase; CE, capillary electrophoresis; MD, molecular dynamics; MK, myokinase.

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These enzymes are well known for their

conven-tional reactions:

(a) Adenylate kinase, also called myokinase (MK),

which catalyses the reversible reaction:

AMPþ ATP $ ADP (b) Adenosine kinase (AdK), which catalyses the

irreversible reaction:

Adenosineþ ATP ! AMP þ ADP

(c) Adenosine deaminase (ADA), which catalyses the

irreversible reaction:

Adenosineþ H2O! inosine þ NH3

The AMP–AMP phosphotransferase reaction is:

AMPþ AMP ! ADP þ inosine þ NH3

ADP and inosine were identified by various

meth-ods, including HPLC and diode array analysis; the

enzymes responsible for the reaction were identified

through a complex purification procedure Our

find-ings demonstrate that MK and AdK carried out the

reaction, and ADA enhanced the rate ATP was

formed only in the presence of ADA, and at longer

incubation times, when the ADP concentration passed

the threshold necessary to allow the initiation of the

MK reaction and the AMP concentration decreased

below the inhibitory concentration for MK In this

study, we investigated the complex mechanisms

under-lying this reaction and its physiological role in cell

metabolism

Results

AMP–AMP phosphotransferase reaction

Dialysed rat liver supernatant was incubated with

AMP and Mg2+; HPLC analysis revealed the

forma-tion of two products with retenforma-tion times of 1.3 and

7.1 min, which were identified as inosine and ADP,

respectively (Fig 1) Using purified [32P]AMP as a

sub-strate, ADP formation was consistent with the transfer

of 32P between two molecules of [32P]AMP to form

[32P]ADP[a,bP] Thus, starting from [32P]AMP with a

specific radioactivity of 50 500 ± 1517 d.p.m.Ænmol)1

(mean of five experiments), we obtained ADP with a

specific radioactivity of 108 125 ± 4325 d.p.m.Ænmol)1,

double that of the starting AMP Hydrolysis of the

product yielded two moles of32Piper mole of ADP

These findings indicate that supernatants starting

from low-energy precursors catalyse the AMP–AMP

phosphotransferase reaction:

AMPþ AMP ! ADP þ inosine þ NH3 ð1Þ resulting from reactions (2) and (3):

AMPþ AMP ! ADP þ adenosine ð2Þ Adenosineþ H2O! inosine þ NH3ðADA reactionÞ ð3Þ ADA converts adenosine to inosine and ammonia in stoichiometric amounts with respect to ADP

Enzyme purification and identification by mass spectrometry

Protein purification demonstrated that ADP forma-tion occurred via the activities of MK and AdK, with the cooperation of ADA The crude supernatant and P90d fraction showed AMP–AMP phosphotransferase activity, but any further chromatography led to the loss of activity, presumably because the two different proteins were separated from one another Therefore,

we purified each protein individually by an appro-priate procedure AMP–AMP phosphotransferase activity was restored every time we combined the two separate protein preparations ADA was also isolated When ADA was added to the assay mixture, AMP– AMP phosphotransferase activity was greatly enhanced

The purifications were performed as reported in Table 1 The final SDS-PAGE showed a single band for each protein preparation

Fractions X2 and Y2 were identified as MK and AdK, respectively, by electrospray mass spectrometry The X2 fraction spectra revealed a component with a molecular mass of 26 232.5 ± 0.5 Da (Fig 2) Twenty signals ranging in mass from m⁄ z 780.7 to m ⁄ z 1726.8 were selected and entered into the non-redundant National Center for Biotechnology Information data-base using pro found software The query returned a

Fig 1 Identification of ADP and inosine in the reaction mixture The figure shows the typical HPLC pattern of an assay mixture incubated with crude extract of rat liver (0.5 mg) for 50 min Numbered peaks: 1, inosine; 2, AMP; 3, ADP.

