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In the present study, we observed a significant decrease in the respiratory activity of rat liver mitochondria with aging, and an increase in the advanced gly-cation endproduct-modified pr

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rat liver mitochondrial matrix during aging

Maud Hamelin, Jean Mary, Michal Vostry, Bertrand Friguet* and Hilaire Bakala*

Laboratoire de Biologie et Biochimie Cellulaire du Vieillissement, Universite´ Paris 7-Denis Diderot, France

Aging is characterized by gradual deterioration of

cel-lular functions [1] associated with cumulative damage

to intracellular macromolecules, particularly proteins

[2] Several lines of evidence suggest that mitochondria

play a key role in aging, both by producing

intracellu-lar reactive oxygen species (ROS) and by being the

most adversely affected organelles during aging [3]

The electron transport chain located in the inner

mito-chondrial membrane is implicated in production of

ATP via oxidative phosphorylation, and is also known

to be the major intracellular site of free radical

genera-tion, such as that of superoxide anions and, sub-sequently, other potentially deleterious ROS [4] Numerous data have indicated an age-related increase

in the rate of mitochondrial free radical generation and in the extent of oxidative damage to mitochondrial macromolecules [5–9], especially enzymes involved in the respiratory chain, leading to impairment of respira-tory activity [7,9–12]

Although direct oxidation of proteins and other macromolecules is believed to be the main type of endogenous damage during aging [3,13,14], ROS can

Keywords

aging; glycation; liver mitochondria;

proteomics; urea cycle enzymes

Correspondence

H Bakala, Laboratoire de Biologie et

Biochimie Cellulaire du Vieillissement,

EA3106 ⁄ IFR117, Universite´ Paris 7-Denis

Diderot, 2 place Jussieu, 75251 Paris,

Cedex 05, France

Fax: +33 1 44 27 39 25

Tel: +33 1 44 27 82 27

E-mail: bakala@paris7.jussieu.fr

*These authors contributed equally to this

work

(Received 22 May 2007, revised 21

Septem-ber 2007, accepted 25 SeptemSeptem-ber 2007)

doi:10.1111/j.1742-4658.2007.06118.x

Aging is accompanied by gradual cellular dysfunction associated with an accumulation of damaged proteins, particularly via oxidative processes This cellular dysfunction has been attributed, at least in part, to impair-ment of mitochondrial function as this organelle is both a major source of oxidants and a target for their damaging effects, which can result in a reduction of energy production, thereby compromising cell function In the present study, we observed a significant decrease in the respiratory activity

of rat liver mitochondria with aging, and an increase in the advanced gly-cation endproduct-modified protein level in the mitochondrial matrix Wes-tern blot analysis of the glycated protein patWes-tern after 2D electrophoresis revealed that only a restricted set of proteins was modified Within this set,

we identified, by mass spectrometry, proteins connected with the urea cycle, and especially glutamate dehydrogenase, which is markedly modified in older animals Moreover, mitochondrial matrix extracts exhibited a signifi-cant decrease in glutamate dehydrogenase activity and altered allosteric regulation with age Therefore, the effect of the glycating agent methylgly-oxal on glutamate dehydrogenase activity and its allosteric regulation was analyzed The treated enzyme showed inactivation with time by altering both catalytic properties and allosteric regulation Altogether, these results showed that advanced glycation endproduct modifications selectively affect mitochondrial matrix proteins, particularly glutamate dehydrogenase, a crucial enzyme at the interface between tricarboxylic acid and urea cycles Thus, it is proposed that glycated glutamate dehydrogenase could be used

as a biomarker of cellular aging Furthermore, these results suggest a role for such intracellular glycation in age-related dysfunction of mitochondria

Abbreviations

AGE, advanced glycation endproduct; CEL, N-(carboxyethyl)lysine; CML, carboxymethyl-lysine; GDH, glutamate dehydrogenase; GO, glyoxal; MGO, methylglyoxal; OCT, ornithine carbamoyl transferase; RCR, respiratory control ratio; ROS, reactive oxygen species; TCA, tricarboxylic acid.

