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LETTERS
Pluripotentstemcellsinducedfromadultneuralstemcells by reprogramming with two factors
Jeong Beom Kim
1
*
, Holm Zaehres
1
*
, Guangming Wu
1
, Luca Gentile
1
, Kinarm Ko
1
, Vittorio Sebastiano
1
,Marcos J. Arau´zo-Bravo
1
, David Ruau
2
, Dong Wook Han
1
, Martin Zenke
2
& Hans R. Scho¨ler
1
Reprogramming of somatic cells is a valuable tool to understandthe mechanisms of regaining pluripotency and further opens upthe possibility of generating patient-specific pluripotent stemcells. Reprogramming of mouse and human somatic cells intopluripotent stem cells, designated as induced pluripotent stem(iPS) cells, has been possible with the expression of the transcrip-tion factor quartet Oct4 (also known as Pou5f1), Sox2, c-Myc andKlf4 (refs 1–11). Considering that ectopic expression of c-Myccauses tumorigenicity in offspring 
2
and that retroviruses them-selves can cause insertional mutagenesis, the generation of iPScells with a minimal number of factors may hasten the clinicalapplication of this approach. Here we show that adult mouseneural stem cells express higher endogenous levels of Sox2 andc-Myc than embryonic stem cells, and that exogenous Oct4togetherwitheitherKlf4orc-MycissufficienttogenerateiPScellsfrom neural stem cells. These two-factor iPS cells are similar toembryonicstemcellsatthemolecularlevel,contributetodevelop-ment of the germ line, and form chimaeras. We propose that, ininducing pluripotency, the number of reprogramming factors canbe reduced when using somatic cells that endogenously expressappropriate levels of complementing factors.
Mouse and human somatic cells can be reprogrammed into iPScells by the expression of a defined set of factors (
Oct4 
,
Sox2 
,
c-Myc 
and
Klf4 
, as well as
Nanog 
and human
LIN28 
)
1–11
. It is possible togenerate iPS cells from mouse and human fibroblasts in the absenceof 
c-Myc 
retrovirus
6,7
,andthereforeitwassuggestedthatendogenousexpression ofc-Myccouldhavearoleinthereprogrammingprocess.Neural stem cells (NSCs) endogenously express Sox2, which may function in maintaining the stemness and multipotency of NSCs
12,13
, and Sox2 was suggested in maintaining cellular pluripo-tencybyregulatingtheexpressionofOct4(ref.14).NSCswereestab-lished from adult Oct4–GFP (OG2)/ROSA26 heterozygoustransgenic mouse brains
15–17
, expressing green fluorescent protein(GFP) under the control of the
Oct4 
promoter (Oct4–GFP) andthe
Escherichia coli lacZ 
transgene from the constitutive
ROSA26 
locus (
ROSA26 lacZ 
). The established NSCs had the capacity forself-renewal and multipotency (Supplementary Fig. 1).Compared to embryonic stem cells (ESCs), expression of 
Sox2 
wasapproximately twofold higher in NSCs.
c-Myc 
and
Klf4 
were alsoexpressed at levels about tenfold higher and eightfold lower inNSCs than in ESCs, respectively (Fig. 1a). Western blot analysesshowed that the relationship between protein and RNA levels inNSCs corresponded to that in ESCs for Oct4, Sox2 and Klf4; thec-Myc protein level was comparable in NSCs and ESCs (Fig. 1b).In this study, we attempted to reprogram NSCs into iPS cells by introducing different combinations of the four factors
Oct4 
,
Sox2 
,
c-Myc 
and
Klf4 
(Supplementary Table 1) using the retroviral MX vector system. We assessed the ability of 15 different transcriptionfactorcombinationstoinduceOct4–GFP-positivecolonyformation.Sixcombinationswereabletoinducethegeneration ofiPScellsfromNSCs, as judged by the formation of GFP
1
colonies and the estab-lishment of iPS cell lines. We observed GFP
1
cells 4days after trans-ductionwiththecombinationcontainingallfourfactors—thatis,thecontrolcombination—andnotedagradualincreaseinthenumberof GFP
1
colonies during the first 2 weeks post-infection(Supplementary Fig. 2a). We established four-factor iPS cells fromGFP
1
