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Targeting the Shc adaptor to lipid rafts by engineering the adaptor with a raft targeting tail led to altered biochemical signaling resembling, in several respects, signal transduction d

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by PTB-adaptor engagement

T K Lundgren, Moritz Luebke, Anna Stenqvist and Patrik Ernfors

Division of Molecular Neurobiology, Department of Medical Biochemistry and Biophysics, Karolinska Institute, Stockholm, Sweden

The Ret receptor tyrosine kinase (RTK) has many

dif-ferent functions during development and in adult life

Ret is activated upon engagement of its ligands glial

cell line-derived neurotrophic factor (GDNF),

neurtu-rin, persephin, and artemin, and the coreceptors

GDNF family receptors (GFRs) a1–4 Dysregulated

Ret is implicated in diseases such as the multiple

endo-crine neoplasia (MEN) syndromes (MEN2a and MEN2b), as well as in agangliosis of the colon, one of the most common developmental defects in young chil-dren [1] In the developing nervous system, neural crest cell migration depends on Ret ligands produced in the surrounding tissue, which guide the migrating cells

to a correct position within the embryo [2] In the

Keywords

fractionation; Frs2; lipid rafts; PTB adaptors;

Ret

Correspondence

P Ernfors, Division of Molecular

Neurobiology, Department of Medical

Biochemistry and Biophysics, Karolinska

Institute, 171 77 Stockholm, Sweden

Fax: +468 341960

Tel: +468 52487659

E-mail: patrik.ernfors@ki.se

(Received 14 December 2007, revised 23

February 2008, accepted 26 February 2008)

doi:10.1111/j.1742-4658.2008.06360.x

Glial cell line-derived neurotrophic factor family ligands act through the receptor tyrosine kinase Ret, which plays important roles during embryonic development for cell differentiation, survival, and migration Ret signaling

is markedly affected by compartmentalization of receptor complexes into membrane subdomains Ret can propagate biochemical signaling from within concentrates in cholesterol-rich membrane microdomains or lipid rafts, or outside such regions, but the mechanisms for, and consequences

of, Ret translocation between these membrane compartments remain lar-gely unclear Here we investigate the interaction of Shc and Frs2 phos-photyrosine-binding domain-containing adaptor molecules with Ret and their function in redistributing Ret to specialized membrane compartments

We found that engagement of Ret with the Frs2 adaptor results in an enrichment of Ret in lipid rafts and that signal transduction pathways and chemotaxis responses depend on the integrity of such rafts The competing Shc adaptor did not promote Ret translocation to equivalent domains, and Shc-mediated effects were less affected by disruption of lipid rafts How-ever, by expressing a chimeric Shc protein that localizes to lipid rafts, we showed that biochemical signaling downstream of Ret resembled that of Ret signaling via Frs2 We have identified a previously unknown mecha-nism in which phosphotyrosine-binding domain-containing adaptors, by means of relocating Ret receptor complexes to lipid rafts, segregate diverse signaling and cellular functions mediated by Ret These results reveal the existence of a novel mechanism that could, by subcellular relocation of Ret, work to amplify ligand gradients during chemotaxis

Abbreviations

CO, cholesterol oxidase; CTB, cholera toxin B; DRG, dorsal root ganglia; DRM, detergent-resistant membrane; E, embryonic day; eGFP, enhanced green fluorescent protein; ERK, extracellular signal-related kinase; GFR, glial cell line-derived neurotrophic factor family receptor; GNDF, glial cell line-derived neurotrophic factor; HA, hemagglutinin; MAPK, mitogen-activated protein kinase; MCD, methyl-b-cyclodextrin; MEN, multiple endocrine neoplasia; MLS, membrane localization signal; PI3K, phosphoinositide-3-kinase; PTB adaptor, phosphotyrosine-binding domain-containing adaptor; PVDF, poly(vinylidene difluoride); RTK, receptor tyrosine kinase; SUP, detergent-soluble supernatant; TfR, transferrin receptor.

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developing and adult mouse, Ret is also instrumental

in promoting survival of neurons, including

parasym-pathetic neurons and dopaminergic neurons of the

sub-stantia nigra pars compacta [3–5]

Many of the functions regulated by Ret activation

depend on an intact Tyr1062 in the intracellular

domain [6,7] Upon Ret phosphorylation, this tyrosine

mediates biochemical signal transduction via

interac-tion with adaptor proteins Several

phosphotyrosine-binding domain-containing adaptors (PTB adaptors)

compete for interaction with Tyr1062, but only one

adaptor may interact at any given time [8] The

dis-tinct functional outcome of Ret activation is

corre-lated with the different PTB adaptors interacting with

Ret [9,10] We have shown previously that expression

of Ret mutants, selective for binding to either the

adaptor protein Shc or Frs2 to Tyr1062, results in

distinctly different patterns of plasma membrane

localization of Ret Frs2 recruitment resulted in a

concentration of Ret into membrane foci [4,11] The

Ret receptor has recently been shown to signal from

within different cellular compartments The oncogenic

precursor of Ret (Men2B) can be activated and

induce downstream signaling from within the

endo-plasmic reticulum [12] Ret can also be recruited to

specialized lipid raft domains in the plasma

mem-brane, where it can be phosphorylated, interact with

adaptor proteins and induce downstream signaling

[12–14]

