other and in this way form self-reinforcing
networks that issue outputs to interneurons, motor neurons, secretomotor neurons
and vasodilator neurons (1—3). IPANs thus provide the ENS with the kind of information
that this “brain of the gut” requires for its autonomic control of digestion.
The IPANs are complemented by two groups of
extrinsic sensory neurons,
vagal and spinal afferents, which convey information from the gut to the brain.
In addition, some of them serve an efferent-like function by releasing neuropeptide
transmitters such as calcitonin gene-related peptide and tachykinins (substance
P, neurokinin A) from their peripheral endings, these transmitters in turn influencing
the activity of enteric neurons and GI effector systems (4). The vagal and spinal
afferents originate from somata in the nodose and dorsal root ganglia (
Fig.
1), respectively, and differ in a number of neurochemical and functional
properties (5, 6). Importantly, 80—90% of the axons in the vagus nerves are
afferent nerve fibres that project to the nucleus tractus solitarii and area
postrema of the brainstem (7—9). The spinal afferents supplying the gut terminate
predominantly in distinct laminae of the dorsal spinal cord where they are organized
in a segmental manner but, unlike those of somatic afferents, distributed over
several spinal segments (10, 11).
Except for particular spatial arrangements in the myenteric plexus and muscle
(9, 12), the visceral endings of the vagal and spinal afferents have no end
organs or morphological specializations. Associated mostly with nonmyelinated
and some thinly myelinated axons, the extrinsic sensory nerve fibres supply
mucosa, submucosa (particularly arterioles), muscle, myenteric plexus and serosa
(4, 7, 8, 9, 12, 13).
With these projections and their sensory modalities, they can respond to changes
of the chemical environment in the lumen, interstitial space and vasculature
and to mechanical distortion of the gut wall, typically distension, but also
to contraction or relaxation of the muscle (4, 7—9, 12, 13).
The major task of extrinsic afferents is to notify the central nervous system
about processes and conditions that are relevant to energy and fluid homeostasis
of the body. Therefore, they participate in autonomic and neuroendocrine reflex
circuits, but the information which they convey to the brain is rarely perceived
as a conscious sensation, at least under physiological conditions (14). In a
number of FBDs, however, patients suffer from GI pain and discomfort, and there
is now ample evidence that hypersensitivity of the extrinsic afferent system
is an important factor in the complaints of these patients (15).
Sensory neuron interactions with enteroendocrine cells
Dispersed among the epithelial cells, EECCs of
the GI mucosa produce a variety of digestive hormones. Many of these cells are
in close proximity to nerve fibres which, on the one hand, may regulate the
activity of EECCs and,.on the other hand, be targets of the hormones released
from EECCs (16—18). Experimental evidence indicates that EECCs function as detectors
that analyze the luminal contents, survey the mucosal status and hence act like
“taste buds” of the gut. By releasing hormones they not only contribute to the
endocrine regulation of digestion but also serve as interface between the GI
lumen and sensory neurons in the lamina propria of the mucosa.
The enterochromaffin cells are the major source of 5-hydroxytryptamine (5-HT)
in the body, contributing more than 80% to the total amine content (19). These
cells release 5-HT in response to certain constituents of food, mechanical distortion
of the mucosal villi (16), bacterial products such as cholera toxin (20), cytostatic
drugs such as cisplatin (19) and mucosal injury (21). 5-HT in turn activates
intrinsic and extrinsic sensory nerve fibres
via interaction with distinct
5-HT receptors. The 5-HT receptors on intrinsic sensory neurons of the
submucosal
plexus are predominantly of the 5-HT
1P and
5-HT
4 type (22), while those on intrinsic sensory
neurons of the
myenteric plexus (23) and on extrinsic afferents are preferentially
of the 5-HT
3 type (19, 24).