ESC-likecoloniesonday14.Thesefour-factoriPScellsstainedpositive for stage-specific embryonic antigen-1 (SSEA-1) and alkal-ine phosphatase (Supplementary Fig. 2b), showed messenger RNAexpression patterns similar to those in ESCs (Fig. 2a, Supplementary Fig. 2c), and led to teratoma formation on injection into nude mice(Supplementary Fig. 2d). Our results demonstrate that NSCs can bereprogrammed into iPS cells by the four transcription factors: Oct4,Sox2, c-Myc and Klf4.Three different combinations of three factors were also capable of generating iPS cells from NSCs:
Oct4 
,
Klf4 
and
c-Myc 
(OKM);
Oct4 
,
Klf4 
and
Sox2 
(OKS); and
Oct4 
,
c-Myc 
and
Sox2 
(OMS;Supplementary Table 1). We did not observe GFP
1
colonies forthe three-factor combinations that did not include Oct4. GFP
1
col-onies were observed 1week after transduction with the OKM com-bination (without
Sox2 
). However, GFP
1
colony formation wasobserved only after 14–21days with the OKS combination (without
c-Myc 
), and was even more delayed with the OMS combination(without
Klf4 
; Supplementary Table 1). Nonetheless, these OKM,OKS and OMS three-factor iPS cells had similar gene expressionprofilestoESCs(SupplementaryFig.3a),andalltypesofthree-factoriPS cells differentiated into all three germ layers (Supplementary Fig.3b). Taken together, these results indicate that three-factor iPS cellscould be generated in the absence of 
Sox2 
,
Klf4 
or
c-Myc 
retrovirusesin NSCs, which endogenously express these three factors.We next assessed the ability of two-factor combinations to inducethe generation of iPS cells from NSCs. Only two combinations weresuccessful in reprogramming NSCs. We first observed GFP
1
coloniesinNSCculturesinfectedwith
Oct4 
and
Klf4 
(OK)and1–2weekslaterin those infected with
Oct4 
and
c-Myc 
(OM; Supplementary Table 1).The two-factor OM iPS cells showed an ESC-like gene expressionpattern and contributed to the three germ layers in teratomas(Supplementary Fig. 3a, b). Klf4 and c-Myc may exert similar func-tions.ItisknownthatKlf4hasaroleintheinactivationofthe
 p53
(alsoknownas
Trp53
)tumour-suppressorgene,whichleadstocellimmor-talization; Klf4 also works in conjunction with the RAS
V12
oncogenicsignal transduction protein to stimulate cellular proliferation
18,19
.
*
These authors contributed equally to this work.
1
Department of Cell and Developmental Biology, Max Planck Institute for Molecular Biomedicine, Ro¨ntgenstrasse 20, 48149 Mu¨nster, NRW, Germany.
2
Institute for BiomedicalEngineering, Department of Cell Biology, RWTH Aachen University Medical School, Pauwelsstrasse 30, 52074 Aachen, NRW, Germany.
1
 ©2008
Macmillan Publishers Limited. All rights reserved
 
Similarly,theimmortalizinggeneproductc-Myc,inconjunctionwithmutant RAS, exhibits an oncogenic effect
20
. It has been reported thatc-Myc increases telomerase activity in NSCs, a mechanism possibly responsible for the immortalization of NSCs
21
. Because c-Mycincreases tumorigenicity in chimaera pups
2
, the recent studies dem-onstrating iPS cell generation without the
c-Myc 
retrovirus
6,7
repre-sentasignificantfinding.Importantly,ourresultsofinducingiPScellsfromNSCswith
Oct4 
and
Klf4 
andwithout
c-Myc 
bringusevencloserto the generation of iPS cells suitable for transplantation.We compared two-factor OK iPS cells with four-factor iPS cellsand ESCs. On day 14 post-infection, five GFP
1
colonies were dis-sociated and propagated under ESC culture conditions (Fig. 1c, f), yielding three (that is, 60%) two-factor OK iPS cell clones (B-2, D-7and F-4) that were morphologically indistinguishable from ESCs(Fig. 1d, g). No colonies formed from NSCs infected with controlvirus(MX)(Fig.1e,h).WeestimatedthereprogrammingefficienciesfromthenumberofOct4–GFP
1
coloniesandtransductionrateswithMX–GFP control virus on NSCs for the two-factor OK iPS and thefour-factor iPS by time course (Fig. 1i, j). Thereby, we calculated areprogramming efficiency of 3.6% for four-factor reprogramming of NSCsand0.11%forthetwo-factorapproach,whichiscomparabletoreprogramming of fibroblasts with selection (
,
0.08%)
1–3
andwithout selection (0.5%)
5
(Fig. 1j and Supplementary Table 2).Transduction with all four factors had a positive impact on the tim-ingandnumberof GFP
1
colonies.Integration oftheviral transgeneswas confirmed by genotyping by polymerase chain reaction (PCR;Supplementary Fig. 4). The viral transgenes of all four factors weredetectedinfour-factoriPScells,whereastwo-factorOKiPScellsonlcontained the