Lipid rafts are membrane microdomains, rich in

sphingolipids and cholesterol, that form lateral

assem-blies in the plasma membrane [15] Lipid rafts

seques-ter a number of different proteins that are

palmitoylated or contain a number of other lipid

anchors, which may be regulated by the selective

interaction with these domains In this way, the

recruitment of Ret to lipid rafts can lead to the

acti-vation of distinct signaling pathways, due to the

com-partmentalized cell signaling events However, the

mechanism for directing Ret into lipid rafts remains

largely unknown We report here that the PTB

adap-tor Frs2 functions to translocate Ret to membrane

subdomains of the lipid raft type Interactions of Ret

with the Shc adaptor, which, in contrast to Frs2,

lacks a palmitoylation tail that confers attachment to

lipid rafts, did not result in redistribution of Ret to

lipid rafts Targeting the Shc adaptor to lipid rafts by

engineering the adaptor with a raft targeting tail led

to altered biochemical signaling resembling, in several

respects, signal transduction downstream of Frs2 We

show that the distinct biological outcomes of Ret

acti-vation largely depends on the targeting to, and

signal-ing from within, lipid rafts

Results

A PTB-adaptor-dependent membrane relocation

of Ret receptors

To investigate whether Tyr1062 is important for Ret translocation into lipid rafts, we performed crude fractionations of neuronal SK-N-MC cells transfected with the MEN2a version of Ret (2aRet) The MEN2a mutation C634R is found in more than 85% of patients with MEN syndrome, and renders Ret con-stitutively active, thus omitting the need for ligand for its activation Lipid rafts and nonraft membranes were isolated according to their resistance to and sol-ubilization by detergent Cells expressing 2aRet or a

2aRetY1062F mutant that is incapable of adaptor inter-action with the phosphorylated Tyr1062 were har-vested in detergent, and the detergent-resistant membrane (DRM) fraction was separated from the detergent-soluble supernatant (SUP) fraction by cen-trifugation Each fraction was subjected to PAGE and transferred to poly(vinylidene difluoride) (PVDF) membranes Immunoblotting against Ret revealed that the majority of Ret partitioned in the DRM fraction This was in contrast to the 2aRetY1062F mutant, where the majority of Ret was found in the SUP fraction (Fig 1A) To test whether Ret distribution was affected by interaction with either the Frs2 or the Shc adaptor, we overexpressed Shc or Frs2 constructs together with 2aRet in SK-N-MC cells Lysates from cells overexpressing Shc or Frs2 were divided into equal halves, and each half was immunoprecipitated for Shc or Frs2 and immunoblotted against Ret Nearly all Ret precipitated with the overexpressed adaptor, showing that overexpression of one adaptor leads to outcompetition of the other with respect to Ret interaction (Fig 1B) Next, we fractionated lysates of cells expressing 2aRet or 2aRetY1062F together with overexpressed Frs2 or Shc into DRM and SUP fractions Adaptor overexpression led to high amounts of Ret in the DRM fraction in both Frs2 and Shc conditions, whereas 2aRetY1062F, as before, displayed a much lower partitioning into DRMs (Fig 1C) Ret localization to the supernatant fraction was largely unaffected by the Y1062 muta-tion in the presence of Frs2 or Shc To confirm the relative purity of raft and nonraft membranes, DRM and SUP fractions were immunoblotted towards the lipid raft marker flotillin-1 and the transferrin recep-tor (TfR), the latter of which is excluded from raft-like domains As expected, flottillin-1 was found nearly exclusively in the DRM fraction, whereas TfR was mainly found in the SUP farction (Fig 1C)

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Furthermore, the DRM and SUP fractions were

im-munoblotted towards the adaptor proteins themselves

Both Shc and Frs2 were found predominantly in the

DRM fractions (Fig 1D) These results suggest that

Ret localization to nonraft membrane regions occurs

independently of adaptor engagement, whereas its

localization to lipid rafts depends on Shc or Frs2

interactions with Ret Tyr1062

To examine whether Ret and adaptor partitioning

is affected by disruption of lipid-ordered regions, we

exposed cells to methyl-b-cyclodextrin (MCD), an

agent that is commonly used to extract cholesterol

from cell membranes in order to disrupt lipid-ordered

regions in the plasma membrane Cells were treated

for 30 min with 8 mm MCD or vehicle prior to

frac-tionation and subsequent immunoblotting towards

Ret The amount of Ret present in the DRM fraction

was significantly reduced, with a corresponding

increase of Ret in the SUP fraction, when cells were

treated with MCD (Fig 1D) This indicates that the

integrity of cholesterol-rich regions is necessary for

Ret partitioning into DRMs, agreeing with previous data [14]