Stimulation of 5-HT
3 receptors on vagal afferents
elicits emesis, which explains why vomiting, caused by cytostatic drugs that
release 5-HT from EECCs, is prevented by 5-HT
3
receptor antagonists such as ondansetron (19). 5-HT
3
receptors on spinal sensory neurons are involved in the afferent signalling
of colorectal distension (25), but the use of 5-HT
3
receptor antagonists in controlling GI sensitivity is limited because 5-HT3
receptors are also present on various neurons of the ENS (23, 26). Accordingly,
5-HT
3 receptor antagonists inhibit peristalsis
in the guinea-pig intestine (27) and cause constipation in humans (28).
Cholecystokinin (CCK) is released from the duodenum in response to products
of fat and protein digestion (17). The peptide, in turn, stimulates extrinsic
vagal afferents involved in satiation, reflex inhibition of gastric motility
and emptying, reflex increase in gastric blood flow and mucosal protection (29—34).
CCK-evoked excitation of vagal sensory neurons is mediated by CCK
1
(CCK
A) receptors, which is consistent with the
expression of this receptor type on nodose ganglion afferents of the rat (35,
36). CCK
1 receptor antagonists hold some potential
in the treatment of gastro-oesophageal reflux disease and functional dyspepsia
(37), given that CCK
1 receptors appear to be
involved in the transient lower oesophageal sphincter relaxations induced by
gastric distension (38) and in the meal-like fullness and nausea associated
with intraduodenal lipid and gastric distension (39).
Gastric acid entering the duodenum releases secretin from endocrine S cells
in the proximal small intestine to enhance pancreatic exocrine secretion and
bile flow. In addition, secretin leads to stimulation of vagal afferent neurons
and subsequent inhibition of gastric contractility and emptying
via a
vago-vagal reflex (40). Accordingly, the neural reflex inhibition of gastric
motility in 508.response to duodenal acidification seems to be initiated, in
part, by secretin (41). Somatostatin, a peptide released from endocrine D cells
may exert an inhibitory effect on extrinsic afferents because octreotide, a
long-acting analogue of somatostatin, has been found to reduce perception of
gastric and rectal distension (37). Furthermore, corticotropin-releasing factor
released in the GI mucosa from EECCs or immune cells may increase extrinsic
afferent nerve activity, as has been suggested by a study of the perception
of rectal distension in human volunteers (42).
Sensory neuron interactions with GI immune cells
Besides sensory neurons and EECCs, immune cells constitute
another major defence system that oversees the integrity of the digestive tract.
As the human GI mucosa extends for an area of 200—300 m
2
and is home to some 10
13 bacteria and other microorganisms
which may threaten to invade and translocate the gut wall (43), the alimentary
canal possesses a highly specialized immune system that contains organized and
nonorganized cellular elements (18, 44). The gut-associated lymphoid tissue
comprises antigen-sampling M cells, lymphocytes that may either occur in aggregates
(such as in Peyer’s patches) or lie loosely scattered in the epithelium and
lamina propria, and immune-associated cells including macrophages, eosinophils,
neutrophils, and mast cells. In addition, many epithelial cells are able to
secrete chemokines (e.g., interleukin-8) and thus to recruit immune cells (45).
The GI immune system is called into operation whenever the mucosa is affected
by microbial infection, allergen exposure, inflammation or other types of injury.
Following their release from activated immune cells, cytokines, prostaglandins
(PGs), leukotrienes, bradykinin, histamine, 5-HT and proteases can either acutely
excite sensory nerve fibres or alter their sensitivity in the long term.
Vagal afferent neurons play a role in the communication between the peripheral
immune system and the central nervous system. Thus, intravenous injection of
interleukin-1ß (IL-1ß) causes increased firing in vagal afferents
(46) and activation of neurons in the brainstem (47). Since IL-1
1
receptors are expressed by nodose ganglion cells, IL-1ß may excite vagal
afferents by a direct action on the axons although PG formation and activation
of PG receptors on vagal sensory neurons could also contribute (48). In addition,
IL-1ß increases the sensitivity of gastric vagal afferents to fire in
response to CCK, and there is information that CCK acting
via CCK
1
receptors mediates part of the excitatory action of IL-1ß on vagal afferents
(46).