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also affect protein function through either

lipoperoxi-dative production of reactive aldehydes [15] or

glycoxi-dation pathways [16] Indeed, there is a causal

relationship between hyperglycemia-induced ROS

generation and intracellular advanced glycation

end-product (AGE) formation [17] This rise in AGE was

shown to be primarily if not exclusively due to a rapid

increase in AGE-forming methylglyoxal concentration

[18]

Alpha-dicarbonyl compounds such as glyoxal (GO)

and methylglyoxal (MGO) are physiological, highly

reactive intermediates involved in the Maillard reaction

[19] Interestingly, MGO may originate from various

biochemical pathways, including dephosphorylation of

glycolytic intermediates, metabolites of the polyol

pathway and metabolism of aminoacetones [20] At

physiological concentrations, MGO primarily targets

the arginine residues of proteins, leading primarily to

AGE-imidazolone [21,22] and -lysine residues to form

AGE adducts: N-(carboxyethyl)lysine (CEL) and

meth-ylglyoxal lysine dimer [23]

In a previous study, using an immunochemical

method, we showed that glycated proteins accumulate

in the rat liver mitochondrial matrix with aging [24]

In the present study, we show that glycation targets a

limited set of proteins, the most severely affected being

glutamate dehydrogenase, a crucial enzyme at the

interface between the tricarboxylic acid (TCA) and

urea cycles, indicating the interference of matrix

enzymes in mitochondrial impairment function with

aging

Results

Age-related changes in the respiratory function

of isolated mitochondria

Mitochondria were isolated from the livers of young

(3-month-old) and old (27-month-old) Wistar rats and

their respiratory parameters were measured As shown

in Table 1, there was a significant decrease in the rates

of mitochondrial oxygen consumption between 3-month-old and 27-month-old rats with gluta-mate⁄ malate or succinate as substrates The ADP ⁄ O ratios (P⁄ O) obtained whatever the substrate used indi-cated no change in coupling efficiency Although the respiratory control ratio (RCR) slightly decreased with glutamate⁄ malate substrate during aging, no change was observed using succinate In the presence of 2,4-dinitrophenol, oxygen consumption rates were the same in the presence of ADP (state 3) at each age Together, these results indicated age-related impair-ment of mitochondrial function

Determination of 6D12-detectable AGE content

in mitochondrial matrix proteins

We next investigated the levels of AGE-modified pro-teins to determine whether dysfunction of the organelle parallels the accumulation of modified mitochondrial matrix proteins For this purpose, we used monoclonal anti-AGE IgG (6D12) in a competitive ELISA using carboxymethyl-lysine (CML)-modified BSA as standard to evaluate AGE-modified protein content (Fig 1) AGE content increased significantly, by 48% from 12.01 ± 0.71 AUÆlg protein)1 (n¼ 8) in 3-month-old rats to 17.81 ± 1.83 AUÆlg protein)1 (n¼ 9) in 27-month-old rats (P < 0.01) These data indicated age-associated accumulation of AGE adducts

in mitochondrial matrix proteins

Identification of glycated mitochondrial matrix proteins by LC-MS⁄ MS after 2D gel

electrophoresis and western blotting 2D gel electrophoresis and western blotting Standard 2D gel electrophoresis of liver mitochondrial matrix proteins from young and old rats was run in parallel (Fig 2) Electrophoregrams showed a typical pattern of total protein, and more than a thousand

Table 1 Biochemical respiratory parameters in rat liver mitochondria from 3-month-old and 27-month-old rats Oxygen consumption rates were measured polarographically in the presence of either 10 m M succinate or 5 m M glutamate ⁄ 5 m M malate State 3 respiration was deter-mined after adding 310 nmol of ADP State 4 respiration is the rate of O 2 consumption after depletion of ADP Data represent the mean ± SEM n, number of animals *P < 0.05; **P < 0.01 versus 3-month-old rats.

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spots were detected using 2D Elite Master Software.

Western blot analysis using anti-AGE IgG (Fig 3) in

samples from both young and old rats revealed that

only a small number of proteins among the thousand

protein spots observed on gel stained with colloidal

Coomassie blue were targeted for glycation Moreover,

AGE-modified proteins were already present, although

to a lesser extent, in samples from young rats We

noted individual variations both in the nature of

modi-fied proteins and in their extent of labelling within

Fig 1 Determination of 6D12-detectable AGE protein content in

liver mitochondrial matrix with aging The AGE adduct content in

mitochondrial matrix proteins from 3-month-old and 27-month-old

rats was assayed by competitive ELISA using monoclonal anti-AGE

IgG (clone 6D12) The results are expressed as AUÆlg protein)1and

represent the mean ± SEM **P < 0.01 versus 3-month-old rats.

Number of animals is given in parentheses above the bar graphs.

A

250

-150

-100

-75

-50

-37

-25

-pI

250

150

100

75

50

37

25

pI

Fig 2 2D gel electrophoresis profile of liver

mitochondrial matrix proteins Liver

mito-chondrial matrix proteins (150 lg) were

sub-jected to isoelectrofocusing and subsequent

SDS ⁄ PAGE electrophoresis under reducing

conditions Gels containing samples from

(A) 3-month-old and (B) 27-month-old rats

were stained with colloidal Coomassie blue.