The insoluble material recovered in the DRM frac-tion represents a collecfrac-tion of many discrete lipid-ordered structures, and is not exclusively composed of lipid rafts [16] Previous studies have found that Ret partitions into DRM fractions upon ligand induction [14], and that interaction of Ret with the Frs2 adap-tor, but not the Shc adapadap-tor, occurs in such fractions [13] To further investigate whether the previously determined localization of Ret to membrane foci, depending on adaptor engagement [11], had any rela-tion to lipid rafts, we expressed Ret tagged with enhanced green fluorescent protein (eGFP) (ReteGFP)

in cells Fusion of eGFP to Ret allows direct visuali-zation of the subcellular distribution of Ret ReteGFP was expressed with Shc or Frs2 in SK-N-MC neuro-nal cells that were stained for visualization of the membrane lipid rafts using a fluorescently conjugated cholera toxin B (CTB) subunit that binds to the pen-tasaccharide chain of plasma membrane ganglioside GM1, which selectively partitions into lipid rafts [17] Using confocal microscopy, we found that there was

a consistent punctuate pattern of lipid rafts with high abundance in neurites after 30 min of ligand stimulus Interestingly, extensive localization of ReteGFPto lipid rafts was seen only in Frs2-expressing cells, and not

in cells expressing Shc (Fig 2A,C) The distinct recep-tor localization was also correlated with morphologi-cal differences between Shc- and Frs2-expressing cells; Frs2-expressing cells often contained markedly more cell processes and neurites than Shc-expressing cells Frs2 is exclusively localized to lipid rafts [18], whereas Shc has been shown to inducibly localize to lipid-ordered regions in some instances, e.g in T-cell recep-tor signaling [19] To examine whether lipid raft targeting of Shc could mediate translocation of Ret

to a subcellular localization similar to that mediated

by Frs2, we expressed an Shc construct containing the Ras membrane localization signal (MLS) (ShcMLS), which permanently localizes Shc to lipid rafts [20] This Shc construct was shown in previous work to activate the mitogen-activated protein kinase (MAPK) pathway constitutively [20] Intriguingly, sustained activation of MAPK is one prominent dis-tinguishing feature of Ret signaling by Frs2 recruit-ment, in contrast to Ret signaling via Shc [4] When ShcMLS was overexpressed in cells along with ReteGFP, the membrane localization of Ret was lar-gely confined to lipid raft regions, similar to that of ReteGFP- and Frs2-expressing cells (Fig 2B), indicat-ing that lipid raft targetindicat-ing of Shc leads to a redistri-bution and enrichment of Ret in lipid rafts

A

C

D

B

Fig 1 Tyr1062 is necessary for Ret partitioning into lipid rafts (A)

Mutation of Tyr1062 results in a loss of2aRet partitioning to the

DRM fraction.2aRet WT or2aRet Y1062F was expressed in SK-N-MC

cells Cells were harvested in 1% Triton buffer, separated into

DRM or SUP fractions, separated on polyacrylamide gels, and

transferred to PVDF membranes, with subsequent blotting for Ret

(n = 3 with similar results) (B) Overexpression of Shc or Frs2

adap-tors forces 2a Ret to interact with either adaptor at the expense of

the other Lysates of SK-N-MC cells were separated, and each half

was immunoprecipitated for Shc or Frs2 and immunoblotted

against Ret (n = 2) (C) Overexpression of Shc or Frs2 adaptors

results in2aRet WT but not 2aRet Y1062F partitioning into DRM

frac-tions SK-N-MC cells were treated and harvested as in (A), and

blot-ted for detection of Ret (n = 3) (D) Shc and Frs2 partitioning of Ret

to the DRM fraction depends on intact lipid rafts SK-N-MC cells

expressing2aRet WT and2aShc or Frs2 were treated with MCD or

vehicle, harvested, and separated into DRM and SUP fractions.

Immunoblot towards Ret and Frs2 or Shc as indicated (n = 3).

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A critical role of Ret membrane localization

in downstream signaling events in cell lines

and primary cells

We next examined the intracellular signaling

down-stream of Ret in cells expressing the Shc, ShcMLS or

Frs2 adaptors Phosphorylated (active) Akt or p42,44

extracellular signal-related kinase (ERK) MAPK

pro-tein levels were examined by immunoblotting at

differ-ent time points after Ret ligand stimuli ShcMLS

expression together with Ret resulted in signal

activa-tion resembling the Ret⁄ Frs2 coexpression

characteris-tics Specifically, a higher and sustained ERK p42,44

MAPK level was also observed at 12 h in cells

express-ing ShcMLS as compared to Shc (Fig 3A)

Further-more, Ret signaling via ShcMLS did not lead to as

robust activation⁄ phosphorylation of Akt as did Shc,

but levels were higher than for signaling via Frs2

(Fig 3C) To investigate the importance of rafts for

downstream ERK p42,44 MAPK activation, we applied cholesterol oxidase (CO) to cells during ligand stimuli CO was chosen because cells cannot withstand MCD treatment for long time periods, and CO has been shown to disrupt the biochemical effects of mem-brane-localized Ret as well as other receptor complexes

in a fashion similar to what is accomplished by using MCD [11,21,22] In the presence of CO, ERK p42,44 MAPK activation was markedly reduced both in cells expressing Ret⁄ ShcMLS as well as in cells expressing Ret⁄ Frs2 at all time points, whereas activation of ERK p42,44 MAPK downstream of Shc was much less affected (Fig 3A) The specificity of cholesterol species for phosphorylated ERK p42,44 MAPK was also important in MEN2a versions of Ret when expressed with Shc or Frs2 In cells expressing2aRet, phosphory-lated ERK p42,44 MAPK levels were high when Shc and Frs2 were coexpressed CO treatment attenuated phosphorylated ERK p42,44 MAPK levels

Fig 2 Labeling of plasma membrane gan-glioside GM1 lipid rafts shows extensive colocalization of Frs2-associated but not Shc-associated Ret to lipid rafts (A–C) SK-N-MC cells expressing Ret eGFP (green) together with Shc (A,A¢), Shc MLS (B,B¢) or Frs2 (C, C¢) Actin was stained with

Alexa-648 phalloidin and Alexa-546 (red) conju-gated to CTB to mark lipid rafts (n = 5) Arrows indicate colocalization of Ret eGFP

with lipid rafts Scale bar = 25 lm.

