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Hypothalamic Keywords Cre ⁄ loxP system; dopaminergic cells; dopamine transporter; gene expression; transgenic mice Correspondence F.. We generated a transgenic mouse line expressing the

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pattern ) generation of DAT-iCre transgenic mice

Marc Turiault1,*, Se´bastien Parnaudeau1,*, Aude Milet1, Rosanna Parlato2, Jean-Denis Rouzeau1, Monique Lazar1 and Franc¸ois Tronche1

1 CNRS UMR7148, Molecular Genetics, Neurophysiology and Behavior, Colle`ge de France, Institut de Biologie, Paris, France

2 Molecular Biology of the Cell I, German Cancer Research Center, Heidelberg, Germany

Dopamine-synthesizing neurons modulate many

biolo-gical functions in a dynamic manner In the

mamma-lian brain, dopamine neurons are distributed as groups

of cells in the ventral midbrain area (A8, A9, A10),

diencephalon (A11–A15), olfactory bulb (A16) and

retina (A17) Dopaminergic systems are involved in the

regulation of motor and motivational control,

cardio-vascular and respiratory activities via ascending and descending projections that are widely distributed in the mammalian brain and spinal cord [1,2] In the ventral midbrain, the A9 nucleus (substantia nigra) has several functions, including the modulation of motor control, and the A10 nucleus (ventral tegmental area)

is involved in reward mechanisms Hypothalamic

Keywords

Cre ⁄ loxP system; dopaminergic cells;

dopamine transporter; gene expression;

transgenic mice

Correspondence

F Tronche, UMR7148 CNRS, 11 place

Marcelin Berthelot, 75005 Paris, France

Fax: +33 1 44 27 13 22

Tel: +33 1 44 27 13 08

E-mail: francois.tronche@gmail.com

*These authors contributed equally to this

work

(Received 16 February 2007, revised

10 May 2007, accepted 16 May 2007)

doi:10.1111/j.1742-4658.2007.05886.x

The dopamine transporter is an essential component of the dopaminergic synapse It is located in the presynaptic neurons and regulates extracellular dopamine levels We generated a transgenic mouse line expressing the Cre recombinase under the control of the regulatory elements of the dopamine transporter gene, for investigations of gene function in dopaminergic neu-rons The codon-improved Cre recombinase (iCre) gene was inserted into the dopamine transporter gene on a bacterial artificial chromosome The pattern of expression of the bacterial artificial chromosome–dopamine transporter–iCre transgene was similar to that of the endogenous dopamine transporter gene, as shown by immunohistochemistry Recombinase activ-ity was further studied in mice carrying both the bacterial artificial chromo-some–dopamine transporter–iCre transgene and a construct expressing the b-galactosidase gene after Cre-mediated recombination In situ studies showed that b-galactosidase (5-bromo-4-chloroindol-3-yl b-d-galactoside staining) and the dopamine transporter (immunofluorescence) had identical distributions in the ventral midbrain We used this animal model to study the distribution of dopamine transporter gene expression in hypothalamic nuclei in detail The expression profile of tyrosine hydroxylase (an enzyme required for dopamine synthesis) was broader than that of b-galactosidase

in A12 to A15 Thus, only a fraction of neurons synthesizing dopamine expressed the dopamine transporter gene The bacterial artificial chromo-some–dopamine transporter–iCre transgenic line is a unique tool for target-ing Cre⁄ loxP-mediated DNA recombination to dopamine neurons for studies of gene function or for labeling living cells, following the crossing

of these mice with transgenic Cre reporter lines producing fluorescent proteins

Abbreviations

BAC, bacterial artificial chromosome; DAT, dopamine transporter; GFP, green fluorescent protein; iCre, codon-improved Cre recombinase; IRES, internal ribosome entry site; TH, tyrosine hydroxylase; X-Gal, 5-bromo-4-chloroindol-3-yl b- D -galactoside staining.