A

B

Fig 3 2D gel electrophoresis and western blotting analysis of liver mitochondrial matrix proteins; identification of AGE-modified pro-teins with aging Samples (150 lg) were subjected to isoelectrofo-cusing and subsequent SDS ⁄ PAGE under reducing conditions Gels were subjected to western blotting using monoclonal anti-AGE IgG (clone 6D12) to detect AGE-modified proteins in samples from (A) 3-month-old and (B) 27-month-old rats Matched proteins were identified by tandem LC-MS ⁄ MS mass spectrometry as: (1) gluta-mate dehydrogenase; (2) catalase; and (3) ornithine carbamoyl transferase.

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each age group Furthermore, we observed that

anio-nic isoforms appeared to be modified with age for

trails 1 and 3 (Fig 3B) Only spots from trails of

iso-forms numbered 1–3 that exhibited a significantly

increased yield of modifications with aging in a

repro-ducible way were retained for further analysis

(Fig 3A,B) and were matched with colloidal

Coomas-sie blue staining spots (Fig 2B), which were subjected

to LC-MS⁄ MS protein identification

Protein identification Proteins from selected spots were identified automati-cally by a computer program Three series of MS⁄ MS spectra were obtained (data not shown) At least two spots for each trail of isoforms were identified with sufficient peptide coverage (13–32%), and analysis led

to the characterization of three proteins in a narrow pI range (7.15–9.12), with a molecular mass in the range

Table 2 Identification of proteins located in spots 1–3 Tryptic peptides from in-gel digestion were subjected to LC ⁄ MS ⁄ MS analysis as described in the Experimental procedures Peptide identification was evaluated using Xcorr scores that measure similarities between mass-to-charge (m ⁄ z) ratios for fragment ions predicted from amino acid sequences and fragment ions observed in the MS ⁄ MS spectrum Xcorr, cross correlation scores; z, charge of the precursor ion; M*, oxidized methionine, C@, alkylated cysteine.

Spot No.

Protein UniProt

accession No.

Mass

dehydrogenase 1,

mitochondrial precursor,

P10860

K.KGFIGPGIDVPAPDM*STGER.E 212–231 2060.01 0.64 3 2.72 K.GFIGPGIDVPAPDM*STGER.E 213–231 1931.9 0.43 2 3.04

carbamoyltransferase,

mitochondrial, P00481

DM z Xcorr

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39.9–61.4 kDa (Table 2) The number of peptides

lead-ing to the identification of each protein varied between

six and 12 The proteins identified corresponded to

glutamate dehydrogenase (GDH) for trail 1, catalase

for trail 2 and ornithine carbamoyl transferase (OCT)

for trail 3 By contrast to catalase, a potent enzyme in

antioxidant defense, the other two enzymes (GDH and

OCT) involved in the mitochondrial urea cycle

exhib-ited a wide extent of modification at 27 months

compared to 3 months Further investigations were

performed with GDH because this enzyme is relevant

due to its essential role in between the urea and TCA

cycles

Age-associated increased glycation and

decreased activity of GDH

Using anti-GDH serum, we first checked by western

blotting that GDH content within the mitochondrial

matrix was unchanged with age (data not shown)

GDH was then immunoprecipitated from matrix

sam-ples and western blotting was carried out to investigate

the increased GDH glycation rate with aging (Fig 4)

Spots of GDH protein exhibited identical intensity in

3-month-old (49.86 ± 3.10 AU) and 23-month-old

rats (54.98 ± 2.39 AU), whereas AGE-labelling

inten-sity significantly increased by 45%, from 32.71 ±

2.65 AU (n¼ 4) in 3-month-old rats to 47.29 ±

3.67 AU (n¼ 3) in 23-month-old rats (P < 0.01)

(Fig 4B) The rate of glycation expressed as the ratio

of AGE⁄ GDH intensity was significantly increased by 30%, from 0.66 ± 0.05 (n¼ 4) in 3-month-old rats to 0.86 ± 0.03 (n¼ 3) in 23-month-old rats (P < 0.01) (Fig 4B) These data indicate that GDH underwent increased glycation with aging

We next examined whether the glycation modifica-tion demonstrated above was associated with impair-ment of GDH function For this purpose, we measured GDH activity in mitochondrial matrix extracts (50 lg of total protein) in the absence and presence of allosteric effectors (ADP and GTP) (Table 3) The results obtained revealed a significant decrease (23%) in GDH activity with aging, from 2.47 ± 0.19 UÆmg)1 in 3-month-old rats to 1.89 ± 0.07 UÆmg)1 in 23-month-old rats (n¼ 4) (P ¼ 0.02)

In the presence of the ADP activator, this activity was enhanced by 1.20-fold in young samples, although slightly less (1.14-fold) in old samples However, this GDH activity markedly collapsed with the GTP inhibi-tor, with residual activity barely representing 8% in young samples compared to 12% in old samples (P < 0.01) These data suggest an inhibitory effect of glycation on GDH activity, in addition to a slightly altered response to allosteric regulation with aging