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significant match with the protein MK (isoenzyme 2)

from Rattus norvegicus The mass signals used in the

search procedure accounted for 72% of the entire MK

sequence, confirming identification The electrospray

mass spectra of Y2 revealed a component with a

molecular mass of 38 344.2 ± 2.1 Da (Fig 3) Twenty

signals ranging in mass from m⁄ z 1235.94 to m ⁄ z

3316.66 were selected, and the query returned a highly

significant match with the protein AdK from R

nor-vegicus The mass signals used in the search procedure

accounted for 80% of the entire AdK sequence,

confirming identification Mass analysis of fractions

X2 and Y2 confirmed the purity of the two purified

proteins (Figs 2 and 3)

AMP–AMP phosphotransferase activity by

recombinant enzymes

One microgram of Escherichia coli-expressed human

recombinant AdK, mixed with commercial

recombi-nant MK and ADA, exhibited AMP–AMP

phospho-transferase activity Commercial preparations of

purified MK and ADA from several sources produced

the same result as the purified rat liver enzymes (AdK

was not commercially available)

General properties of the reaction AMP–AMP phosphotransferase activity was detectable

in different rat tissues (muscle, brain, spleen) and was absolutely specific for AMP The formation of ADP and inosine progressed over time and no products were formed when the protein extract was denatured by heat or acid The time course of the reaction was linear for at least 60 min when only MK and AdK were present When ADA was added, the shape of the curve remained as before for the first 15 min; thereafter, the reaction proceeded linearly for 50 min, at a rate 20 times greater than that for MK and AdK alone (Fig 4)

ADP and inosine formation varied according to the incubation temperature, pH, AMP concentration and

Mg2+ concentration Maximum activity was found at

pH 6.5–6.8, and the activity decreased sharply at pH values above 7.5 and below 5.5 The Km value for AMP was 0.8 mm (Fig 5A) Mg2+ was essential for the AMP–AMP phosphotransferase reaction, with the optimal concentration in the range 0.8–1.5 mm The apparent Km value of Mg2+ was 0.35 mm (Fig 5B) Certain ions added to the incubation mixture inhibited (NHþ

4, Li+ and SO2

4 ) or had no effect (Ca2+ and

Table 1 Purification of rat liver X2 and Y2 proteins The two proteins responsible for AMP–AMP phosphotransferase activity in rat liver were purified The procedure started with a common trunk and had three steps: supernatant production, ammonium sulfate precipitation and dialysis During these steps, the two proteins were not separated and phosphotransferase activity was present in all fractions After DE-52 chromatography, the purification process diverged: non-retained fractions were utilized for the purification of protein X (X1 or X2; finally identified as MK) and part of the retained fractions was used for the purification of protein Y (Y1 or Y2; finally identified as AdK) AMP–AMP phosphotransferase activity was only detected when suitable amounts (0.003–0.5 mg) of fractions from each purification branch were pooled (i.e X1 + Y1, X2 + Y2).Total AMP–AMP phosphotransferase activity is expressed in IU, which corresponds to the number of micromoles of ADP or ADP + ATP formed per minute ND, AMP–AMP phosphotransferase activity was not detectable.

Common trunk

Step

Supernatant

P90d

DE-52

Retained (R)

X protein

X1 Blue Sepharose

X2 Superdex 75

Y protein

Y1 AMP Sepharose

X1 + Y1

Y2 + Y2

Y2 Superdex 75

ND ND ND

ND ND

ND ND ND ND

ND ND

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PO23 ) on the reaction Zn2+ completely eliminated

phosphotransferase activity

The amounts and ratios of enzymes in the assay

mixture reflected those reported to be present in rat

liver, namely MK@ 0.45 IU and AdK @ 0.015 IU,

with an MK : AdK ratio of approximately 30 [11,12]

In our assay mixture, we used 1–3 lg of pure protein,

corresponding to approximately 0.21–0.63 IU MK and

0.006–0.018 IU AdK, with a ratio of about 10–30

When ADA was present in the assay mixture, ATP

was formed when the incubation time exceeded

15–20 min, and increased with time ATP, with a

retention time of 9.2 min on the HPLC chromatogram,

was identified using the same criteria as for ADP

Using [32P]AMP as a substrate, the ATP formed had a

specific radioactivity of 146 338 ± 7316 d.p.m.Ænmol)1,

three times that of AMP ATP was formed by the

con-ventional MK reaction, when the ADP concentration

in the mixture passed the threshold of 0.05 mm (data

not shown); at lower ADP concentrations, MK activity

was inefficient because of a lack of substrate At longer incubation times, when the ADP concentration exceeded the Kmvalue for MK (0.3 mm) and the AMP concentration fell below the inhibitory concentration (Ki of AMP for MK, 2.13 mm), MK exerted its con-ventional activity, eventually reaching equilibrium [12] The Ki value of AMP for MK was not influenced by the presence of AdK