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dopamine cells form five groups (A11–A15) A11

neu-rons from the posterior hypothalamus project into the

spinal cord A13 neurons from the zona incerta project

locally into the hypothalamus and are engaged in

gonadotropin-releasing hormone neuron control In

the arcuate nucleus (A12 group) and the preoptic

area⁄ anterior hypothalamus (A14 group), most of the

dopamine neurons are endocrine neurons, secreting

dopamine into the portal blood system) which bathes

the anterior lobe of the pituitary gland) or directly

into the median pituitary lobe These neurons control

prolactin secretion and growth hormone secretion from

the anterior pituitary gland and

melanocyte-stimula-ting hormone secretion from the intermediate lobe

Dopaminergic neurons are able to synthesize

dop-amine because they contain the rate-limiting enzyme

tyrosine hydroxylase (TH) and the widely expressed

3,4-dihydroxyphenylalanine decarboxylase Some

sub-sets of dopamine neurons express other specific genes,

such as that encoding the dopamine 2 receptor, which

functions as an autoreceptor, or that encoding the

dopamine transporter (DAT), an essential regulator of

extracellular dopamine levels in the synaptic cleft

Dopamine system dysfunction is associated with

sev-eral diseases of the motor and limbic systems A loss

of dopamine neurons is one of the characteristics of

Parkinson’s disease, whereas changes in the activity or

function of dopaminergic nuclei are associated with

depression, schizophrenia and addiction to drugs of

abuse [3–7]

The use of the Cre DNA recombinase allows

target-ing of DNA recombination events to desired cell

popu-lations Efforts have recently been made to generate

transgenic mice expressing the recombinase in restricted

neuronal populations [8] We generated a transgenic

mouse line [bacterial artificial chromosome (BAC)–

DATiCrefto, hereafter referred to as BAC-DATiCre]

(iCre is codon-improved Cre recombinase) in which

Cre is specifically expressed in DAT-containing

neurons, as a molecular genetic tool for the

investiga-tion of dopamine cell funcinvestiga-tion When crossed with

reporter mice expressing b-galactosidase after

recombi-nation (R26R mice [9]), the BAC-DATiCre line

dis-played irreversible labeling of all cells that were

expressing or had expressed the DAT gene We found

that some subsets of neurons from the A12 to A15

regions never coexpressed the TH and DAT genes,

whereas all TH-positive neurons from the A8 to A10

regions were recombined

Used in combination with other conditional alleles

containing loxP sites, the BAC-DATiCre transgenic

line will be an invaluable tool with which to analyze

dopamine neuron biology and dopamine presynaptic

alterations in physiopathologic disorders involving dopaminergic systems

Results

Generation of the DATiCre transgenic line

We used a large (177 kb) DNA genomic segment from the C57BL⁄ 6 mouse strain in a BAC clone encompas-sing the entire mouse DAT (slc6) gene to ensure that the iCre transgene [10] was correctly expressed (Fig 1A) We generated the transgene by recombina-tion in bacteria, using the tools developed by Lee et al [11]

The final construct encompassed 97 kb of DNA upstream from the DAT gene start codon and 38 kb

of DNA downstream from the polyadenylation signal sequence of the gene The homology arms of the BAC targeting vector used for recombination in bacteria were chosen such that the ATG of the iCre was in the same position as the ATG of the DAT gene (Fig 1A) Following recombination and elimination of the selec-tion cassette, bacterial colonies were screened and the modified BAC was analyzed for the desired recombina-tion event by restricrecombina-tion enzyme analysis A 177 kb DNA fragment was excised by digestion with PmeI and AscI restriction enzymes, purified and injected into mouse FVB⁄ N zygotes

We obtained two transgenic lines In both lines, the Cre recombinase protein was produced in the ventral tegmental area and the substantia nigra, as shown

by immunohistochemistry with an antibody directed against Cre [12] Immunostaining for Cre was com-pared with that for TH, on successive serial sections of the mesencephalon, and a perfect match was found Cre expression in the ventral midbrain was restricted to dopamine neurons of the A9 and A10 nuclei (Fig 1B)

Cre-mediated recombination pattern

We investigated the distribution of Cre expression and the DNA recombination pattern in the transgenic mouse line, by analyzing one transgenic line in detail (BAC-DATiCre line), and crossing this line with the R26R reporter line [9] In transgenic animals carrying both the R26R and BAC-DATiCre transgenes, the Cre recombinase catalyzed the removal of a DNA sequence, leading to b-galactosidase production A detailed analysis of serial sections stained with 5-bromo-4-chloroindol-3-yl b-d-galactoside (X-Gal) and for TH clearly showed that recombination was restricted, in the mesencephalon, to dopaminergic structures (Fig 1C)