Effect of MGO on purified GDH activity and its allosteric regulation

To ascertain whether the glycation impact upon GDH activity was due to modifications in lysine⁄ arginine

A

B

Fig 4 Immunochemical identification of glycated GDH from mitochondrial matrix GDH from matrix samples (500 lg) was

immunoprecipitat-ed and samples resolvimmunoprecipitat-ed by SDS ⁄ PAGE electrophoresis Two identical gels from each sample run in parallel were subjected to western blotting using either monoclonal anti-AGE IgG or GDH polyclonal antibody to detect AGE modifications or GDH antigen content, respectively,

in samples from 3-month-old and 23-month-old rats (A) Blots were semiquantified by densitometry scanning and density expressed in arbitrary units (AU) Results are presented as the ratio of the AGE ⁄ GDH rate (B) Values are the means ± SEM **P < 0.01 versus 3-month-old rats.

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residues, we used MGO as a potent glycating agent

generated intracellularly to investigate the effect on

purified GDH

In vitro MGO modification of GDH

A purified GDH sample (10 lg) was incubated with

varying concentrations of MGO for up to 24 h and

subjected to western blotting (Fig 5) with monoclonal

anti-AGE IgG or polyclonal anti-GDH serum After

24 h of incubation, the GDH protein was

MGO-derived AGE-modified and the extent of modification

increased with the concentration of MGO (Fig 5A),

whereas the GDH immunolabelling intensity

concur-rently decreased (Fig 5B) These results clearly

demon-strated that glycated enzyme partially lost its

antigenicity, indicating that some antigenic sites of

GDH could be masked by glycation adducts

Effect of MGO on GDH activity and kinetic parameters

In vitro MGO treatment of purified GDH resulted in decreased activity with time of incubation and an increasing MGO concentration (Table 4) With 1 mm MGO, GDH activity significantly decreased at 30 min

of incubation, and markedly dropped by 37% within

5 h compared to control This MGO effect on GDH activity was compared with that induced by GO, an a-dicarbonyl metabolite presumed to possess noxious specificity like MGO Treatment with GO led to a sig-nificant inhibition effect with as little as 50 lm (38%), whereas a high concentration (1 mm) led to strong inhibition (67%), providing evidence that GDH activ-ity is altered by glycation modification Using amino acid analysis, we noted that both arginine (24 : 30) and lysine (22 : 32) residues were damaged in MGO-treated GDH, whereas 22 and eight residues, respectively, were damaged with GO, indicating that MGO was equally noxious to lysine and arginine residues, leading to the loss of part of its activity In addition, analysis of enzymatic parameters (Vmax and

Km) of GDH modified by 1 mm MGO for 24 h using a-ketoglutarate as substrate (Fig 6) showed that MGO treatment altered only the maximum velocity (Vmax), whereas the apparent Kmvalue did not change

Effect of MGO on GDH allosteric regulation

We next analyzed the allosteric regulator effects on purified GDH activity before and after treatment with MGO At indicated times, the activity of the enzyme incubated with 1 mm MGO was measured in the pres-ence of constant concentrations of allosteric effectors (activators: 250 lm ADP or 10 mm leucine; inhibitor:

30 lm GTP) GDH activity at t0 in the absence of ef-fectors (28.6 UÆmg enzyme)1) was set at 100% activity

As shown in Fig 7, GDH activity spontaneously decreased with time of incubation This decline was accentuated when GDH was incubated with 1 mm MGO (Fig 7A) and the activity ratio shifted from 0.85-fold to 0.71-fold at t240 versus t0 In the presence

of an ADP effector (Fig 7B), native GDH activity exhibited a significant 1.66-fold increase (Fig 7B, left panel), whereas this increase fell to 1.44-fold when GDH was preincubated with 1 mm MGO (Fig 7B, right panel) With leucine as an effector, we observed a similar phenomenon (Fig 7C); the activity increased

by 1.55-fold at 240 min with native enzyme (Fig 7B, left panel) and by 1.24-fold when the enzyme was preincubated with MGO (Fig 7B, right panel) These

Table 3 Age-related changes in GDH activity and allosteric effector

sensitivity Liver mitochondrial matrix extracts (50 lg total protein)

from young (3-month-old) and old (23-month-old) rats were

sub-jected to the GDH activity assay in the direction of a-ketoglutarate

amination, in the absence (control) or presence of constant

concen-trations of allosteric effectors (activator: 250 l M ADP; inhibitor:

10 l M GTP) Values are expressed as specific activity (UÆmg)1)

(control) and activity determined in the presence of effectors is

given as a percentage of control in the absence of effectors, at

each age Data represented the mean ± SEM (n ¼ 4) *P ¼ 0.02;

**P < 0.01 versus 3-month-old rats.

GDH activity

Age

3 months (n ¼ 4)

23 months (n ¼ 4) GDH activity (no effector) 2.47 ± 0.19 1.89 ± 0.07*

% of GDH activity (+ ADP) 120.88 ± 4.75 114.71 ± 16.71

% of GDH activity (+ GTP) 8.23 ± 0.75 11.57 ± 0.59**

4 3 2

1

E

G

A

A

4 3 2 1 H D G

B

Fig 5 Western blot detection of purified GDH modified by MGO.