Reversibility of the reactions

We considered the reversibility of the reactions: AMPþ AMPðAdK;MK;ADAÞ! ADPþ inosine þ NH3 ð1Þ AMPþ AMPðAdK;MKÞ! ADPþ adenosine ð2Þ Reaction (1) was irreversible and reaction (2) also appeared to be irreversible; in the presence of ADP and adenosine alone, MK activity was absolutely

A

B

Fig 2 Electrospray ionization mass spectra of the X2 fraction identified as MK (A) Gaussian-type distribution of multiply charged ions (B) The m ⁄ z spectrum converted to a molecular mass profile by maximum entropy processing The mass profile is dominated by a single com-ponent, showing the purity grade of the protein The molecular mass of the sample was calculated by the processing software associated with the mass spectrometer to a mass accuracy of 0.01%, and can be read directly on the graph.

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predominant, and so equimolar amounts of AMP and

ATP were formed by the very rapid conventional MK

reaction [see reaction (a) in the introductory section]

Moreover, if we added 0.2 lCi of [14C]adenosine to

the incubation mixture, no [14C]AMP was formed

Reaction mechanism studies – cooperation

between AdK and MK

Micro-equilibrium dialysis experiments

No reaction products were formed when AdK and

MK were separated by a dialysis membrane during the

reaction, regardless of the presence of ADA The

AMP–AMP phosphotransferase reaction could only be

detected when AdK and MK were incubated in the

same chamber, in which case the presence of ADA

only affected the rate of the reaction

Detection of reaction intermediates by HPLC, capillary electrophoresis (CE) and NMR analysis

HPLC, CE and NMR analysis of the incubation mix-ture at several points during the incubation indicated that no intermediate products were formed In all cases,

we observed a decrease in AMP concentration over time and an increase in adenosine or inosine, ADP and ATP concentrations; no other products were detected

Enzyme–phosphate intermediate trapping When enzyme–phosphate intermediate trapping experi-ments were performed, no spot was detected on the autoradiography slide, indicating direct transfer of the phosphoryl group from one AMP molecule to another, without the formation of an intermediate

A

B

Fig 3 Electrospray ionization mass spectra of the Y2 fraction identified as AdK (A) Gaussian-type distribution of multiply charged ions (B) The m ⁄ z spectrum converted to a molecular mass profile using maximum entropy processing The mass profile is dominated by a single component, showing the purity grade of the protein The molecular mass of the sample was calculated by the processing software associ-ated with the mass spectrometer to a mass accuracy of 0.01%, and can be read directly on the graph.

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phosphoenzyme species When vanadate was added to

the reaction mixture, no inhibition of AMP–AMP

phos-photransferase was observed Moreover, the addition of

[14C]nucleoside (adenosine, guanosine, inosine) did not

promote the formation of [14C]AMP, [14C]GMP or

[14C]IMP The formation of ADP was unchanged

Gel filtration and SDS-PAGE

Assay mixtures incubated for 0 and 60 min were

sub-mitted to gel filtration The resulting chromatograms

showed no differences in retention times, indicating

that no stable protein complex was formed Moreover,

when the same samples were resolved by SDS-PAGE,

no bands were observed at a molecular mass higher

than AdK, ruling out the formation of covalent bonds

between the proteins

Docking simulation studies

We performed a molecular dynamics (MD) simulation

of the interaction between AMP and AdK or MK

using autodock This procedure suggested that, from

an energy point of view, each protein had two binding

sites, either of which could be occupied by AMP gromacs was then used to optimize the molecular models with energy minimizations followed by a 1 ns

MD simulation Using the MD trajectory, possible changes in AMP position and in the network of bonds between the AMP molecules and binding pockets were examined In the case of rat MK, one of the two bound AMP molecules maintained the same location and orientation during all MD runs, whereas the other molecule changed position and approached the first AMP molecule Indeed, the distance between these two molecules was 8.19 A˚ at the beginning of the docking simulation, and 5.61 A˚ at the end of the simulation (Fig 6B), close to the 4.5 A˚ distance between the natu-ral ligands AMP and ATP (Fig 6A) With regard to AdK, the MD simulation showed that the two AMP molecules were bound in positions very distant from each other (Fig 7)

Kinetic analysis Kinetic experiments were performed in the absence

of ADA AMP–AMP phosphotransferase activity

9

A

B

6

3

0

0

200

150

100

50

0

10 20 30

Minutes

Minutes

40 50 60

0 10 20 30 40 50 60

Fig 4 Time course of ADP formation by the AMP–AMP

phospho-transferase reaction (A) 0.38 IU of purified rat liver MK and

0.012 IU of AdK The amount of ADP produced (nmol) is shown on

the ordinate (B) 0.38 IU of purified rat liver MK, 0.012 IU of AdK

and 0.9 IU of ADA The amount of ADP + ATP formed (nmol) is

shown on the ordinate.