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We investigated the pattern of Cre expression

fur-ther, by carrying out double staining on sections of the

ventral tegmental area and the substantia nigra Using

a combination of fluorescent secondary antibodies and

primary antibodies directed against TH and Cre, we

found that Cre (red) was restricted to the nuclei of

TH-expressing neurons (Fig 2A)

We used TH immunostaining to identify cells

con-taining dopamine, because DAT labeling of the cell

body is very weak Nevertheless, the labeling of mid-brain sections for both DAT and Cre showed that all neurons producing the Cre recombinase (red) also pro-duced DAT protein (green, Fig 2B) Similarly, when analyzed at the cellular level, we showed that DNA recombination was restricted to DAT-expressing neu-rons In mice carrying both the iCre and the R26R transgenes, b-galactosidase expression (green) was con-fined to the domain containing DAT neurons (red)

A

Fig 1 Expression of Cre recombinase in the midbrain dopaminergic nuclei of DATiCre mice (A) Schematic representation of the BAC-DATiCre construct and its structure The position of the DAT gene within the RP23–408F13 BAC is indicated, together with those of the putative genes contained within this BAC The exon ⁄ intron structure of the DAT gene is shown The DAT gene has 14 exons (rectangles) The recombination targeted the second DAT exon, inserting the Cre transgene at the level of the ATG of the DAT gene The iCre ORF is rep-resented as a white rectangle, whereas intron sequences and the polyadenylation signal of the bovine GH gene are shown as a dark gray rectangle The ampicillin resistance gene (light gray rectangle), flanked by FLP recombinase target sites (black half-circles), was removed from the final construct by flp-mediated recombination from the final construct (lowest representation) (B) The Cre recombinase is expressed in TH-positive neurons from the mesencephalon Serial sections labeled by immunohistochemistry, using antibodies directed against TH (left panel) and Cre (right panel) proteins (C) DNA recombination occurs in TH-positive neurons from the mesencephalon Serial sections showing the distribution of TH-positive neurons (left) and X-Gal staining indicating Cre-mediated recombination of a LacZ-expressing reporter gene (right) Magnification: scale bars ¼ 400 lm.

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in the A10 region of the mesencephalon (Fig 2C).

b-galactosidase expression was clearly restricted to

DAT-positive neurons (Fig 2D)

We used the same approach for systematic analyses

of the DNA recombination pattern in groups of

dop-aminergic cells, from the mesencephalon to olfactory

bulbs and retina in serial sections labeled

immunohis-tochemically for TH and stained for b-galactosidase

activity with X-Gal substrate In the retrorubral field

(A8), substantia nigra (A9), ventral tegmental area

(A10) and glomerular layer of the olfactory bulb

(A16), the number of recombination events was similar

to the number of TH-expressing cells in serial fields

(Fig 3) In contrast, in hypothalamic groups of cells,

the overlap was only partial between recombination

and TH expression Whereas in the periventricular

organ (A11) and the cell group of the dorsomedial and

lateral arcuate nucleus (A12) the number of

recom-bined cells was similar to the number of TH positive cells, we did not detect b-galactosidase activity in the ventromedial region of the arcuate nucleus In the zona incerta (A13) and periaqueductal gray area, recombination events appeared to be restricted to a minority of TH-positive neurons No X-Gal staining was detected in A14 and A15 nuclei X-Gal staining of retina slices showed some recombination events (data not shown)

Behavioral analysis of BAC-DATiCre mice The BAC-DATiCre line was, in part, used to study behavioral consequences of mutations targeted to dop-aminergic neurons It was therefore essential to verify that the presence of the BAC-DATiCre transgene does not alter behavior During the generation of the BAC-DATiCre line, we minimized the risks of any

A

B

C

D

Fig 2 In the midbrain of BAC-DATiCre mice, both Cre expression and recombination are restricted to dopamine neurons Confocal images from multiple staining of double-transgenic mouse brain coronal sections (30 lm) (A) Immunofluorescence of TH (green) and Cre (red) in midbrain dopamine neurons DNA was stained with TO-PRO  3 iodide (TOPRO) The left panel is an overlay of the three previous panels (B) As for (A), except that the green staining corresponds to the DAT protein (C) Cre-mediated recombination visualized by immunofluorescence staining, using antibodies against b-galactosidase (green) (D) Low-power magnification showing that Cre-mediated recombination corresponds to DAT-expressing neurons (red), using a higher magnification All nuclei of this region are stained with TOPRO Magnification: scale bars ¼ 50 lm.