Purified GDH (Sigma) samples (10 lg) were incubated with or

with-out varying MGO concentrations for 5 h and aliquots (1 lg) were

subjected to SDS ⁄ PAGE under reducing conditions Gels were

sub-mitted to western blotting using either monoclonal anti-AGE IgG

(clone 6D12) to detect MGO-derived AGE in GDH samples (A) or

GDH polyclonal antibody to determine whether the GDH load was

preserved (B) Lane 1, control, 0 l M MGO; lane 2, 50 l M MGO;

lane 3, 200 l M MGO; lane 4, 1 m M MGO.

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results indicate that GDH stimulation induced by

allo-steric effectors was partly abolished when the enzyme

was previously treated with MGO On the other hand,

the GTP inhibitor (Fig 7D) exhibited an efficient

effect on GDH activity; the activity, which was barely

0.44-fold at 240 min (Fig 7D, left panel), rose to

0.71-fold when GDH was pretreated with MGO (Fig 7D,

right panel), indicating the capacity of MGO to

abro-gate the inhibitory effect of GTP on GDH activity

Together, these results clearly demonstrate the

inhibi-tory effect of MGO on GDH activity, which

rein-forced the decline of its activity with time In addition,

this MGO modification deeply altered the

responsive-ness of GDH to its respective allosteric effectors, in

agreement with data observed in vivo

Discussion

In the present study, we have demonstrated a decline in mitochondrial respiratory chain activity upon aging concomitant with an accumulation of AGE-modified proteins in the mitochondrial matrix These modifica-tions affect several proteins that are targets for glyca-tion damage and, although some of these proteins are already modified at a young age (3-month-old rats), the level of damage significantly increased with increasing age (27-month-old rats) Among the glycated proteins, trails of isoforms appeared, in agreement with modifi-cations targeting the basic amino acids arginine and lysine, subsequently turning off their ionic charge In support of this assertion, recent studies have shown that glycation was associated with both loss of basic groups and shifts in pK of the acidic groups, consistent with a reduction in effective anionic charge [23,25] The identification of these proteins, which are increasingly glycated with aging, revealed three enzymes, GDH, OCT and catalase Catalase, a crucial antioxidant defense enzyme highly expressed in peroxisomes, and also constitutively present in the heart and liver mito-chondria [26–28], is maintained with GDH and OCT, which belong to the urea cycle GDH, in particular, which markedly emerged as being modified at

27 months, plays a key role in connecting TCA to the urea cycles, and exhibited a loss of activity and altera-tions in its allosteric properties with aging Interest-ingly, all these modified proteins are different from those preferentially found to be oxidized during aging

in the mitochondrial matrix, as previously identified (i.e aconitase [29] and adenine nucleotide translocase [30]) To determine whether the age-related inhibition

of GDH activity demonstrated here was related to glycation modification, purified GDH was treated with the a-dicarbonyl metabolite MGO Incubation of GDH with this compound resulted in time-dependent inacti-vation of the enzyme, consistent with the damaging

Table 4 Effect of MGO and GO concentrations on GDH activity Purified GDH samples (10 lg) were incubated in 100 m M TEA-HCl buffer

pH 7.3 with varying concentrations (0.05, 0.200 and 1 m M ) of either MGO or GO as glycating agents for variable times At indicated times, enzyme activity was determined spectrophotometrically on aliquots (1 lg) Data are expressed as a percentage of GDH activity control at t0for each concentration and represent the mean ± SEM (n, number of animals) *P < 0.05; **P < 0.01; ***P < 0.001 traited versus control at t0.

MGO [m M ]

GDH activity (n ¼ 4) Incubation time

GO [m M ]

Incubation time

Fig 6 Effect of MGO modification on GDH parameters Vmaxand

K m Kinetic analyses were performed to determine the effect of

MGO (1 m M ) on GDH enzymatic parameters V max and K m Purified

GDH (Sigma) samples (10 lg) were incubated with or without

MGO for 24 h and aliquots (1 lg) were taken for enzymatic assay.

Lineweaver–Burk plots were used with a-ketoglutarate as substrate

(S) in native (1) or MGO-treated (2) GDH.