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stream of Shc and led to an almost complete absence

of phosphorylated ERK p42,44 MAPK downstream of

Frs2 (Fig 3B)

Most studies on receptor signaling from within rafts

have been performed on immortalized cell lines in

cul-ture or by using artificial membranes We attempted to

see whether the PTB adaptors determine Ret

subcellu-lar distribution and signaling in primary neurons of a

more complex system To this end, we electroporated

ReteGFP and either adaptor into the neural tube of

chicken embryos DNA was injected in ovo into the

neural tube of embryos at Hamburger–Hamilton

stage 11 [approximately embryonic day (E)2], and

elec-troporation was performed by placing electrodes along

the rostral–caudal axis of the neural tube The egg was

then closed and placed in an incubator until the

embryos had grown to stage 24 (E4.5) At this stage,

the expression of transfected ReteGFP was found in the

developing spinal cord and in dorsal root ganglia

(DRG), and the transfected tissue was dissected under

fluorescent light (supplementary Fig S1)

eGFP-posi-tive spinal cord segments were pooled and weighed,

and equal amounts of tissue were trypsinized into

sin-gle cells and immediately incubated with Ret ligands

for 30 min After ligand stimulation, the cells were fractionated into DRM and SUP fractions In accor-dance with the results obtained with neuronal

SK-N-MC cells, immunoblotting against Ret revealed that it was predominantly located in DRM fractions, regard-less of which adaptor (Shc, ShcMLS, or Frs2) was con-comitantly overexpressed (Fig 3D) Immunoblotting

of DRM and SUP fractions against the adaptor pro-teins showed that ShcMLS and Frs2 were nearly exclu-sive to the DRM fraction, whereas Shc was present also in the SUP fractions (Fig 3E)

We investigated the activation of MAPK and Akt pathways in the developing chick embryo Previous studies have found that the ERK MAPK pathway [and

to a lesser extent, also the phosphoinositide-3-kinase (PI3K)⁄ Akt pathway] can be temporally and quantita-tively affected by activation within or outside DRM fractions [23] To examine the role of the PTB adaptors

in the activation of the MAPK and Akt pathways, spinal cords electroporated with Ret together with either Shc, ShcMLS or Frs2 were separated into DRM and SUP fractions, as above Phosphorylated Akt lev-els were generally low for all PTB adaptors in the DRM fraction, showing that phosphorylated Akt

A

C

G E

B

Fig 3 The raft-targeting adaptors Frs2 and Shc MLS show enhanced ERK phophorylation, which is dependent on the integrity of cholesterol-rich domains (A) SK-N-MC cells expressing Ret and either Shc, Shc MLS or Frs2 adaptors were lysed after Ret ligand stimulation for indicated times and subsequently immunoblotted towards phosphorylated ERK and all ERK CO was applied to cells as indicated (B) Experiments as

in (A) with MEN2a versions of Ret (C) Experiments as in (A) with immunoblotting towards phosphorylated Akt and Akt (D) E2 chicken embryos were electroporated in ovo with Ret and adaptor constructs as indicated, and allowed to develop to E4.5 At E4.5, positively trans-fected spinal cord segments were dissected out and pooled to equal amounts After dissociation into single cells and stimulation with Ret ligands for 30 min cells, were lysed and fractionated into DRM and SUP fractions Fractions were immunoblotted for detection of Ret (E) Experiments as in (C) with immunoblotting for the hemagglutinin (HA)-tag of Shc and Shc MLS or for Frs2 (F, G) Experiments as in (C) with immunoblots towards phosphoryated Akt or phosphorylated ERK.

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signaling predominantly takes place in cellular

struc-tures partitioning into the SUP fraction (Fig 3G)

Phosphorylated Akt levels were greater in the SUP

fraction when Shc or ShcMLS was expressed with Ret

as compared to Frs2, where levels were overall lower

and more similar between the DRM and SUP fractions

(Fig 3G) Phosphorylated ERK p42,44 MAPK

immu-noblotting revealed the strongest signal when Frs2 was

expressed with Ret in both the DRM and the SUP

fractions (Fig 3F) ShcMLS resulted in activation of

ERK p42,44 MAPK in the DRM Shc, on the other

hand, was very poor in mediating ERK p42,44 MAPK

activation in the DRM fraction (Fig 3G) These results

therefore agree with the results obtained with

SK-N-MC cells, and further support the idea that

phos-phory lated ERK p42,44 MAPK is often localized to

complexes of PTB adaptors with Ret in lipid rafts and

that phosphorylated Akt is mostly found outside rafts

To characterize the Ret distribution in greater detail

than is permitted by DRM⁄ SUP fractionation, we used

a recently published fractionation protocol that omits

the need for detergent and is highly specific with

regard to molecular distribution within and outside

lipid raft fractions [24] Dissected spinal cords from

chick embryos were lysed in detergent-free buffer

and fractionated by ultracentrifugation in OptiPrep

gradients Seven fractions were aspirated from

columns, and the protein content within each fraction was concentrated by precipitation, loaded on poly-acrylamide gels, and transferred to PVDF membranes The separation of raft fractions from nonraft fractions was confirmed by immunoblotting against flotillin-1 and TfR (Fig 4) As determined by immunoblotting against Ret, this more sensitive method revealed that the fractional distribution of Ret varied with the adap-tor expressed in the embryos Frs2 overexpression led