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perturbations that could arise from undesired

expres-sion of gene products encoded by the BAC transgene,

by designing a transgene from which DAT proteins

should not be produced To rule out the existence of

any problem, we performed comparative behavioral

tests in BAC-DATiCre males (n¼ 11) and their

con-trol littermates (n¼ 11) maintained on an FVB ⁄ N

genetic background The general appearance of

trans-genic and control littermates was undistinguishable

The weights of animals from both groups were similar

(BAC-DATiCre, 33.9 g ± 0.8; controls, 33.4 g ± 0.7),

as were the levels of muscular strength (for two

limbs – BAC-DATiCre, 1.2 N ± 0.1; control, 1.3 N ± 0.1; and for four limbs) BAC-DATiCre, 2.4 N ± 0.1; and controls, 2.5 N ± 0.1) and locomo-tion (Fig 4A) Anxiety-like behavior was not different between the two genotypes as assessed by two tests based on the natural avoidance behavior of mice: the dark–light transition test (latency to exit the dark com-partment for the first time, 15 s ± 2.9 versus 17.9 s ± 2.5, P¼ 0.46, and time spent in the lit com-partment, 174.9 s ± 7.8 versus 186.8 s ± 5.9, P¼ 0.24, for transgenic and control mice, respectively) and the elevated plus maze test (time spent in open arms:

Fig 3 Pattern of b-galactosidase activity in dopamine cell groups A8–A16 in DATiCre mice For each group of dopamine cells, TH expression was detected by immunohistochemistry, in mice carrying both the BAC-DATiCre and R26R transgenes (left panels) Recombination events were detected on serial sections stained with X-Gal for the detection of b-galactosidase activity (right panels) In A8, A9, A10, A11, A12 and A16, iCre-induced recombination matches the pattern of TH expression, but some discrepancies are observed in the periaqueductal gray area, the ventromedial part of the arcuate nucleus (arrow) and A13 In the A14 and A15 group of cells, recombination events are less fre-quently observed than TH expression The highlighted structures on these anatomic drawings from representative coronal sections of the adult mouse brain modified from Paxinos [38] contain dopaminergic cells Magnification: scale bars ¼ 50 lm.

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150 s ± 16 versus 146 s ± 18 for transgenic and

con-trol mice, respectively) Similarly, no differences were

observed when despair-like behavior was studied using

the forced swim test (Fig 4B)

Discussion

Transgenesis with small DNA regions regulating

tran-scription is inherently prone to problems of ectopic

expression, mosaic expression or the absence of

expres-sion, due to the influence of genomic sequences

surrounding the integration site or the absence of

essential DNA elements The expression of a transgene

under the control of an endogenous regulatory

ele-ment, using knock-in approaches, can prevent these

problems but disrupts one copy of the gene, generating

results that may be difficult to interpret The use of a

large (100–250 kb) DNA segment that contains a gene

with an interesting expression profile, including all

DNA regions required for correct gene expression, alleviates this problem [13,14] We targeted Cre recom-binase expression to dopamine neurons, by generating

a mouse transgenic line expressing the iCre recombin-ase under the control of the DAT gene encompassed within a 177 kb BAC We used the iCre ORF, an improved version of the Cre recombinase gene that is more efficiently expressed in mammalian cells [10]

In the ventral tegmental area and the substantia ni-gra, the pattern of iCre expression was similar to the pattern of expression of the endogenous DAT gene reported in previous in situ mRNA hybridization stud-ies [15–17] When recombination events were analyzed

in situfollowing the Cre-dependent recombination of a LacZ reporter construct in double-transgenic animals, b-galactosidase expression was found to be confined to areas of endogenous DAT gene expression Recombi-nation was restricted exclusively to TH- and DAT-positive neurons Discrepancies have been reported between TH and DAT expression in the ventral midbrain in rats [17], but we detected no TH-positive neurons that did not display recombination in the ventral midbrain group of cells This suggests that the TH-positive, DAT-negative cells detected in previous studies may have been in a transient state or may have expressed DAT during development