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effect of glycation Interestingly, kinetic analysis of modified GDH showed that treatment with MGO reduced only the maximum velocity without affecting

Km, indicating that MGO-modified GDH is inacti-vated In addition, amino acid analysis performed on MGO- and GO-treated GDH revealed that lysine resi-dues were more sensitive to MGO than to GO modifi-cations, whereas arginine was equally sensitive to both dicarbonyl compounds, suggesting that GDH inactiva-tion was at least partly due to MGO-lysine⁄ arginine modifications, in accordance with the observed trails of isoforms in the 2D electrophoregram Indeed, numer-ous data have claimed that MGO modifications of criti-cal arginine⁄ lysine residues cause structural distortion, leading to enzyme inactivation [23,31] In addition, recent data on GDH studies indicate that among

33 lysine residues constitutive of its primary sequence, the prominent lysine 126 directly interacts with the a-carbon constituent on the substrate [32], suggesting that MGO modification affects enzymatic activity In support of this assertion, a lysine residue involved in inactivation of brain GDH isoproteins by O-phthal-aldehyde has been identified [33] Moreover, loss of GDH activity was reported under multiple system atro-phy conditions in which GDH activity was decreased

to a greater extent than other mitochondrial enzymes [34] indicating that GDH is more sensitive to insults Interestingly, a recent in vitro study showed that incu-bation of mitochondria with MGO led to rapid inhibi-tion of mitochondrial respiratory rates through particular protein target modifications [35] Both the TCA cycle and the electron respiratory chain were inhibited, indicating a link between mitochondrial MGO modified enzymes and altered function Mamma-lian GDH is allosterically regulated by a number of small molecules [36] and its regulation is of particular biological importance, as exemplified by the observa-tion that some regulatory mutaobserva-tions of the gene for GDH are associated with severe clinical manifestation

in children [32] In addition, as shown in a recent study

Control

0

20

40

60

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140

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180

0 1 MGO (m M )

0 15 120 240

ADP 250 µM

0

20

40

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Leu 10 mM

0

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GTP 30 µM

0

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B

C

D

A

1

1

1

** **

****

***

# # #

# # #

**

**

# # #

# # # #

****

****

# # # #

# # # #

# # # #

# # # #

# # #

# # #

Fig 7 MGO effect on GDH activity and allosteric regulation Puri-fied GDH samples (10 lg; Sigma) was incubated with 1 m M MGO for up to 240 min and aliquots (1 lg) were subjected to the GDH activity assay in the absence (A) or presence of constant concentra-tions of allosteric activators ADP (B) and leucine (C), or inhibitor GTP (D) Values expressed as specific activity (UÆmg)1) are given as percentage of control (activity determined in the absence or pres-ence of MGO at t 0 ; left and right panels, respectively) in each range Data represented the mean ± SEM (n ¼ 6) *t-test: effector versus control at each time; #t-test: MGO 1 m M -treated versus nontreated MGO at each time Equivalent to * or #; P < 0.05;

P < 0.01; P < 0.005; P < 0.001.