to Ret being directed towards lower-density fractions (corresponding to lipid rafts), peaking in fraction 2 Shc overexpression resulted in significantly less Ret partitioning into the fractions of lowest density, with little or no Ret in fraction 2 and peak levels in frac-tion 3 (Fig 4) Overexpression of the raft-localizing ShcMLS construct resulted in a significant portion of Ret being partitioned into both fraction 2 and frac-tion 3 Immunoblotting against the adaptors them-selves showed that whereas both Shc and ShcMLS were present in both low-density and high density fractions, Frs2 was nearly exclusive to the same fraction as Ret (fraction 2) (Fig 4)

Functional consequences of signaling from raft and nonraft membrane compartments

Association of Ret with the different adaptors may result in distinct cellular responses to Ret ligand stimu-lation [4,9] In functional terms, the chemotactic prop-erties of Ret signaling via Frs2 are much greater than those of Ret signaling via Shc [11] Ret signaling via recruitment of Shc, on the other hand, is necessary for Ret-mediated cell survival when neuronal cells are pre-sented to toxic agents, and also for neurite formation

in certain cell types [4,9] We investigated how disrup-tion of lipid-ordered domains affected these funcdisrup-tional aspects, and whether the ShcMLS adaptor is similar to Frs2 or Shc in terms of cellular response to Ret stimu-lation Chemotactic migration towards Ret ligands was examined by seeding neuronal SK-N-MC cells express-ing Ret receptors that are selective for bindexpress-ing of only Frs2 (RetFrs+) or Shc (RetShc+) to the region of Tyr1062 [4] Ret mutants binding to Frs2 showed a nearly three-fold higher migrational capacity than did RetShc+, and this effect was ligand-dependent, as all conditions displayed a similar low level of random cell migration without ligand being supplied to the lower culture compartment (Fig 5A) Cells expressing RetShc+⁄ Shc did not show any statistically significant ligand-induced migration Cells expressing ShcMLS together with RetShc+ displayed a two-fold increase of migration towards Ret ligand (Fig 5A) The same experiment was then performed in the presence of CO

A

Fig 4 Density fractionation of the membrane localizes Frs2 and

Shc partly to different membrane compartments (A) E2 chicken

embryos were electroporated in ovo and allowed to develop to E5.

At E5, positively transfected spinal cord segments were dissected

out and pooled to equal amounts of input After dissociation into

single cells and stimulation with Ret ligands for 30 min, cells were

harvested in detergent-free buffer and subjected to

ultracentrifuga-tion in OptiPrep density gradients Fracultracentrifuga-tions were taken out after

centrifugation, and concentrated protein from each fraction was

immunoblotted against Ret or HA-tag for Shc and Shc MLS or

against Frs2, as indicated Fraction 1 is the less dense fraction, and

fraction 7 is the fraction with the highest density The bottom panel

shows immunoblotting for the lipid raft marker flotillin-1 and the

nonraft marker TfR.

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CO treatment of cells led to a large decrease in the

number of migrating cells, almost reaching baseline

levels in all conditions (Fig 5A), indicating that the

integrity of cholesterol-rich membrane domains is

nec-essary for the occurrence of directional migration

towards Ret ligand

The ability of Ret selective mutants to promote cell survival was next examined SK-N-MC cells treated with anisomycin to induce apoptosis were rescued by supplementing the medium with Ret ligands To detect the apoptotic response to anisomycin with high sensi-tivity, single cells were examined in the comet assay In this system, fragmented DNA moves out of the cell soma in the shape of comet tails when cells are embed-ded in agarose and subjected to an electric field After staining of DNA with SYBR-green, images were cap-tured using fluorescence microscopy The comet-tail moment was determined by measuring pixel intensity

in the comet head and tail, and calculating the momentum of the comet tails [25] In accordance with what was previously found [4], Ret ligands resulted in

a much greater cell survival effect for cells expressing RetShc+than for those expressing RetFrs+(Fig 5B,C) When ShcMLS was coexpressed with RetShc+ to enforce Shc signaling in lipid rafts, an intermediate survival-promoting effect was seen (Fig 5B,C) Dis-ruption of cholesterol-rich membrane compartments with CO had no effects on cell survival (Fig 5B,C) Furthermore, CO alone without anisomycin did not result in any detectable cell injury (Fig 5B,C), suggest-ing that CO at the concentrations used does not have

an effect on cell survival Thus, these data suggest that Ret-mediated cell migration, but not cell survival, requires intact lipid rafts