The perfect concordance between Cre and DAT expression in the midbrain of BAC-DATiCre mice led

us to use this strategy to improve the definition

of DAT gene expression in other dopaminergic cell groups in which the precise distribution of DAT was unclear In double-transgenic mice carrying the Cre and R26R (reporter) transgenes, transient or low-level DAT expression should be paralleled by Cre sion, leading to permanent, irreversible LacZ expres-sion, increasing the sensitivity of DAT detection We focused on the various cell groups of the hypothalamic region (from A11 to A15)

No DAT-iCre-mediated recombination was observed

in the ventromedial neurons of the arcuate nucleus in mice Interestingly, in other mammals, this region has been shown to contain monoenzymatic neurons expres-sing TH or aromatic l-amino acid decarboxylase, but not the entire enzymatic machinery required for dop-amine synthesis Dopdop-amine produced in this region may result from an exchange of precursor molecules between complementary cells [18,19] Previous studies have suggested that no DAT mRNA is produced in this region [17,20,21] The authors detected very low levels of DAT expression in the dorsomedial part of the arcuate nucleus, and our results are consistent with the presence of very few recombined cells per section DAT mRNA levels, which were considerably lower in

A

B

Fig 4 The presence of the BAC-DATiCre transgene did not affect

locomotion and despair behavior (A) The presence of the transgene

had no effect on locomotor activity measured within a circular

maze The numbers of 1 ⁄ 4th turns per 5 min are indicated for

trans-genic and control animals (B) Transtrans-genic and control littermates did

not show differences in immobility time in a forced swim test.

Immobility was measured for 6 min, in 2-min periods, over two

con-secutive days Results obtained with transgenic (n ¼ 11) and

con-trol (n ¼ 11) mice are shown in black and gray, respectively.

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the A13 region than in the ventral midbrain [17,21,22],

may account for the smaller number of

b-galactosi-dase-expressing neurons than of TH-positive neurons

Expression of DAT in the A14 nucleus of rats remains

controversial, as some studies have reported the

detec-tion of very low levels of DAT mRNA in scattered

cells [21], whereas others detected neither DAT mRNA

nor protein [22,23] DAT-iCre-expressing mice allow

us to answer this question, as the lack of

recombina-tion in the A14 nucleus indicates an absence of DAT

in these dopaminergic neurons in mice

The discrepancies observed between TH and X-Gal

labeling in A15 are consistent with the absence of

DAT expression in A15, as shown by in situ mRNA

hybridization or immunohistofluorescence [17,21–23]

All previous published data on DAT expression

pat-terns were obtained with rats Our study is thus the

first to confirm that the pattern of DAT expression in

the brain is very similar in mice and rats

A recombination pattern restricted to dopamine cells

has been previously achieved [24,25] The approaches

used involved insertion of the Cre gene, at different

positions, into the DAT gene by homologous

recombi-nation in embryonic stem cells In two cases, this led to

inactivation of the endogenous targeted DAT allele and,

in the last case, to an alteration of its expression,

prob-ably due to the presence of the Cre construct in the

3¢-UTR of DAT mRNA [26] An important advantage

of using BAC transgenesis is that it leaves both

endog-enous DAT alleles intact This is essential, as changes in

DAT gene expression levels lead to atypical behaviors

[26–31] We confirmed that the presence of the

BAC-DATiCre transgene had no effect on muscular strength,

locomotion, or anxiety-related or despair behaviors

The BAC-DATiCre transgenic line is likely to prove

a valuable tool for targeting DNA recombination

events, resulting in reporter gene expression or gene

inactivation, in studies of dopamine neuron biology

and presynaptic alterations in physiopathologic

disor-ders involving dopaminergic systems Combined with

mice expressing cre-dependent fluorescent protein, it

will facilitate the localization and the study of

dopam-ine cells in living tissues Combdopam-ined with conditional

alleles of relevant genes, it will allow us to distinguish

their function in dopamine cells from their function in

other cell types This will be particularly useful in the

context of Parkinson’s disease, in which several genes

potentially involved in the pathogenesis have a

wide-spread expression pattern In this respect, we have

already used the BAC-DATiCre vector to inactivate

the cAMP-response-element-binding (CREB) gene

[32] In combination with the D1Cre line, which

allows targeting of recombination in dopaminoceptive

neurons expressing dopamine 1a receptor [33,34], the BAC-DATiCre line will allow us to distinguish between presynaptic and postsynaptic gene functions The BAC-DATiCrefto transgenic line has been deposited in the European Mouse Mutant Archives (http://www emma.rm.cnr.it)