Fig 5 (A) Direct and double reciprocal plot of initial velocities with variable AMP concentration (0.0–6.0 m M ) at a constant Mg 2+ concentration (1.0 m M ) (B) Direct plot of the initial velocities with variable Mg 2+ concentration (0.1–1.4 m M ) and constant AMP concentration (4.0 m M ) Values are the mean ± standard deviation

of five experiments Assay mixtures contained 0.15 IU MK, 0.004 IU AdK and 0.3 IU ADA The amount of ADP + ATP formed (nmolÆh) is shown on the ordinate.

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gradually increased to a plateau, following a typical

hyperbolic curve (Fig 8), and reached a maximum

saturating value when the concentration of AdK

exceeded 2 nmol (AdK : MK ratio > 2000) The Kd

value was 1.496 lm, as determined by the Scatchard

equation [13]

Inhibition experiments

The results of the inhibition assays are presented in

Table 2 Each inhibitor completely blocked the

activity of its respective enzyme, i.e Ap5A inhibited

MK and A134974 inhibited AdK Neither inhibitor

interfered with the progress of the other reaction

AMP–AMP phosphotransferase activity was only

affected by the presence of Ap5A, when it fell to

zero A134974 had only a slight effect on the

AMP–AMP phosphotransferase reaction (< 10%

inhibition)

AMP–AMP phosphotransferase reaction in human colorectal mucosa from cancer patients

Table 3 shows the activities of AdK, MK, ADA and AMP–AMP phosphotransferase in normal and cancer-ous human colorectal mucosa The MK activity did not vary, but the AdK and ADA activities were signifi-cantly elevated (P < 0.0001) in cancer tissue with respect to the surrounding normal mucosa AMP– AMP phosphotransferase activity was only detectable

in tumour tissue

A

B

Fig 6 (A) Backbone superimposition of human (green) and rat

(red) MK The natural ligands (AMP and ATP) and the two AMP

molecules (red) are shown in bold (B) Backbone superimposition of

rat MK before (magenta) and after (green) the MD run Note the

reduction in the distance between the two ligands.

Fig 7 Backbone superimposition of human (blue) and rat (magenta) AdK The natural ligands (ADA and ATP, blue) and the two AMP molecules (magenta) are shown in bold.

Fig 8 Direct plot of AMP–AMP phosphotransferase activity (as percentage of total activity) in mixtures containing various amounts

of AdK (0.1–4.0 nmol in a final volume of 0.25 mL) and a fixed amount of MK (0.0008 nmol) in the absence of ADA Inset: Scatchard plot.

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Cooperation between the three enzymes

Our data demonstrate that the AMP–AMP

phospho-transferase reaction occurs by cooperation between

MK and AdK specifically, and is enhanced by ADA;

none of the three enzymes could be substituted by

others

ADA strongly accelerates the AMP–AMP

phospho-transferase reaction, carrying out the coupled reaction

(3) (adenosine fi inosine + NH3) ADA does not

participate directly in ADP synthesis, but its coupled

reaction enables the subtraction of a reaction product,

helping to drive the reaction forwards Moreover, the

exergonic formation of inosine and ammonia from

adenosine fills the gap in the energy balance of

phosphoryl transfer (DG ranges from )4 to )10

-kcalÆmol)1) [14] ADA has a Keq value of

approxi-mately 105 [15], a low Km value and a high efficiency

close to the diffusion-limited rate [16], and is found in

most mammalian tissues [17] It follows that the

AMP–AMP phosphotransferase reaction always

bene-fits from the presence of ADA in vivo

The AMP–AMP phosphotransferase reaction is irre-versible Any attempt at producing AMP by incubat-ing ADP, inosine and NH3 together is ineffective In the absence of ADA, the addition of [14C]adenosine to the mixture does not produce [14C]AMP

