The endocrine cells of the pancreas are grouped in the islets of Langerhans. Discovered in 1869 by the German pathological anatomist Paul Langerhans, the islets of Langerhans constitute approximately 1 to 2% of the mass of the pancreas. There are about one million islets in a healthy adult human pancreas, which are interspersed evenly throughout the organ, and their combined weight is 1 to 1.5 grams. Each islet contains approximately one thousand cells and is 50-500 um in diameter.
Hormones produced in the Islets of Langerhans are secreted directly into the blood flow by (at least) four different types of cells:
Beta cells producing Insulin and Amylin (65-80% of the islet cells)
Alpha cells releasing Glucagon (15-20%)
Delta cells producing Somatostatin (3-10%)
PP cells containing polypeptide (1%)
Islets can influence each other through paracrine and autocrine communication, and beta-cells are coupled electrically to beta-cells (but not to other cell-types).
7B2
a
possible marker for nonfunctioning pancreatic islet cell tumor.
PMID: 1662184,
PMID: 1466150
secreted by both A- and B-cells of the
hamster pancreatic islets and its secretion is regulated under
certain conditions.
PMID: 1811274
originally isolated from pituitary, has been
shown to be present in endocrine tumors of high concentrations
in pancreatic islet tumors.
PMID: 2512184,
PMID: 3342519
The high incidence of elevated IR-7B2
concentrations in pancreatic islet tumors and the increase in
IR-7B2 concentrations in the term pancreas and particularly in
nesidioblastosis suggest that the novel protein 7B2 may serve as
an islet marker.
PMID: 2822517
7B2 immunoreactivity is produced by a variety
of different tumors and may serve as a tumor marker, especially
in patients with certain pancreatic islet tumors.
PMID: 3525602
Adenylylcyclase
can be used as a marker to assess islet cell
viability as well as differences in preservation media and may
predict islet cell transplant success.
PMID: 10478718,
PMID: 10218770
Amylase
measurement of amylase levels after pancreas
preservation may have potential clinical application as a marker
to evaluate pancreatic tissue injury.
PMID: 14529937
Beta-2/NeuroD
a key regulator of pancreatic islet
morphogenesis and insulin gene transcription.
PMID: 10633878
expressed in both alpha- and beta-cells.
PMID: 8799146
expressed in endocrine cells during pancreas
development and is essential for proper islet morphogenesis, and
is pancreatic
islet-specific marker.
PMID: 10757813
a basic helix-loop-helix transcription
factor, is a key regulator of pancreatic islet morphogenesis and
insulin gene transcription.
PMID: 16186386
a member of a helix-loop-helix (HLH) family
that is specifically expressed in the endocrine pancreas.
PMID: 12861411
CGRP (Calcitonin gene-related peptide)
CGRP-containing cells were found primarily in
the peripheral portion of the pancreatic islets. In addition,
CGRP-containing cells also contained somatostatin, which
identifies the islet CGRP-containing cells as D cells.
PMID: 2896352
Chromogranin A/B
In porcine pancreas at early fetal stage, CgA
was visualized throughout the immature granules in all
neuroendocrine cells. Later, CgB also was expressed with the
same pattern in most granules in all types of cells. In neonatal
islets, CgA was localized in the periphery of immature alpha-
and beta-cell granules and throughout the matrix of delta-cell
granules; CgB was distributed throughout the matrix of these
granules. In adult islets, alpha-cell granules stored CgA in the
halo and CgB in both the core and the halo, beta-cell granules
stored both CgA and CgB in their cores, and in delta-cell
granules, both substances were mixed throughout the matrix.
PMID: 12826904
CgA molecule is cleaved in different ways in
the various pancreatic endocrine cell types, giving rise to a
variety of biologically functional fragments.
PMID: 11259451
Chromogranin A is a useful
immunohistochemical marker for pancreatic tumors of
neuroendocrine origin and their metastases.
PMID: 9185968
At the light microscopic level, CGA-like
immunoreactivity was found in the same cells that react with
antibodies against insulin, glucagon, and somatostatin.
PMID: 2878021
C-reactive protein (CRP)
a
very early marker for islet stress in the rat pancreas.
PMID: 9704587
FoxA1
a novel function for Foxa1 in the regulation
of oxidative phosphorylation in pancreatic beta-cells.
PMID: 17003337
FoxA3
a member of the proglucagon gene G2 element
binding-protein family that, unlike Foxa1, is not essential for
control of islet or intestinal proglucagon gene expression in
vivo.
PMID: 12000309
GAD (glutamic
acid decarboxylase)
has been identified as one of the major
auto-antigens expressed in islet cells, and used as a targeting
moiety for islet cell targeting.