Experimental procedures

DNA construction and transgenesis

Using the Ensembl genome database, we chose a 210 kb BAC (RP24 408F13) encompassing the DAT (slc6) gene from a CHORI BAC library [35] This BAC was modified by homologous recombination [11], to insert a 2950 bp DNA cassette containing the ORF of the improved Cre recombin-ase, iCre [10], followed by a DNA sequence containing intron and polyA sequences from the bovine GH gene, and

an ampicillin resistance gene flanked by two FLP recombi-nase target sites in the same orientation, which was subse-quently removed (Fig 1) The BAC was modified as follows:

a 319 bp 5¢ DNA fragment of the DAT gene was ampli-fied by PCR (using the primers 5¢-CTAGGTACCA CAAGCCGGCGTTAATGTGAA and 5¢-CTAATCGAT GGAGCCCGAGGAAGTCTGTTT), digested with ClaI and KpnI, and inserted upstream from the iCre DNA cassette

in pMT1 The pMT1 plasmid was derived from the vector iCre-internal ribosome entry site (IRES)-green fluorescent protein (GFP)-polyA [36], by removing an NsiI–BsrGI DNA fragment containing the IRES and eGFP sequences, and then the 3¢ protruding NsiI end, and filling in the 5¢ BsrGI end before ligation (sequence available on request) The 3¢ homology region was a 200 bp DNA fragment of DAT ge-nomic DNA amplified by PCR (using the primers 3¢-CAT GCTAGCTAAAAGCAAATGCTCCGTGGG and 3¢-CTA GTATACGAAACCTCCAGACATTGGCCA), digested with BstZ17I and NheI, and inserted downstream from the DNA cassette Recombination was induced as previously described [11] Briefly, EL250 bacteria (a gift from N G Copeland, National Cancer Institute, Frederick, MO, USA) containing the RP24 408F13 BAC were electroporated with the targeting vector The bacteria were incubated for 15 min

at 42C to induce recombination Correctly targeted BACs were identified by DNA restriction analysis of ampicillin-resistant colonies The ampicillin resistance cassette used for selection was excised by inducing Flpe recombinase expres-sion by adding 0.1% l-arabinose to the culture medium for

1 h Correctly recombined BACs were identified by DNA restriction analysis and pulsed-field gel electrophoresis A

177 kb DNA fragment was excised from the BAC DNA by digestion with PmeI–AscI and purified by chromatography

on Sepharose CL4b columns (Pharmacia, New York, NY, USA) [37] This fragment was then used for pronuclear injec-tions into fertilized FVB⁄ N oocytes

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Mouse genotyping and breeding

Transgenic BAC-DATiCrefto animals were maintained in

an FVB⁄ N background as well as being backcrossed for

five generations onto the C57BL⁄ 6 background These

animals were genotyped using DNA obtained by tail

biopsy, by dot blot DNA hybridization, with a32

P-radio-labeled DNA fragment from the iCre ORF Rosa 26

repor-ter animals [9] were maintained on a mixed background

and genotyped by PCR amplification, using LacZ-Forward

(5¢-GTCGTTTTACAACGTCGTGACT-3¢) and

LacZ-Reverse (5¢-GATGGGCGATCGTAACCGTGC-3¢)

prim-ers Animals were housed under specific pathogen-free

conditions at 22C, with a 12 h light ⁄ 12 h dark cycle and

free access to water and a rodent diet All experiments were

performed in accordance with French (Ministe´re de

l’Agri-culture 87-848) and European Union (EEC86-6091)

guide-lines for care of laboratory animals

Histology

Vibratome sections (30 lm) prepared from the brains of

perfused mice (4% paraformaldehyde in 0.1 m NaCl⁄ Pi)