Oct4 
and
Klf4 
transgenes.Two-factor OK iPS cells stained positive for SSEA-1 and alkalinephosphatase (Supplementary Fig. 5), and exhibited expression pat-ternsofESCmarkergenes similar to those infour-factor iPS cellsandESCs (Fig. 2a). Quantitative real-time PCR (qRT-PCR) resultsdemonstrated that expression of endogenous
Oct4 
,
Sox2 
,
c-Myc 
and
Klf4 
in two-factor OK iPS cells was comparable to that in ESCs(Supplementary Fig. 6a), and showed the silencing of the viral tran-scripts in two-factor OK iPS cells with a 1,000-fold reduction after 30days(SupplementaryFig.6b).Inaddition,infour-factoriPScells,theendogenous expression levels were similar to those in ESCs, and theexpression of the transgenes was completely silenced (Supplementary Fig. 6c, d). Global gene expression of two-factor iPS also clusteredclose to ESCs and four-factor iPS (Fig. 2b and Supplementary Fig. 7).ScatterplotsofDNAmicroarrayanalysesdemonstratedahighersimi-larity between two-factor iPS cells and ESCs than between two-factor
a
020406080
   G   F   P
  +
   c  o   l  o  n  y  n  u  m   b  e  r
Two factorsFour factors
Day 7Day 14Day 21
i
Oct4NanogKlf4Sox2c-Myc
 b
-actin
   E   S  C  s   N   S  C  s
Oct4Klf4Sox2c-Myc
β
-actin
   E   S  C  s   N   S  C  s
b
Four factorsTwo factors (Oct4 + Klf4)GFP
+
colonyGFP
+
colonyDay 77 ± 3 0 ± 0 0 ± 0Day 1429 ± 71.45 ± 0.30.35 ± 0.15 ± 2Day 2173 ± 113.6 ± 0.511 ± 20.11 ± 0.02
 j
Efficiency (%)Efficiency (%)0.05 ± 0.02
Day 14Day 30
Oct4GFPOct4GFPControlMock
NSCs
dgeh
c
f
Oct4Klf4Sox2
c-
Myc
   G  e  n  e  e  x  p  r  e  s  s   i  o  n  r  e   l  a   t   i  v  e   t  o   E   S   C  s
10
2
10
0
10
–2
10
–4
10
–6
Figure 1
|
Generation of two-factor Oct4/Klf4 (OK) iPS cells from adultNSCs of OG2/ROSA26 transgenic mice. a
, RT–PCR and qRT–PCR analysesof 
Oct4
,
 Nanog 
,
Klf4
,
Sox2
and
c-Myc 
inESCsandNSCs(
n
5
3;errorbars indicate s.d.).
b
-actin
was used as a loading control.
b
, Western blotanalysesofthe fourfactors inESCsandNSCs.
A
nti-actin antibodywas usedas a loading control.
c
, Morphology of two-factor OK iPS cell colony on day 14 post-infection. Shown is an ESC-like colony expressing Oct4–GFP(
f
).
d
,
g
, Morphology of established two-factor OK iPS cells (clone F-4) onday 30 post-infection, grown on irradiated MEFs. Phase contrast (
d
) andOct4–GFP (
g
) are shown.
e
,
h
, Morphology of NSCs and mock infection onday30post-infection.
i
,GenerationofGFP-positivecoloniesatday7,14and21 after two-factor OK and four-factor infection (
n
5
3; error bars indicates.d.).
j
, Reprogramming efficiency of generating two-factor and four-factoriPS cells (
n
5
3). Indicated are the total numbers of GFP
1
colonies per50,000 plated NSCs on day 7, 14 and 21 after infection.
LETTERS
NATURE
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 ©2008
Macmillan Publishers Limited. All rights reserved
 
iPS cells and NSCs (Fig. 2c, d). Thus, two-factor iPS cells (clone F-4)seemed to be very similar to mouse ESCs at the global transcriptionlevel.The differentiation ability of two-factor OK iPS cells was con-firmed by 
in vitro 
differentiation into embryoid bodies. These cellsexpressed the ectoderm (Tuj1), endoderm (
a
-fetoprotein) andmesoderm (Flk1) markers (expressed by beating cells mimickingcardiomyocytes; Fig. 3a and Supplementary Video 1). Teratomascontained derivatives of all three germ layers (Fig. 3b) and expressedmarkers of the three germ layers (Supplementary Fig. 8). No tera-toma had formed from donor cells (NSCs). These data demonstratethat two-factor OK iPS cells exhibit a pluripotent phenotype
in vitro 
and
in vivo 
.To investigate their developmental potential, two-factor OK iPScells were aggregated with 8-cell-stage embryos. iPS cells had con-tributed to the formation of the inner cell mass in developing blas-tocysts (Fig. 4a). After transferring aggregated blastocysts intopseudopregnant females, we obtained 16 live embryos on embryonicday (E)13.5, 2 of which showed germ cell contribution in the foetalgonads,judged fromOct4–GFP expression (Fig. 4b).