PMID: 16139384
GAD65/GAD67
GAD65 was primarily detected in rat
pancreatic islet beta-cells, whereas alpha-cells had weak GAD65
staining. In contrast, GAD67 was detected primarily in
alpha-cells.
PMID: 7822765
Gdf11
expressed in embryonic pancreatic epithelium
during formation of islet progenitor cells that express
neurogenin 3, and regulates the production and maturation of
islet progenitor cells in pancreas development.
PMID: 15548585
GLP-1 (Glucagon-like
peptide)
a gut hormone that
stimulates insulin secretion, gene expression, and beta-cell
growth.
PMID: 15561911
a growth factor for pancreatic cells and it
is a regulator of islet cell mass.
PMID: 15289644
a growth factor for pancreatic endocrine
cells and can increase islet cell mass.
PMID: 12673779
a differentiation factor for pancreatic
ductal cells and that its effect requires the expression of
IDX-1.
PMID: 11289043
enhances beta-cell neogenesis and islet size.
Thus, in addition to stimulating insulin secretion, GLP-1
stimulates the expression of the transcription factor IDX-1
while stimulating beta-cell neogenesis and may thereby be an
effective treatment for diabetes.
PMID: 10905482
Glucagon
In the native rat pancreas glucagon is
expressed by alpha cells in the outer layer of the spheroid
islet, producing a circular immunohistochemical staining
pattern.
PMID: 16298637
expressed in A cells of the pancreatic islets
due to an interaction between multiple regulatory elements
within the 5'-flanking region of its gene directing glucagon
gene transcription.
PMID: 9088537
chicken pancreatic islet A cells contain
glucagon, serotonin, and aromatic L-amino acid decarboxylase, an
enzyme involved in the synthesis of serotonin.
PMID: 2404608
a peptide hormone which
regulates hepatic carbohydrate metabolism, is processed from a
larger precursor, proglucagon. The gene encoding proglucagon is
expressed at high levels in the A cells of the pancreatic islets
and the L cells of the intestine, indicating that specific
factors present in these two phenotypically distinct cells
direct cell-specific expression.
PMID: 3316202
glucagon gene is expressed in both pancreatic
islets and neuroendocrine L-cells of the gastrointestinal tract.
PMID: 2879843
GLUT1/GLUT3 mRNA (Glucose Transporter 1, 3)
expressed in all human islet cell tumors and
normal islets examined. In contrast, GLUT2 mRNA, which is
present at high levels in normal islets, was not detected in
insulinomas or other types of islet cell tumors. These results
imply that GLUT1 and GLUT3 are primarily responsible for glucose
uptake by these tumors.
PMID: 8421107
GLUT-2 (Glucose
Transporter 2)
specifically localized immunocytochemV cally
in beta-islet cells.
PMID: 12114701
an essential component of the glucose-sensing
mechanism of the pancreatic beta-cell.
PMID: 9451476
the only detectable transporter isoform in
normal islets.
PMID: 1489658
mainly expressed in the hepatocytes of the
liver and the beta-cells of the pancreatic islets.
PMID: 1683793
GLUT-4 (Glucose transporter 4)
is present in human and rat pancreatic islets
and may suggest its specific role in glucose homeostasis in
conjunction with IRAP/P-LAP.
PMID: 14751249
hCG (human chorionic gonadotropin)
hCG and its subunits are prevalent and
specific markers for islet-cell carcinoma, and suggest that
ectopic secretion results from malignant derepression of the
genome rather than overproduction by an aberrant "cell rest."
PMID: 196194
IA2/ICA512
the precursor of the islet 40- and 37-kDa
polypeptide autoantigens and as one of the ICA specificities.
PMID: 7594559
a novel autoantigen of type 1 diabetes.
PMID: 9024221
IAPP/Amylin
(Islet amyloid polypeptide)
a normal secretory
product of the beta cells,
is
synthesized in the islets of Langerhans.
PMID: 7771063,
PMID: 1618637
a novel peptide recently isolated from the
amyloid substance of pancreatic islets, has been localized in
normal islet B cell granules, suggesting its physiological
action in exocrine pancreatic function.
PMID: 1724710
co-stored with insulin in secretory granules
of pancreatic islet beta-cells.
PMID: 14565847
an increased beta cell storage of IAPP
independent of insulin may be an important factor in the early
phase of the development of islet amyloid in this form of
diabetes.
PMID: 10036583
the protein component of amyloid deposits
commonly seen in pancreatic islets of patients with type 2
diabetes mellitus.
PMID: 9281227
may be useful as an additional marker for
neuroendocrine tumour growth in selected cases.