were incubated overnight with a rabbit polyclonal anti-Cre

serum (1 : 3000 dilution [12]), 1 : 400 dilution) or a

mono-clonal b-galactosidase antibody (Monosan, Am Uden, the

Netherlands; 1 : 2000 dilution) Immunohistochemistry was

carried out with the avidin–biotin system from Vector

Laboratories (Vectastain, Burlingame, CA, USA) For

double-immunofluorescence labeling, we used

Alexa-488-coupled anti-mouse serum (Molecular Probes, Eugene, OR,

USA) and Cy3-coupled anti-rabbit serum (Jackson

Immu-noresearch, West Grove, PA, USA) at a 1 : 400 dilution as

the secondary antibodies Immunostaining controls were

performed in the same way, but without primary

antibod-ies Nuclei were stained with 0.5 lm TO-PRO-3 iodide

(Molecular Probes) b-galactosidase enzymatic activity was

detected on brain cryosections (30 lm) The histologic

immunofluorescence of sections was assessed with a Leica

TCS SP 2 confocal laser scanning microscope (Leica

Micro-systems, Heidelberg, Germany) For cryostat sectioning,

brains were transferred to 15% sucrose in NaCl⁄ Piat room

temperature, and then embedded in 7% gelatin and 15%

sucrose in NaCl⁄ Pi, before being frozen by immersion in

isopentane at ) 140 C Cryosections were cut and

incuba-ted at 37C overnight in X-Gal staining solution [4 mm

potassium hexacyanoferrate(III), 4 mm potassium

hexa-cyanoferrate(II), 2 mm MgCl2, 0.02% NP-40, 0.01% sodium

deoxycholate, 5 mm EGTA, and 4 mgÆmL)1X-Gal]

Behavioral studies

Mutant (n¼ 11) and control (n ¼ 11) littermate males

matched for age (4–6 months) were housed together One

hour before each behavioral test, mice were isolated in

individual cages Muscular strength was quantified using the Grip strength test (Bioseb, Chaville, France) To meas-ure spontaneous locomotor activity, mice were placed, for

130 min, in a circular corridor (4.5 cm width, 17 cm exter-nal diameter) crossed by four infrared beams (1.5 cm above the base), equally spaced (Imetronic, Pessac, France) The locomotor activity was counted when animals interrupted two successive beams and thus had traveled at least a quar-ter of the circular corridor Anxiety was assessed using the dark–light transition test and the elevated plus maze The dark–light box was 45· 20 · 25 cm, and separated into two compartments connected by a central aperture (5· 5 cm) (Ligna, Paris, France) The dark compartment (black PVC, 15 cm) was covered, and in the lit compart-ment the intensity of light (white PVC, 30 cm) was 700 lux Animals were tracked for 5 min using the Ethovision video tracking system from Noldus (Wageningen, The Nether-lands); both time of exit from the dark compartment and the time spent in the lit compartment were measured The elevated plus maze consists of two elevated (1 m high) and open arms (24· 8 cm) positioned opposite to one another and separated by a central platform and two arms of the same dimension, but enclosed by walls (20 cm high), form-ing a cross The maze was lit by a light placed above the central platform (30 lux in each arm) The mouse was placed on the platform, and allowed to explore for 10 min, and the time spent and the number of presentations (two paws) and entries (four paws) in the open arms were recor-ded and quantified using Ethovision To assess despair be-havior, we performed a forced swim test We placed mice (for 6 min during two consecutive days) in a glass cylinder (height 25 cm, diameter 11 cm) containing water 8 cm deep (23C) Immobility time was measured during three peri-ods of 2 min Data were expressed as means ± SEM and analyzed by Student’s t-test or ANOVA followed by the post hocScheffe test

Acknowledgements

We are grateful to E Casanova for the gift of piCre-IRES-GFP-polyA plasmid, and to N G Copeland for the gift of EL250 bacteria We thank M Cohen-Salmon, S Vyas, J P Tassin, S Mhaouty-Kodja,

G Schu¨tz and P.V Piazza for discussions, critical reading or support We thank H Cambier for excellent technical assistance This work was supported by the

‘Centre National de la Recherche’ and the Colle`ge de France, by grants from the ‘Ministe`re de l’Education National de la Recherche et de la Technologie’ (‘Action Concerte´e Incitative neurosciences’), the

‘Agence Nationale de la Recherche’ (neurosciences), the ‘Mission Interministe´rielle de Lutte contre la De´pendance et la Toxicomanie’ (MILDT), the ‘Fonda-tion pour la Recherche Me´dicale’ (FRM) and the

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‘Fondation NRJ’ M Turiault held PhD fellowships

from MILDT and FRM

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