ARTICLES
10 VOLUME 18 | NUMBER 1 | JANUARY 2015 NATURE NEUROSCIENCE
Mechanotransduction, the conversion of mechanical force into
electrochemical signals, is the basis for several sensory phenomena,
including hearing, balance and touch sensation. Touch receptors are
highly specialized sensory neurons that innervate the skin and con-
fer the capacity to discriminate shape and fine texture of objects
1
.
In vivo, touch receptors, also referred to as low-threshold mechano-
receptors (LTMRs), are intermingled with sensory neurons finely
tuned to detect a variety of other thermal and chemical stimuli
2
.
Unlike LTMRs, nociceptors are transducers of high-threshold
noxious and painful stimuli and thus constitute a separate category
of sensory neurons. These functional differences correlate with the
expression of different molecular markers
3
.
The heterogeneity of sensory ganglia and very low abundance of
modality-specific sensory neuron subtypes in vivo have hampered
efforts to isolate and characterize them. Both human nociceptors and
LTMRs are inaccessible for functional characterization.
Recent studies describe the generation of nociceptors from hES
cells by use of small-molecule inhibitors
4,5
, opening up new possi-
bilities for studying nociceptor function and deriving new cellular
disease models relevant for pain therapy. However, no in vitro model
of human LTMRs has been described, and neuronal mechanisms
that mediate rapid transduction of small mechanical stimuli have
remained largely elusive.
Two molecules implicated in sensing mechanical force in differ-
ent cellular contexts are the large transmembrane proteins Piezo1
and Piezo2 (refs. 6–9). Piezo2 is expressed in both nociceptors and
LTMRs and is also found in epidermal Merkel cells that are part
of terminal touch-receptive specializations
6,8–10
. Indeed, Merkel
cells modulate and tune touch responses of LTMRs in a Piezo2-
dependent manner, as shown by specific and restricted Merkel cell
knockout and knockdown studies
8–10
. These studies clearly show that
Piezo2 conveys mechanical sensitivity to epidermal Merkel cells, but
touch responses are not abolished in these mouse models, and it is
currently not known whether Piezo2 is a transducer of mechanical
stimuli in LTMRs, nociceptors or both.
To examine human touch receptor function, we recapitulated sensory
neuron development in vitro and established a multistep differentiation
protocol to generate LTMRs via the intermediate production of hES
cell–derived neural crest (NC) cells, the sensory neuron progenitors
3,11
.
The resulting neurons express a highly distinct set of LTMR-specific
genes. They also convert mechanical stimuli into electrical signals, their
most salient characteristic in vivo. The differentiation procedure is not
only effective in hES cells but also facilitates the conversion of hiPS
cells into LTMRs. Additionally, we find that viral delivery of NGN2,
a key transcription factor in sensory neuron development, promotes
differentiation and directs a larger fraction of progenitor cells to adopt
LTMR fate. Finally, we demonstrate that Piezo2 expression is required
for mechanotransduction of hES cell–derived LTMRs.
RESULTS
hES cell–derived NC cells generate sensory neurons in vitro
In vivo, peripheral sensory neurons derive from multipotent NC cells.
During development, NC cells delaminate from the neural tube and
generate functionally distinct subpopulations of dorsal root ganglion
(DRG) neurons in consecutive waves
11,12
. Inducing hES cells to form
neuroectodermal spheres mimics key aspects of neural tube formation
1
Department of Pharmacology, University of Heidelberg, Heidelberg, Germany.
2
Max Delbrück Center for Molecular Medicine, Berlin, Germany.
3
Skin Cancer Unit,
German Cancer Research Center (DKFZ), Heidelberg and University Medical Center Mannheim, Mannheim, Germany. Correspondence should be addressed to
J.S. (jan.siemens@pharma.uni-heidelberg.de).
Received 6 October; accepted 13 November; published online 3 December 2014; doi:10.1038/nn.3894
PIEZO2 is required for mechanotransduction in human
stem cell–derived touch receptors
Katrin Schrenk-Siemens
1
, Hagen Wende
1
, Vincenzo Prato
1
, Kun Song
1
, Charlotte Rostock
1
, Alexander Loewer
2
,
Jochen Utikal
3
, Gary R Lewin
2
, Stefan G Lechner
1
& Jan Siemens
1
Human sensory neurons are inaccessible for functional examination, and thus little is known about the mechanisms mediating
touch sensation in humans. Here we demonstrate that the mechanosensitivity of human embryonic stem (hES) cell–derived touch
receptors depends on PIEZO2. To recapitulate sensory neuron development in vitro, we established a multistep differentiation
protocol and generated sensory neurons via the intermediate production of neural crest cells derived from hES cells or human
induced pluripotent stem (hiPS) cells. The generated neurons express a distinct set of touch receptor–specific genes and convert
mechanical stimuli into electrical signals, their most salient characteristic in vivo. Strikingly, mechanosensitivity is lost after
CRISPR/Cas9-mediated PIEZO2 gene deletion. Our work establishes a model system that resembles human touch receptors,
which may facilitate mechanistic analysis of other sensory subtypes and provide insight into developmental programs underlying
sensory neuron diversity.
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