Consistent with these effects of peripheral IL-1ß is the concept that
vagal afferents participate in the behavioural responses to infection and inflammation,
which in the rat comprise fever, anorexia, somnolence, decrease in locomotor.activity,
decrease in social exploration and hyperalgesia (49, 50). This “sickness behaviour”
is initiated by cytokines and can be reproduced by peripheral administration
of IL-1ß IL-6, tumour necrosis factor-alpha or bacterial endotoxin which
induces IL-1ß. Although proinflammatory cytokines can access the brain
via circumventricular organs that are devoid of a blood-brain barrier,
experiments involving subdiaphragmatic vagotomy have shown that the cytokine-evoked
decrease in locomotor activity, decrease in social exploration and hyperalgesia
depend in part on afferent signalling by vagal sensory neurons (49—51).
IL-1ß also sensitizes splanchnic afferents to the excitatory effects of
mesenteric ischaemia and histamine and, at high dosage, excites them
via
stimulation of IL-1
1 receptors (52). These cytokine
effects on extrinsic afferents may be of relevance to a number of GI diseases
in which there is an activation of the immune system with subsequent release
of cytokines. In addition, it needs to be considered that IL-1ß , IL-6
and/or tumour necrosis factor-alpha are formed in response to ischaemia (52),
acid-induced gastric mucosal injury (53, 54) and surgical trauma (55). Thus,
it may be in a wide range of pathological circumstances that cytokines come
into play and modify the activity of afferent neurons.
Following intestinal anaphylaxis, vagal and spinal afferents in the rat are
stimulated
via histamine acting at H1 receptors and 5-HT acting at 5-HT
3
receptors (56—58). It can be anticipated that histamine and 5-HT are released
from mast cells under these conditions and that, by stimulating extrinsic afferents,
they contribute to anaphylaxis-evoked disturbances of intestinal motor activity
(59) and cardiovascular reflex responses (60). Apart from anaphylaxis, ischaemia
is another condition that causes release of 5-HT and histamine, these amines
contributing to the ischaemia-evoked discharge of sensory neurons (56, 61).
Further mediators that influence afferent nerve activity include serine proteases
such as mast cell tryptase, trypsin and thrombin. These proteases activate a
particular group of cell surface receptors termed proteinase-activated receptors
(PARs) through proteolytic cleavage of the extracellular N-terminal domain of
the receptors and exposure of a new N-terminal domain that acts as “tethered
ligand” and thus causes self-activation of the receptors (62). Of the 4 PARs
identified thus far, PAR-1 and PAR-2 are expressed by dorsal root ganglion neurons,
and a PAR-2 agonist elicits excitation of afferent axons in jejunal mesenteric
nerves (62).
PGs are key mediators of inflammatory hyperalgesia, which is in keeping with
the expression of prostaglandin EP and IP receptors on primary sensory neurons.
PGE
2 excites mesenteric afferent nerve fibres
supplying the rat jejunum through direct activation of EP
1
receptors on the axons and through prostanoid-mediated contraction of the bowel
(63). In addition, PGs sensitize visceral afferents to other algesic substances
such as bradykinin which per se.increases the discharge of serosal afferents
from the rat jejunum by activation of bradykinin B
2
receptors (64).
Sensory neuron activation by contraction and distension
The GI tract with its interstitial cells of Cajal
as pacemaker cells is continuously moving, and the associated changes in the
mechanical status of the gut are recorded by both intrinsic and extrinsic afferent
neurons. The mechanosensitivity of IPANs enables the ENS to react to distortion
of the mucosal villi and to distension of the gut wall and thus to regulate
GI motility, secretory activity and vascular perfusion according to the digestive
needs. Both intrinsic enteric and vagal mechanosensors contribute to the adaptive
relaxation of the gastric fundus in response to distension by food intake (65,
66).
Distension of the stomach or the colorectal region beyond a certain level gives
rise to pain which is thought to be mediated by spinal afferents (7, 8, 13).