Demonstrating the association between AdK and

MK was the key to understanding the mechanism of AMP–AMP phosphotransferase We obtained the following results

(a) No free intermediates were formed during any incubation experiment (that is, no transient dinucleo-tide compound), in contrast with the NADase reaction [18] HPLC, CE and NMR analysis produced no evidence of a reaction intermediate

(b) The existence of an enzyme–phosphate interme-diate, as is formed with 5¢-nucleotidase [19], was ruled out No direct evidence of an enzyme–phosphate com-plex was found Experiments using vanadate or the addition of nucleoside also yielded negative results We found no evidence of AdK–phosphate or MK–phos-phate complex formation in the literature

(c) Microdialysis demonstrated that MK and AdK must be able to associate and work together When they were physically separated, no ADP was formed (d) Electrophoresis and gel filtration experiments excluded covalent or similarly strong interactions (e) In silico simulations suggested that MK contains the active site of the AMP–AMP phosphotransferase reaction, but raised the issue of how the two AMP molecules achieve the correct distance for interaction, and the role of AdK in the reaction

(f) Inhibition experiments confirmed the role of MK

in the reaction Ap5A inhibited MK and AMP–AMP phosphotransferase activities, whereas A134974 only inhibited AdK activity, indicating that the active site

of AdK is not essential for the AMP–AMP phospho-transferase reaction

(g) Kinetic experiments (Fig 8) demonstrated that interactions occurred between the two proteins When

we fixed the amount of MK at a low concentration and increased the concentration of AdK, we obtained

a hyperbolic curve with a saturation trend resembling protein–protein interaction Indeed, isothermal curves, such as the enzyme–substrate curves of Michaelis– Menten, hormone–receptor curves and antigen–anti-body affinity curves, all represent an association between two different molecules and, in the last two cases, between two different proteins [20] In all of these curves, the ordinate values indicate the amount

of dimer formed In the case of Michaelis–Menten plots, the value of V is related to the enzyme–substrate complex; in the case of hormone–receptor curves, the ratio B⁄ Bmax represents the amount of receptor joined

Table 2 Rat liver MK, AdK and AMP–AMP phosphotransferase

specific activities, expressed as lmolÆ(min mg))1 (means ±

stan-dard deviation of eight experiments), were tested in the presence

or absence of 0.25 l M Ap5A (specific inhibitor of MK) or 0.1 n M

A134974 (specific inhibitor of AdK) The mixtures contained 0.2 IU

MK, 0.02 IU AdK and 2 IU adenosine deaminase (the latter only in

the AMP–AMP phosphotransferase assay).

MK activity in the presence of Ap5A 0

MK activity in the presence of A134974 61.42 ± 1.18

AdK activity in the presence of A134974 0

AdK activity in the presence of Ap5A 5.46 ± 0.3

Table 3 MK, AdK, ADA and AMP–AMP phosphotransferase

activi-ties in normal and human colorectal cancer mucosa were assayed.

Partially purified protein preparations (20, 40, 80 and 100 lg,

respectively) were incubated The activities of AdK, ADA and AMP–

AMP phosphotransferase are expressed as lmolÆ(h mg))1, and are

the mean activity ± standard deviation from 10 different patients.

*P < 0.0001.

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to the hormone with respect to the maximum complex

possible In our case, the ordinate shows the formation

of ADP, which represents the number of dimers

formed between the two proteins In all of these cases,

the association is caused by non-covalent interactions,

such as hydrogen or ionic binding, hydrophobic

inter-actions or van der Waals’ interinter-actions

We conclude that AdK and MK associate through

transient interactions that induce a slight

conforma-tional change in the active site of MK, thereby

bring-ing two already bound molecules of AMP together at

the correct distance for interaction and phosphoryl

transfer from one molecule to the other, ultimately

forming ADP

Physiological role

Our data support the conclusion that the AMP–AMP

phosphotransferase reaction is physiologically

impor-tant The reaction occurs in homogenates and crude

supernatants at pH values close to 7 The apparent Km

value for the substrate is close to its intracellular

con-centration in rat liver [21–23], and is of the same order

of magnitude as that of many enzymes involved in

nucleotide metabolism [24–28] The AdK, MK and

ADA concentrations used in these experiments

coin-cide with the concentrations found in rat liver tissue

[11,12]