Discussion

The present study was conducted to determine whether PTB adaptors determine Ret localization to different membrane compartments, and whether pathway-spe-cific signaling and functional outcomes are affected by signaling from within and outside rafts It has previ-ously been shown that Ret localizes to lipid rafts in

a glycophosphatidylinositol-bound GFRa1 coreceptor-dependent fashion [26] However, more recent results have demonstrated that both soluble and

glycophos-A

B

C

Fig 5 Frs2- dependent chemotaxis but not Shc-dependent cell survival depends on raft integrity (A) SK-N-MC cells expressing Ret mutants were analyzed in transwell chemotaxis assays after 14 h, with Ret ligands supplied below the membrane and CO supplied above and below the membrane Two-way ANOVA compared to the maximally migrating Ret Frs+ ⁄ Frs2 condition, ***P < 0.001 (n = 3) (B) SK-N-MC cells were treated with anisomycin and CO as indi-cated, and subjected to the comet assay (C) Quantification of (B) The cell-rescuing effect of Ret ligands against the apoptosis-induc-ing agent anisomycin was measured by quantifyapoptosis-induc-ing apoptosis ⁄ DNA damage expressed as comet-tail momentum Two-way ANOVA compared to the maximally rescuing RetShc+⁄ Shc condition,

**P < 0.01, *P < 0.05 (n = 3) Scale bar in (B) = 50 lm.

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phatidylinositol-anchored GFRa1 increase Ret

distri-bution to rafts [13] In the same study, Frs2 and Shc

adaptor engagement with Tyr1062 was found to occur

predominantly within and outside rafts, respectively

We report here that PTB adaptor engagement is

criti-cal for Ret to locriti-calize to lipid rafts, suggesting that

upon ligand engagement, the PTB adaptor association

results in a movement of Ret from nonraft to raft

membrane regions Consistently, phosphorylated ERK

p42,44 MAPK signaling by a MEN2a form of Ret,

which signals even in the absence of ligands and

GFRa1, was more sensitive to disruption of

choles-terol-rich membranes by CO upon Frs2 as compared

to Shc recruitment Our data also suggest that

recruit-ment of Ret to rafts by PTB adaptors results in

dis-tinct signal transduction patterns The biochemical

integrity of Ret signaling depends on undisrupted

lipid-ordered domains when Ret assembles together

with adaptors localizing to rafts, but less so when the

complex is localized outside rafts Furthermore, a

modified version of the Shc adaptor that localizes to

lipid rafts in a similar way to Frs2 results in signaling

resembling Frs2 recruitment by Ret

Our results show that Ret resides largely in DRM

fractions both in cell lines and in vivo in the chick

spinal cord This localization was critically dependent

on Tyr1062 and its interaction with Frs2 and Shc,

because eliminating interactions at this site resulted in

a clear loss of Ret in the DRM fraction and an

increase in the SUP fraction Cyclodextrins such as

MCD effectively remove cholesterol from the plasma

membrane [27] This property has led to the extensive

use of MCD to study the function of lipid rafts, which

are membrane microdomains whose integrity depends

on the presence of cholesterol In this article, we show

that cholesterol depletion by MCD results in a loss of

both Frs2- and Shc-induced increases of Ret in the

DRM fraction

Analysis of the DRM and SUP fractions suggested

that both Shc and Frs2 reside largely in the DRM

fraction, and that signaling from Ret by means of Shc

and Frs2 might be initiated from within lipid raft

membrane compartments However, with the use of a

more sensitive density-dependent fractionation, it was

clear that Ret associated with Frs2 and Shc resides in

different membrane compartments, with Ret

interac-tions with Frs2 being more strongly associated with

the flottilin-containing fractions, which are believed to

include lipid rafts [28] These experiments also showed

that, unlike Shc, which is present in very low amounts

in the Frs2-associated Ret fractions, ShcMLS is located

in both raft and nonraft fractions Details of the lipid

distribution of the sphingolipid- and cholesterol-rich

lipid rafts are not well characterized Clearly, there is a great spatial and functional heterogeneity of these membrane domains Recently, it was found that plasma membrane sphingomyelin-rich domains are spatially distinct from ganglioside GM1-rich mem-brane domains in Jurkat T cells, and may form distinct and unique signaling platforms [29] Distinct cellular localization of Shc and Frs2 with Ret⁄ Frs2 but not Ret⁄ Shc localized to GM1-rich lipid rafts was also confirmed using CTB labeling In this experiment, a clear colocalization of ReteGFP to lipid rafts in the presence of Frs2 but not Shc was evident

We noticed a high baseline activity without ligand in Frs2 conditions throughout our study This is consis-tent with previous results on ERK MAPK and on other downstream effector proteins activated via Frs2, and is most likely due to the sustained interaction of Frs2 with Ret and also other RTKs [4,30] In a recent study, we have shown that selective interaction of Ret with Shc results in the activation of AKT to a much greater extent than when Ret signals by recruitment of Frs2, and conversely that, unlike Shc signaling, Frs2 signaling leads to ERK p42,44 MAPK activation at high levels Interestingly, the difference in signaling was reflected not only by more robust activation, but also

by a significantly sustained activation from 5 min to at least 12 h Signaling by Ret via Shc activates ERK to a lesser degree, and peaks at about 30 min [4] Several lines of evidence suggest that the sustained activation

of ERK is dependent on the raft context rather than it being PTB adaptor-specific Signaling via Frs2 after disruption of the rafts by CO resulted in a marked attenuation ERK MAPK signaling, with a duration of minutes instead of hours Furthermore, recruitment of Shc to lipid rafts by introduction of the Ras membrane localization signal resulted in elevated and sustained ERK p42,44 MAPK activation, similar to that seen for Frs2 Whereas activation of ERK p42,44 MAPK by the normal Shc adaptor was less attenuated by lipid raft disruption, ERK p42,44 MAPK activation by ShcMLS was dependent on intact rafts, and when the rafts were disrupted, ERK p42,44 MAPK activation was almost completely absent On the basis of these results, we conclude that sustained ERK p42,44 MAPK signaling by Ret depends on intact lipid rafts