Although some of the mechanosensitive visceral afferents are high-threshold
sensors, as is typical of somatic nociceptors, most of them are low-threshold
sensors which, however, encode distension or other mechanical stimuli over a
wide range of innocuous and noxious intensities (11, 67). Both populations of
visceral mechanosensors can sensitize under conditions of inflammation (11),
in which case normal distension levels may be encoded at an intensity that causes
visceral discomfort. Alternatively, if there is pseudo-obstruction of the bowel,
postoperative or mechanical ileus, distension intensities may quickly turn into
the noxious range and hence give rise to autonomic reflexes, neuroendocrine
responses and pain.
Sensory neuron activation by GI mucosal injury
Among the bacterial toxins that are most injurious
to the GI mucosa are those produced by
Clostridium difficile. The diarrhoea,
inflammation and necrosis caused by Clostridium toxin A involve, at an early
stage, spinal afferents that release tachykinins which,
via tachykinin
NK
1 receptors, stimulate enteric secretomotor
neurons but also contribute to mast cell degranulation, macrophage activation
and neutrophil infiltration (68—70). Although the mechanism whereby Clostridium
toxin A activates sensory neurons remains to be disclosed, it is known that
the toxin induces epithelial cells to release macrophage-inflammatory protein-2
and other chemokines which may not only induce an inflammatory reaction on their
own but,
via unknown links, also lead to stimulation of sensory neurons
(71). Cholera toxin, to the contrary, acts on enterochromaffin cells to release
5-HT which subsequently stimulates electrolyte and fluid secretion mostly by
an enteric secretomotor reflex (72).
There is a body of evidence that experimental damage of the GI mucosa leads
to activation of spinal afferent neurons which through their efferent-like function
signal for local protective measures in the mucosa or through their afferent
function activate autonomic and neuroendocrine mechanisms of homeostasis (4).
Hydrochloric acid and pepsin are highly aggressive secretions of the stomach
which attack the mucosal tissue if they can overwhelm the epithelial barrier.
This is thought to take place when the mucosal barrier is focally disrupted
by the mechanical forces of digestion, by ingested alcohol, nonsteroidal anti-inflammatory
drugs or irritant food, or by reflux of bile. The surge of acid intruding the
lamina propria stimulates spinal afferents which via a peripheral mechanism
of action increase blood flow through the gastroduodenal mucosa and initiate
other mechanisms of defence (4). Acid-sensitive extrinsic afferents thus represent
a neural emergency system that can be activated by a variety of injurious chemicals
(4). This alarm system also operates in the human gastric mucosa (73) and in
the mucosa of the small and large intestine of experimental animals (74).
While local protective measures in the rat gastric mucosa are initiated by spinal
afferents (4), it is vagal afferents that signal an acute acid challenge of
the rat gastric mucosa to the central nervous system (75, 76). It would hence
seem that vagal and spinal afferents are specialized to mediate completely different
homeostatic reactions to a noxious acid insult of the gastric mucosa (77). Although
vagal afferents in the stomach have long been known to discharge action potentials
when their peripheral terminals are exposed to acid (78), their molecular detector
of H
+ ions remains unknown. Since the acid-evoked
afferent signalling is not altered by pretreatment with the vanilloid capsaicin
(75), it appears improbable that acid transduction is accomplished by the vanilloid
receptor of type 1, which is a polymodal detector of a variety of stimuli including
H
+ (79). Whether acid-sensing ion channels (80)
are involved has not yet been explored. It likewise awaits to be examined whether
the homomeric P2X
2 and heteromeric P2X
2/3
purinoceptor cation channels expressed by afferent neurons (81) are transducers
of GI mucosal damage. Vagal afferents expressing these receptors are activated
or sensitized by adenosine triphosphate which in the injured gut may be released
from damaged mucosal cells, immune cells, endothelial cells as well as sensory,
enteric and sympathetic nerve endings (81, 82). Although 5-HT is released from
the acid-injured mucosa (21), this amine has been ruled out to contribute to
the acid-evoked excitation of vagal afferents (57).