Comparing the rates of the AMP–AMP

phospho-transferase, AdK and MK reactions is inappropriate

because the activities, rates and efficiencies of the

three reactions differ in vitro and in vivo according to

the concentrations of their natural substrates (AMP,

ADP, ATP), which vary continuously in different

situations

Under physiological conditions, the AMP–AMP

phosphotransferase reaction may contribute to the fine

regulation of ADP levels Its importance may be

greater under situations associated with ADP and

ATP deficiency, or increased requirements, such as

prolonged physical exertion (when ATP is dramatically

reduced), fructose-induced hyperuricaemia with ATP

depletion [29] and severe nucleotide depletion, as in

rheumatoid arthritis [30] and during cell division The

reaction may play a specific role during transient

ischaemia, anoxia or after reperfusion The behaviours

of AMP, ADP and ATP under such conditions have

been studied extensively and are similar in the liver [1],

heart and brain [10,31] During ischaemia, levels of

ATP and ADP decrease [1,6,10,12,32], whereas those

of AMP increase sharply, reaching up to 2 lmol AMPÆ

g)1 tissue [32] During prolonged ischaemia, ATP

levels decrease to < 10% [1,6,10,32], and ADP levels

decrease to 25–50% of their respective basal concentra-tions [6,32], whereas AMP levels increase by more than

20 times [32] ADP levels are presumably sustained by continuous regeneration, which is unlikely to occur through the classical MK reaction because, under such conditions, the levels of ATP are too low and the levels of AMP are too high to permit classical MK activity Tamura et al [33] reported that liver MK was inhibited by AMP concentrations above 0.5 mm and that the physiological significance of the data were unclear, as this high concentration greatly exceeds the concentration of AMP in rat liver (0.1 mm) In this sit-uation, MK is inhibited and the action of AMP–AMP phosphotransferase prevails, regenerating ADP Gly-colysis and oxidative phosphorylation can regenerate ATP from ADP and Pi; cooperation between these processes and AMP–AMP phosphotransferase will sustain ADP levels and regenerate ATP

The experiments on human cancer mucosa produced interesting results Hypoxia is a well-known feature of locally advanced solid tumours [34], and induces major adaptive responses, such as the production of angio-genic cytokines that promote vascularization and over-expression of the hypoxia-inducible factor-1a gene, which is a classical feature of tumour tissues [35] and was confirmed in our specimens (data not reported) In tumour tissue, AMP levels are more than five times higher than in normal mucosa, whereas the ATP con-centration is more than 10 times lower In contrast, ADP levels remain almost the same (data not reported) Moreover, AMP–AMP phosphotransferase activity was not detectable in normal mucosa, but was substantial in tumour tissue In the same specimens,

MK activity did not vary, whereas ADA and, espe-cially, AdK activities increased Therefore, in hypoxic tissue, the enzyme ratios reach the correct value for the AMP–AMP phosphotransferase reaction The importance of the reaction in tumour tissues will be the subject of future research

We conclude that, under specific conditions, ADP and ATP may be salvaged and restored through the concerted effects of classical ATP formation pathways and the AMP–AMP phosphotransferase network The latter reaction may represent a compensatory mecha-nism for maintaining and increasing ADP to the levels necessary for the restoration of ATP, with the simulta-neous re-utilization of AMP The existence of ATP turnover pathways has been suggested by other authors in studies on MK in creatine kinase-deficient transgenic hearts [36], specifically the existence of alter-native pathways for phospho transfer in the myocar-dium Our experiments indicate that the cooperative action of different proteins may provide fine regulation

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of metabolic reactions, a poorly understood

phenome-non and an avenue of research that should be explored

further

Experimental procedures

Materials

Male Wistar rats (body weight, 250 g; 9 weeks of age) were

purchased from Harlan Company (S Pietro al Natisone,

Udine, Italy) Nucleosides, nucleotides, bases, enzymes,

analytes and the ATP Bioluminescent Assay Kit

(FLAA-1KT) were obtained from Sigma Life Science (Milan, Italy)

SDS-PAGE reagents and protein assay kits were procured

from Bio-Rad Laboratories s.r.l (Milan, Italy)

Chromato-graphic supports, radioactive compounds and

low-molecu-lar-mass protein molecular mass marker kits were obtained

from GE Healthcare Europe GmbH (Milan, Italy)