Trang 9

[37], the arginine side chain at position 463 of GDH is

thought to be involved in ADP allosteric activation

because the R463A mutant form of this enzyme is

insensitive to ADP stimulation In the present study,

MGO modifications altered the allosteric regulation

properties of the enzyme, suggesting that these effects

are not only due to a change in charge profile, but also

in the conformation of the molecule resulting from

gly-cation of the charged arginine⁄ lysine side-chain

resi-dues The fact that arginine⁄ lysine residues are targets

of glycation [38,39] suggests that the modifications

observed in the present study involve some of these

res-idues, leading to an impairment of allosteric regulation

and the catalytic properties of the enzyme

GDH is important in converting free ammonia and

a-ketoglutatrate to glutamate; it utilizes nicotinamide

nucleotide cofactor NAD+for nitrogen liberation and

NADP+ for nitrogen incorporation; however, it

should be recognized that the reverse reaction is a key

anapleurotic process linking amino acid metabolism

with TCA cycle activity In a reverse reaction, GDH

provides an oxidizable carbon source for the

produc-tion of energy, as well as the reduced electron carrier

NADH Thus, GDH is considered to be significant not

only because it catalyzes a reaction directly connected

to the TCA cycle, but also because of the pivotal

posi-tion in metabolism occupied by both glutamate and

a-ketoglutarate as a result of their ability to enter into

many metabolic pathways [40] Accordingly, the

build-up of an inactive form of GDH demonstrated in the

present study could contribute to a decreased

produc-tion of a-ketoglutarate and a diminished flux through

the TCA cycle, which might be at least partly be

responsible for impairment of mitochondrial function

with advanced age, as demonstrated by the decrease in

respiration driven by the glutamate–malate substrate

In summary, the results obtained in the present study

demonstrate that age-related impairment of

mitochon-drial respiration runs parallel to an accumulation of

AGE-modified matrix proteins Identification of

selec-tively glycated proteins revealed that two of these are

key urea cycle enzymes, among which GDH was the

main target protein and showed a loss of both activity

and sensitivity to allosteric effectors with aging In vitro

alterations in both allosteric regulation and catalytic

properties of this enzyme by the glycating agent MGO

during short-term incubation support the notion of the

dysfunctional power of intracellular glycation In line

with the central role played by this enzyme in cellular

metabolism and energy homeostasis, we hypothesize

that AGE modifications of GDH may contribute, at

least in part, to a defect in mitochondria with aging and

could be used as a biomarker of cellular aging

Experimental procedures

Animals

Experiments were performed on male Wistar rats (WAG⁄ Rij) born and raised in the animal care facilities of the Commissar-iat a` l’Energie Atomique (CEA, Gif-sur-Yvette, France) This strain remains lean even when fed ad libitum and does not suffer from age-associated nephropathy, hypertension or diabetes [41] Cohorts were constituted of young adult (3-month-old) and senescent (27-month-old) animals All stu-dies were conducted in accordance with the animal care policy

of national and European regulations

Chemicals

GDH (bovine liver; EC 1.4.1.3) and horseradish peroxidase-conjugated anti-(mouse IgG) or anti-(rabbit IgG) sera were purchased from Sigma Chemicals (Saint Quentin Fallavier, France) GDH was dissolved in 100 mm of triethanolamine,

pH 7.3 Monoclonal antibody to AGE (clone no 6D12) from Trans Genic Inc (Kumamoto, Japan) shows cross-reaction both to CEL and CML [42] Polyclonal antibody against GDH was obtained from Interchim (Montluc¸on, France) and pro-tein G-agarose bed (ImmunoPure immobilized propro-teinG Plus) from Pierce (Perbio Science Company, Brebie`res, France)

Isolation of mitochondria

A 10% (w⁄ v) tissue homogenate was prepared using a Pot-ter apparatus in an ice-cold medium containing 220 mm mannitol, 70 mm sucrose, 0.1 mm EDTA and 2 mm Hepes,

pH 7.4, supplemented with 0.5% BSA (w⁄ v) Nuclei and cellular debris were pelleted by centrifugation for 10 min at

800 g and 4C Supernatant was centrifuged at 8000 g for

10 min at 4C The mitochondrial pellet was then washed three times with the homogenization medium and used for polarographic measurements

To prepare mitochondrial matrix extract, mitochondria were suspended in 50 mm Tris⁄ HCl, pH 7.9, then dis-rupted by sonication (four times for 10 s) The resulting suspension was centrifuged at 15 000 g for 10 min and then at 100 000 g for 45 min at 4C The supernatant (containing matrix proteins) was stored at )80 C for fur-ther analysis of AGE-modified proteins The protein con-centration was assessed using a Bradford protein assay (Biorad, Mu¨nchen, Germany)

To estimate contamination of mitochondrial preparation with lysosomes, we used acid phosphatase activity as a marker

Measurements of mitochondrial respiration

Oxygen consumption was measured polarographically with

a Clark electrode in the sample, as described by Aprille

Trang 10

and Asimakis [43], in a thermostatically controlled closed

2 mL chamber (30C) The rate of oxygen consumption

was measured in the presence of 310 nmol of ADP and

10 mm of succinate or 5 mm glutamate⁄ 5 mm malate

(state 3) and after all ADP had been consumed (state 4 or

resting state) Oxygen consumption rates are expressed as

ng atoms of oxygen consumedÆmin)1Æmg protein)1 The

rate of oxygen consumption in state 3 and in state 4,

RCR (the ratio of state 3 to state 4 respiration), an index

of electron transport chain activity, and the ADP⁄ O2ratio

were calculated Oxygen consumption in the presence of

40 lm of dinitrophenol (uncoupled state) was also

checked

Determination of 6D12-detectable AGE content in

mitochondrial matrix proteins by competitive

ELISA

The ELISA assay was conducted as previously described

[24] Briefly a 96-well microtiter Nunc-immuno plate

(Nunc, Roskilde Denmark) was coated with 100 lL of

CML-BSA (6.4 nmol CMLÆmL)1) by incubation overnight

at 4C Wells were washed with NaCl⁄ Pi)0.05%

Tween 20 (v⁄ v) (buffer A) and free binding sites were

blocked by incubation for 1 h at room temperature with

100 lL of NaCl⁄ Pi)6% skimmed milk or NaCl ⁄ Pi)1%

BSA (w⁄ v) After washing with buffer A, 50 lL of

com-peting antigen (test samples at 0.1 lgÆlL)1 or serial

dilu-tions of standard CML-BSA from 0.64 mm to 128 mm)