The involvement of vagal afferents in the central signalling of a gastric mucosal
acid challenge (75, 76) and peripheral immune challenge (49, 50) is of obvious
relevance to understanding visceral sensation in health and disease. After it
has long been held that vagal sensory neurons do not play any role in visceral
pain, there is now growing awareness that these neurons make a distinct contribution
to the emotional-affective, neuroendocrine and behavioural aspects of GI nociception
Fig. 2. Processing of a gastric mucosal acid challenge in various
nuclei of the rat brain. The columns depict the unilateral expression
of c-fos messenger ribonucleic acid (mRNA), a marker of neuronal activation,
in various brain nuclei of rats whose stomachs were exposed intragastrically
to 0.15 M NaCl or 0.5 M HCl. The values represent means ± SEM of 5—6 animals;*P
<0.05 versus NaCl. NTS, nucleus tractus solitarii; LPB, lateral parabrachial
nucleus; PVP, paraventricular nucleus of the thalamus, posterior part;
Pa, paraventricular nucleus of the hypothalamus; SO, supraoptic nucleus;
CeA, central amygdala; LHbm, lateral habenular nucleus, medial part; IC,
insular cortex. The data have been taken from reference 76. |
(11, 83). This view is corroborated by the central processing of a gastric mucosal
acid challenge. After the information from the acid-threatened stomach has been
communicated to the brainstem, it is passed on to subcortical brain nuclei (
Fig.
2) involved in emotional, behavioural, autonomic and neuroendocrine reactions
to a noxious stimulus (76). There is, however, no activation of the insular
cortex, the major cerebral representation area of afferent input from the stomach
(Fig. 2), which suggests that vagal afferent signalling of an acute acid insult
to the gastric mucosa does not give rise to perception of pain (76).
GI surveillance systems in concert
The conflicting needs of the digestive system in
the selective absorption of nutrients and rejection of harmful materials require
that the mucosal compartment is closely surveyed. For this purpose, EECCs and
immune cells are strategically positioned in the mucosa of the GI tract to analyze
the luminal contents (
Fig. 3). Following stimulation by food constituents
and gastric secretions, EECCs release their messengers in order to coordinate
various digestive processes according to need.
Fig. 3. GI surveillance systems in concert. Epithelial cells, enteroendocrine
cells, immune cells as well as intrinsic and extrinsic sensory neurons
interact with each other and issue outputs to the enteric and central
nervous system, respectively. |
EECCs and epithelial cells also have the capacity to react to toxins, foreign
macromolecules and infectious microorganisms. This task, though, is the particular
domain of the GI immune system which in anticipation of the continuous threats
from the lumen is the largest in the body.
Afferent neurons, of intrinsic and extrinsic origin, that send their axon terminals
into the lamina propria of the GI mucosa receive information from the adjacent
epithelial and immune surveillance systems (
Fig. 3). Their messages are
transduced by sensory neurons through receptors for enteroendocrine, immune
and mast cell mediators and signalled to the ENS and central nervous systems.
Since EECCs and immune cells are specialized to monitor cell-specific stimuli,
the input from these cells enables afferent neurons to detect luminal stimuli
that otherwise could not be encoded by their peripheral axons. These interactions
between the non-neural surveillance systems and afferent neurons have now been
recognized as important factors in the appropriate regulation of digestion,
gut defence and gut sensation.
GI hypersensitivity in functional bowel disorders
Common to FBDs such as non-ulcer dyspepsia and
irritable bowel syndrome is that the patients complain of sensory discomfort
and pain. Although multiple pathogenic mechanisms underlie the symptoms of FBDs
(15), it is ultimately extrinsic sensory neurons which signal to the brain that
something abnormal happens in the GI tract. There is now good reason to hypothesize
that, in patients with FBDs, events in the GI tract are represented in the brain
in a distorted fashion, be it because there are pathological alterations in
the environment of gut sensors, in the sensory gain of afferent neurons or in
the central processing of afferent information from the GI tract. How are these
changes brought about? It is now recognized that gastroenteritis, which may
have subsided long ago, is a major risk factor for irritable bowel syndrome
and the associated discomfort and pain (84, 85). GI hypersensitivity (
Fig.