It is interesting to note from our in vivo data result-ing from the chick experiments that Shc activates Akt almost exclusively outside of rafts, with there being little ERK p42,44 MAPK activation either within or outside of rafts, as seen by its partitioning into the DRM fraction In contrast, whereas ShcMLS activates Akt mostly outside of rafts, its activation of ERK p42,44 MAPK was almost exclusively within the

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DRM raft fraction Frs2 activation of Akt was overall

very weak, whereas ERK p42,44 MAPK activation

was strong in both the DRM fraction and in the

non-raft SUP fraction Because our density-dependent

fractionation and colabeling of Ret with lipid rafts in

cellular staining both suggest that Ret associated with

Frs2 and Shc resides in different membrane

compart-ments, our results suggest that Akt activation may

result largely from nonraft activation of Shc by Ret

Unlike Akt, ERK p42,44 MAPK, which is activated

downstream of Frs2, appeared to localize both to raft

membranes and to nonraft membrane fractions

Because Ret association with Frs2 is almost exclusive

to the lipid rafts, this suggests that ERK MAPK

par-titioning into nonraft fractions is presumably initiated

from within the raft It is interesting that ERK p42,44

MAPK signaling resulting from ShcMLS association

with Ret takes place exclusively in the DRM fraction,

suggesting that, unlike signaling downstream of Frs2,

components of the ERK MAPK signaling pathway

stay associated with the raft-localized ShcMLS also

after activation

Both Shc and Frs2 have been implicated in cell

migration, and we cannot exclude a role also for

PI3K⁄ Akt signaling from Ret This pathway has

previ-ously been implicated in such cellular functions

mediated by Ret in, for example, kidney epithelial

Madin–Darby canine kidney cells [31] However, our

findings suggest that PI3K⁄ Akt signaling is not

suffi-cient for cell migration by itself in the absence of

MAPK ERK signaling via Frs2 Directional signaling

onto RTKs at the leading edge appears to be critical

for chemotaxis, and defines the direction of actin

poly-merization and subsequent cell migration It is not

clear how the cells measure gradients of RTK ligands

resulting in gradients of receptor activation along the

surface that are translated into polarization of the

cytoskeleton, extension of cell processes, and

eventu-ally translocation of the cell body Our results show

that Ret-mediated chemotaxis is critically dependent

on lipid rafts, whereas cell survival signaling via Shc is

not significantly affected by CO This is consistent with

the conclusion that Shc activation by Ret may take

place in nonraft membrane regions, similar to what

happens with many other tyrosine kinase receptors

[13,32], whereas Frs2, which is necessary for

Ret-elic-ited directional migration, is activated in raft-like foci

[11,18] Our results do not allow us to distinguish

whether the effects of CO on migration result directly

from the loss of ERK signaling or from the physical

localization of Ret to the growing axons, as seen by

the Ret colocalization with CTB As for other RTKs,

Ret-stimulated migration is dependent on ERK

signal-ing, as blocking of this pathway prevents Ret-induced migration [11,33] However, this does not exclude the possibility that that a raft-dependent localization of Ret to filopodia⁄ lamellipodia may also be important for directional migration and axonal extension Consis-tent with this hypothesis are data showing that local stimulation of cells expressing the epidermal growth fac-tor recepfac-tor with a bead soaked in epidermal growth factor leads to ERK activation spreading throughout the cell, whereas actin polymerization remains local [34] Our results open the possibility that Frs2-dependent recruitment of Ret receptors to lipid rafts upon ligand engagement may participate in increasing receptor levels

in the direction of increasing ligand concentrations This may provide a molecular mechanism for cellular amplifi-cation of ligand gradients that could play important roles in directed cell migration

Experimental procedures

Cell culture, DNA constructs, and mutagenesis All Ret mutants were harbored and expressed in PJ7W plas-mids and subcloned into peGFP vectors (Clonetech Inc., Mountain View, CA, USA) to make fluorescent constructs,

as described previously [11] SK-N-MC cells were main-tained in DMEM supplemented with 10% fetal bovine serum, 2% horse serum and 1 mm glutamine Starvations were done in DMEM containing 0.5% total serum All ligand stimulations were performed for 30 min unless stated otherwise, using 50 ngÆmL)1 recombinant human GDNF and 100 ngÆmL)1recombinant human GFRa1⁄ FC chimera (R&D Systems, Minneapolis, MN, USA) CO (Sigma, Munich, Germany) was used at 8 mm, as previously described [21]; specifically, cultured cells were incubated with

CO at 1.8 UÆmL)1for 1 h prior to and during ligand stimu-lation Anisomycin (Sigma) was applied to cells 30 min before ligand application at a final concentration of

12 lgÆmL)1, and cells were incubated for another 2 h before examination Transfections were performed using Lipofecta-mine LTX (Invitrogen, Karlsruhe, Germany), according to the manufacturer’s instructions Growth medium was replaced approximately 7 h after transfection Transfection efficiency was continuously monitored by eGFP fluores-cence

Antibodies and reagents Antibodies against Ret (Ret H-300), and phosphotyrosine (PY99) were obtained from Santa Cruz Biotechnology (Santa Cruz, CA, USA) Antibodies against HA tags were from BD Biosciences (CA, USA) Antibodies against Frs2 were from Sigma Antibodies against flotillin-1 were from Transduction Labs (Lexington, KY, USA) Antibodies

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against TfR were from Zymed (San Francisco, CA, USA).