was added, followed by 50 lL of monoclonal anti-AGE

IgG (clone 6D12) (dilution 1 : 3000) The plate was

incu-bated for 2 h at room temperature, washed and then

incubated with 50 lL horseradish peroxidase-conjugated

anti-(mouse IgG) (dilution 1 : 10 000) for 2 h at room

temperature The wells were washed, then 100 lL of

sub-strate solution (40 mm ABTS and 200 lL of 30%

hydro-gen peroxide in 20 mL sodium acetate-phosphate buffer,

pH 7.2) were added per well and incubated Absorbance

(A) was measured at 405 nm on a micro-ELISA plate

reader (Spectra Rainbow, SLT Labinstruments, Salzburg,

Austria) Results are expressed as the ratio B⁄ Bo (bound ⁄

total), calculated as: experimental A) background A (no

antibody)⁄ total A (no competitor)) background A, versus

CML added, as pmol CMLÆlg protein)1 Finally, data

were expressed as arbitrary unitsÆlg protein)1 (AUÆlg

pro-tein)1) because anti-AGE IgG recognizes both CML and

CEL

The use of 6% skimmed milk (w⁄ v) as an alternative to

1% BSA (w⁄ v) as a blocking agent in the immunochemical

assay introduced an increment of less than 10% at the

CML level, with GO-modified BSA used as standard (data

not shown) We took advantage of these results and used

skimmed milk in all further immunochemical assays

(ELISA and western blotting)

1D and 2D gel electrophoresis of mitochondrial matrix proteins and western blotting

Mitochondrial matrix protein samples (150 lg) from 3-month-old and 27-3-month-old rats were mixed with 200 lL

of 2D sample buffer (7 m urea, 2 m thiourea, 4% Chaps, 1% dithithreitol, 2% Pharmalytes, Amersham Biosciences, Saclay, France; pH 3.0–10.0) The strips were allowed to rehydrate overnight 1D isoelectric focusing was performed

on Immobiline Drystrips (Amersham Biosciences; pH 3.0– 10.0, 13 cm) in a Multiphor II device (Amersham Bio-sciences) for 49 325 Vh After electrofocusing, immobilines were prepared for SDS⁄ PAGE and 2D SDS ⁄ PAGE was run vertically on a 12% polyacrylamide gel using the cool-ing Protean II system (Bio-Rad, Marne La-Coquette, France) The gels were either fixed and stained with colloi-dal Coomassie blue for total protein pattern and

LC-MS⁄ MS analysis, or western blotted onto a nitrocellulose membrane (Bio-Rad) overnight at 30 V The membrane was saturated with NaCl⁄ Pi, pH 7.4, 0.1% Tween 20 (v⁄ v), 5% skimmed milk (w⁄ v) overnight at 4 C, followed by four washes (10 min each) with NaCl⁄ Pi, pH 7.4, 0.2% Tween 20 (washing buffer) The membrane was then incu-bated for 2 h at room temperature with monoclonal anti-AGE IgG clone 6D12 (dilution 1 : 3000) in NaCl⁄ Pi, 0.1% Tween 20 (v⁄ v), washed four times, incubated for 1 h with anti-(mouse IgG) coupled to horseradish peroxidase (dilu-tion 1 : 3000) and given a final wash The proteins were revealed with a SuperSignal West Pico chemiluminescent reagent (Perbio Science Company, Brebie`res, France)

Protein identification by LC-MS⁄ MS

Colloidal Coomassie blue-stained spots matching with bands immunolabelled by monoclonal anti-AGE IgG were excised from gel, cut into 1 mm pieces and then treated for LC-MS⁄ MS analysis Gel pieces were washed twice in

100 mm ammonium bicarbonate buffer pH 8.8 and then dehydrated with acetonitrile The gel pieces were rehydrated

in 10 mm dithithreitol⁄ ammonium bicarbonate solution and proteins alkylated with 50 mm iodoacetamide After dehy-dratation with acetonitrile, gel pieces were rehydrated on ice for 10 min in 20 lL of 20 mm ammonium bicarbonate containing 50 ngÆlL)1 of sequence-grade modified porcine trypsin (Promega, Madison, WI, USA); then supernatants were replaced by 20 lL of 20 mm ammonium bicarbonate, and in-gel digestion was performed for 15 h at 37C The resulting peptides were extracted twice with 20 lL of

20 mm ammonium bicarbonate and then three times in

20 lL of 0.5% trifluoroacetic acid in 50% acetonitrile The peptide extracts were concentrated to 20 lL using an RC 10.22 evaporator concentrator (Jouan, Saint Herblain, France) Samples were then subjected to mass spectrometry analysis

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