4) involves peripheral and central mechanisms of sensitization (86), which
underlie the phenomena of GI allodynia (sensation of pain in response to stimulus
strengths that normally are innocuous) and hyperalgesia (exaggerated sensation
of pain in response to noxious stimulus strengths).
In analogy with somatic states of hyperalgesia it is hypothesized that immunological
and inflammatory processes initiate long-lasting changes in the function and
phenotype of the GI afferent nervous system (11, 14, 84). A recent study has
convincingly shown that mechanical or chemical irritation of the colon in newborn
rats leads to chronic visceral hypersensitivity in the adult animals, although
no pathology in the colon is discernible (87). Such a permanent modification
of the sensory gain is most probably related to changes in the expression of
receptors, ion channels and transmitters, changes in the biophysical properties
of receptors and ion channels (
Fig. 4), and changes in the structure,
connectivity and survival of afferent neurons (11, 88). The peripheral messengers
for these persistent adaptations include nerve growth factor acting on spinal
afferents, brain-derived neurotrophic factor acting on vagal afferents and,
conceivably, cytokines such as leukaemia inhibitory factor (11, 18, 37, 88).
Produced in inflamed tissue, these mediators induce afferent neurons to increase
the expression of neurotransmitters, receptors and ion channels such as the
sensory neuron-specific voltage-gated Na
+ channel
Na
v1.8 (88) which when transported into the
periphery may contribute to persistent hypersensitivity of the nerve terminals.
Sensory neurons as targets for the therapy of functional bowel disorders
The concept that hypersensitivity of primary afferent
neurons contributes to the pain and discomfort associated with FBDs (15) implies
that extrinsic afferents of the gut are prime targets at which novel therapies
may be aimed (24, 99). Drugs that act on nociceptive neurons have some advantages
Fig. 4. Hypersensitivity of extrinsic afferent pathways in functional
bowel disorders. |
over other analgesics, not the least because they hit the first element in the pain
pathways. In addition, these drugs may be manufactured such that they cannot
enter the brain and hence are free of unwanted adverse effects on brain functions.
Ideally, sensory neuron-targeting drugs should block the exaggerated signalling
of hypersensitive afferents, which implies that they aim at molecular targets
that are upregulated (
Fig. 4) in FBDs (89). The complex innervation of
the GI tract, though, complicates the search for specific traits on extrinsic
sensory neurons, and the development of efficacious and safe GI analgesics needs
to address several key questions:
- Which noxious/innocuous stimuli in the gut are relevant to the pain in
FBDs?
- Which receptors/ion channels on extrinsic afferents do these FBD-relevant
stimuli act on?
- Do the FBD-relevant extrinsic afferents express receptors, ion channels
or other molecular targets in a cell-specific manner?
- Is the expression of pain-relevant targets on extrinsic afferents changed
in FBDs?
Excitatory ion channels such as vanilloid receptors of type 1, acid-sensing
ion channels, P2X
3 purinoceptors and tetrodotoxin-resistant
Na
+ channels are of particular relevance because
they are selectively expressed by extrinsic.afferents (89) and, when activated,
increase the intracellular Ca2
+ concentration
and thereby stimulate Ca2
+ -dependent kinases,
regulate gene expression and alter the cellular phenotype. For assessing the
significance of these targets in GI hyperalgesia it is important to know whether
number, subunit composition and biophysical properties of sensory neuron-specific
ion channels and receptors are persistently altered after a visceral insult
(11). In addition, targets such as 5-HT receptors, CCK receptors, glutamate
receptors, tachykinin receptors, calcitonin gene-related peptide receptors,
-aminobutyric acid receptors, opioid receptors, cannabinoid receptors, PG receptors
and PARs are also worth exploring. In developing drugs along these lines it
will be important to assess which quantitative contribution sensory neuron-specific
targets make to the induction of hyperalgesia and whether modulation of a single
target is therapeutically efficacious and safe (89).