b
-galactosidasestaining (visualizing the NSC donor cells that carry the
ROSA26 lacZ 
gene) of embryonic tissue from whole embryos revealed that in theresulting chimaeras two-factor OK iPS cells contributed to thedevelopment of all three germ layers (Fig. 4c, e). The strictest testfor developmental potency, tetraploid (4
) embryo aggregation(
5
122), resulted in 2 dead (arrested) embryos at E13.5 (Fig. 4d).These data demonstrate that iPS cells can give rise to all of the tissuesof a late-stage embryo. In diploid (2
) aggregation, PCR genotypingshowed that 2 out of 13 chimaeras were positive for the Oct4–GFPallele of donor cells (Fig. 4f, g, top panel). To assess whether two-factor OK iPS cells can contribute to the germ line, chimaeras weremated with CD-1females.Two outof 12pups hada Oct4–GFP alleleand 1 out of 12 mice had a
lacZ 
allele, because the donor cells arederived from a heterogeneous mouse (
Oct4-GFP 
1
/
2
ROSA26 
1
/
2
),
a
Oct4NanogSox2Fgf4ErasCfc1 Zfp42Utf1Dppa5a
 b
-actin
NSCsESCs2F iPS4F iPS B-2D-7F-4
c db
NSC 2F iPS 4F iPS ESC246810121416246810121416ESC
   2   F   i   P   S
Oct4Sox2NanogKlf4c-MycLin28
246810121416246810121416NSC
   2   F   i   P   S
Oct4
 
Sox2NanogKlf4c-MycLin28
Figure 2
|
Gene expression profile of iPS cells.a
, RT–PCR analysis of ESC marker geneexpression in ESCs, four-factor (4F) iPS cells(cloneA-2c),two-factor(2F)OKiPS cells(clonesB-2,D-7andF-4), andNSCs.Primersarespecificfor transcripts from the respective endogenouslocus.
b
-actin
was used as a loading control.
b
, The heat map of the different expressed genesamong the NSC, 2F (OK) iPS, 4F iPS and ESCs.Thegene hierarchical cluster was performedwitha cityblock distance and an average linkage. Red,high gene expression; blue, low gene expression.
c
,
d
, Global gene expression patterns werecompared between 2F iPS cells (clone F-4) andESCs(
c
),andbetween2FiPScells(cloneF-4)andNSCs with DNA microarrays (
d
). Black linesindicatetwofoldchangesingeneexpressionlevelsbetween the paired cell types. Genesoverexpressed in 2F iPS cells (clone F-4)compared with NSCs or ESCs are shown in blue;thoseunderexpressedareshowninred.Positionsof pluripotency genes
Oct4
,
Nanog 
,
Sox2
,
c-Myc 
,
Klf4
and
Lin28
in scatter plots are indicated. Thegene expression levels were normalized with theRMA algorithm
26
.
a
EctodermTuJ1 AFPFlk1TuJ1 / DAPINeural rosetteColumnar epitheliumMuscle AFP / DAPIFlk1 / DAPI
b
EndodermMesoderm
Figure 3
|
Two-factorOct4/Klf4 (OK)iPS cells(clone F-4)arepluripotentanddifferentiate
in vitro
and
in vivo
. a
,
In vitro
differentiation into all threegerm layers. After embryoid body formation, aggregates were transferredonto gelatine-coated plates and allowed to differentiate for another 10days.Cells were stained with anti-Tuj1, anti-
a
-fetoprotein (AFP) or anti-Flk1.Nuclei were stained with 4,6-diamidino-2-phenylindole (DAPI).
b
, Teratomas of F-4 iPS cells containing all three germ layers. F-4 iPS cells(1.5
3
10
6
cells) were subcutaneously inoculated into nude mice. After 4 weeks,teratomaswerestainedwithhaematoxylinandeosindyes.Shownisateratoma containing a neural rosette (ectoderm), columnar epithelium(endoderm) and muscle (mesoderm).
NATURE
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Macmillan Publishers Limited. All rights reserved
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