Antibodies against Akt, phosphorylated Akt Ser473,

p44⁄ 42 MAPK and phosphorylated p44 ⁄ 42 MAPK were

from Cell Signalling (Hitchin, UK) Alexa-546-conjugated

GM1-CTB was from Molecular Probes (Leiden, the

Neth-erlands), and was used according to the manufacturer’s

instructions After staining with GM1-CTB, cells were

fixed in 4% paraformaldehyde and examined on

Zeiss LSM 5 exciter microscopes with axiovision software

(Zeiss, Karlsruhe, Germany)

Immunoblotting and SDS⁄ PAGE

Cells were lysed in Laemmli buffer for phospho-protein

blots Precipitated proteins from fractionations were eluted

by boiling in Laemmli buffer Proteins were fractionated on

polyacrylamide gels and immobilized on PVDF membranes

(GE Healthcare, Uppsala, Sweden) Western blot detection

was carried out by the enhanced chemiluminescence method

(GE Healthcare), according to standard darkroom

proce-dures Quantifications were done using imagej software

(http://rsb.info.nih.gov/ij)

Preparation of DRMs and lipid raft fractionation

DRM and SUP fractions were prepared by harvesting cells

in 1% Triton X-100 buffer for SK-N-MC cells and 0.9%

Triton X-100 buffer for chick spinal cord cells, as previously

described [14] Detergent-free fractionation of chick spinal

cord was done by electroporation of E2 (approximately

stage 11) chicken embryos in ovo Electroporator settings

were as described previously [35] The embryos were

incu-bated until E5 At E5, positively transfected spinal cord and

DRG segments were dissected out and pooled to equal

amounts of input (eight embryos were routinely needed per

experiment and condition) After dissociation into single

cells and stimuli with Ret ligands for 30 min, cells were

har-vested in detergent-free buffer and subjected to

ultracentrif-ugation in 0–20% OptiPrep density gradients as previously

described [24] Seven fractions of 0.68 mL each were taken

from columns after centrifugation, and concentrated protein

from each fraction was immunoblotted against proteins as

indicated in the figure legends

Vertical cell migration assays

Cell migration response towards Ret ligands was assessed

using transwell cell culture inserts (Falcon, Europe) with

12 lm pores Transfected cells were seeded (1–5· 105cells)

in the chambers and allowed to migrate towards ligands, as

indicated in the figures Quantification was done under a

microscope by counting cells in three visual fields after

removal of stationary cells on the upper side of membranes

using a cotton-tip

Comet assay SK-N-MC cells were treated as indicated in the figures Cells were spun down in ice-cold NaCl⁄ Piand immediately combined with low-melt agarose to a final concentration of 0.9% agarose The samples were immobilized on precoated agarose slides (Trevigen, Gaithersburg, MD, USA) and allowed to settle for 20 min at 4C The slides were immersed in lysis solution (Trevigen) for 55 min at 4C Slides were submersed in alkaline solution (NaOH⁄ EDTA⁄ H2O) with a pH > 13 for 50 min at room tempera-ture After equilibration in 1· TBE buffer three times for

5 min each, the slides were placed in a horizontal electro-phoresis chamber with a voltage of 0.9 VÆcm)1 for 14 min DNA was fixed in MeOH and EtOH and dried at room temperature Examination was performed by staining slides with SYBR-green, and images were captured at 200· mag-nification Quantification was done on digital images of 25 random cells per condition using tritek comet scoring software bridged to Mac OSX software via a Parallel Win-dows emulator

Acknowledgements

This work was supported by the Swedish Cancer Soci-ety, the Swedish Foundation for Child Cancer, the Swedish Medical Research Council and the Swedish Foundation for Strategic Research (CEDB and DBRM grants) for P Ernfors, the Karolinska Institute MD-PhD programme for T K Lundgren and the LERU Graduate Programme for A Stenqvist

References

1 Arighi E, Borrello MG & Sariola H (2005) RET tyro-sine kinase signaling in development and cancer Cyto-kine Growth Factor Rev 16, 441–467

2 Newgreen D & Young HM (2002) Enteric nervous sys-tem: development and developmental disturbances – part 2 Pediatr Dev Pathol 5, 329–349

3 Kramer ER, Aron L, Ramakers GM, Seitz S, Zhuang

X, Beyer K, Smidt MP & Klein R (2007) Absence of Ret signaling in mice causes progressive and late degeneration of the nigrostriatal system PLoS Biol 5, e39, doi: 10.1371/journal.pbio.0050039

4 Lundgren TK, Scott RP, Smith M, Pawson T & Ernf-ors P (2006) Engineering the recruitment of phosphoty-rosine binding domain-containing adaptor proteins reveals distinct roles for RET receptor-mediated cell survival J Biol Chem 281, 29886–29896

5 Rossi J, Tomac A, Saarma M & Airaksinen MS (2000) Distinct roles for GFRalpha1 and GFRalpha2 signal-ling in different cranial parasympathetic ganglia in vivo Eur J Neurosci 12, 3944–3952

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