N-Hy-droxysuccinimide, 1-ethyl-3-(3-dimethylaminopropyl) and

ethanolamine hydrochloride were purchased from Affinity

Sensor (Cambridge, UK) Aquasafe 300 plus was obtained

from Zinsser Analytic (Frankfurt, Germany) HPLC-grade

trifluoroacetic acid was obtained from Carlo Erba Reagenti

SpA (Rodano, Italy) Recombinant enzymes were

pur-chased from ABNOVA Corporation (Taipei, Taiwan) All

other chemicals and HPLC solvents were of analytical

grade and were acquired from Merck KGaA (Darmstadt,

Germany) and J T Baker Italia (Milan, Italy)

Enzyme assays and identification of reaction

products

Enzyme assays

The AMP–AMP phosphotransferase assay mixture

con-tained 4.0 mm cold AMP or 640 kBq [32P]AMP, 50 mm

Bistris (pH 6.5), 1.0 mm MgCl2, up to 0.5 mg of dialysed

crude rat liver supernatant or 1–3 lg of purified fraction

X2 (corresponding to 0.19–0.57 IU of pure MK), fraction

Y2 (corresponding to 0.006–0.018 IU of pure AdK) and

ADA (0.9 IU) in a final volume of 0.25 mL Incubations

were performed at 37C for 15–50 min and stopped with

perchloric acid (neutralized with KOH) or 0.01 mm EDTA;

aliquots were processed by HPLC according to Webster

and Whaun [37] The ammonium content was assayed using

the hypochlorite–phenol Berthelot reaction, according to

Imler et al [38]

One International Unit (IU) of MK, AdK, ADA or

AMP–AMP phosphotransferase was defined as the amount

of enzyme that produced 1 lmolÆmin)1of reaction product

We considered ADP (or ADP + ATP) to be the product

formed by the AMP–AMP phosphotransferase reaction

AMP and all other substrates used in the assay mixture

were purified by HPLC Traces of ATP in the substrates

and enzyme preparations were excluded using the ATP

Bioluminescent Assay Kit CLS II (Roche Diagnostics GmbH, Mannheim, Germany; Sirius Luminometer-Berthold GmbH, Pforzheim, Germany) AdK and ADA were assayed according to Tavernier et al.[39] MK was assayed according to Zhang et al [40]

HPLC analysis

We used a Perkin-Elmer (Monza, Italy) 1020LC Plus system equipped with a ready-to-use prepacked column (Hypersil SAX 5 lm, 150· 4.6 mm; Alltech Italia s.r.l., Segrate, Italy) washed with 5.0 mm ammonium phosphate (pH 2.9) Elution was achieved with 0.5 m ammonium phosphate buffer (pH 4.8) at a flow rate of 1.5 mLÆmin)1, using a linear gradi-ent from 0% to 100% in 10 min The lower limit of detection

of the method was 100 pmol

When [32P]AMP was used, the ADP and ATP peaks were collected and mixed with 10 mL Aquasafe 300 Plus emulsifying scintillator, and the radioactivity was measured using a Packard Model 1500 TriCarb b-counter (Hewlett Packard, Monza, Italy)

Inosine and ADP were identified by multiple means: (a)

by determining the retention times and adding an internal standard; (b) by determining the ultraviolet (UV) spectra in the 210–350 nm range by diode array analysis with a Per-kin-Elmer 235C detector system in line; and (c) by acid hydrolysis of the presumed ADP peak and identification of the products ADP identification was confirmed by testing its ability to act as a substrate for pyruvate kinase (EC 2.7.1.40) [41] After incubation with pyruvate kinase, the HPLC chromatogram of the mixture revealed the disap-pearance of ADP and the formation of an ATP peak, which was identified in the chromatogram using the same criteria as for ADP and inosine

CE analysis All of the above compounds (nucleosides and nucleotides) were also analysed by CE using a Bio-Rad Biofocus 3000 apparatus equipped with a variable wavelength UV detec-tor The assays were performed in an uncoated silica capil-lary (40 cm· 75 lm) with the following operating conditions: 20 mm borate buffer, pH 10, 12 kV and 10 s; hydrostatic load at 25C; 254 nm The compounds were identified by comparing their retention times with those of known internal standards

Enzyme purification and identification by mass spectrometry

The AMP–AMP phosphotransferase reaction occurred only when two or three different enzymes were combined, which were purified according to the following procedures (Table 1)

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