PMID: 7754100
a novel peptide which was extracted from
islet amyloid deposits in patients with non-insulin-dependent
diabetes mellitus (NIDDM).
PMID: 1541240,
PMID: 1541234
originally identified by chemical analysis of
the amyloid component in a human pancreatic islet cell tumor,
can be used as a tumor marker in pancreatic islet cell tumors
and that high plasma levels of IAPP can inhibit glucose
stimulated insulin secretion.
PMID: 1854502
a pancreatic islet hormone which was first
found in amyloid in insulinomas and in pancreases of patients
with type 2 diabetes.
PMID: 2223885
a 37-amino-acid putative hormone which is
expressed by islet B-cells and most probably is co-released with
insulin.
PMID: 2691219
more widely distributed within the pancreatic
islet cell types than are any of the other islet hormones.
PMID: 8940352
a secretory product of the pancreatic
beta-cell, which is the primary constituent of the islet amyloid
that develops in type II diabetes.
PMID: 7958499
ICA (Islet cell antibodies)
the classical autoimmunity marker for
insulin-dependent diabetes mellitus (IDDM).
PMID: 9439455
present in all Japanese Type 1 diabetic
patients whose diabetic manifestations are less than 0.5 years
duration from onset.
PMID: 3527837
important markers for two subtypes of
insulin-dependent diabetes mellitus and stain the entire islet
in the standard immunofluorescence test.
PMID: 81509
IGRP
selectively expressed in islet beta cells and
is a major autoantigen in a mouse model of type I diabetes.
PMID: 15180990
INGAP (islet neogenesis-associated protein)
participates in the
regulation of islet neogenesis.
PMID: 12740013
INGAP-PP enhances specifically the secretion
of insulin and the transcription of several islet genes, many of
them directly or indirectly involved in the control of islet
metabolism, beta-cell mass and islet neogenesis. These results,
together with other previously reported, strongly indicate an
important role of INGAP-PP, and possibly of INGAP, in the
regulation of islet function and development.
PMID: 16764950
INGAP gene is a novel pancreatic gene
expressed during islet neogenesis whose protein product is a
constituent of Ilotropin and is capable of initiating duct cell
proliferation, a prerequisite for islet neogenesis.
PMID: 9151782
Insulin
is both a hormone regulating energy
metabolism and a growth factor, and can act as a master
regulator of islet survival by regulating Pdx1.
PMID: 17158802
can stimulate islet beta-cell replication
directly, possibly through a receptor for MSA or another
insulin-like growth factor.
PMID: 6759233
IPF1 (Insulin promoter factor 1)
a member of the homeodomain protein family,
serves an early role in pancreas formation. In adults, IPF1
expression is restricted to the beta-cells in the islets of
Langerhans.
PMID: 8799146
Islet-1
one of the earliest markers for motor neuron
differentiation, is also expressed in all classes of islet cells
in the pancreas.
PMID: 14664703
MafB
plays a previously uncharacterized role by
regulating transcription of key factors during development that
are required for the production of mature alpha and beta cells.
PMID: 17360442
the adult pancreas, MafB is only expressed in
islet alpha-cells and contributes to cell type-specific
expression of the glucagon gene through activation of a
conserved control element found between nucleotides -77 to -51.
MafB was also shown to be expressed in developing alpha- and
beta-cells as well as in proliferating hormone-negative cells
during pancreatogenesis. These results indicate that MafB is not
only important to islet alpha-cell function but may also be
involved in regulating genes required in both endocrine alpha-
and beta-cell differentiation.
PMID: 16443760
Nestin
may not be a suitable marker solely for the
identification of endocrine precursor cells in the pancreas.
PMID: 14765974
expanded nestin-expressing cells in vitro
from islet-derived epithelial monolayers are heterogeneous;
clonal analysis of nestin-positive cells reveals that a distinct
subpopulation of nestin/PDX-1-expressing cells is capable of
forming insulin-producing cells.
PMID: 15684341
at least in vitro, nestin-positive cells may
undergo the early stages of differentiation to an islet cell
phenotype and that long-term cultures of nestin-positive human
islet cells may be considered as a potential source of precursor
cells to generate fully differentiated/ functional beta cells.
PMID: 15590972
c-Kit- and nestin-expressing cells represent
endocrine precursor cells that undergo marked changes in
population dynamics during the transition from prenatal to
postnatal pancreatic development in the rat.
PMID: 15042705
nestin-positive cells play an important role
in the growth and maintenance of the islet.
PMID: 14514633
Neurogenin 3 (Ngn3)
controls endocrine cell fate specification in
uncommitted pancreatic progenitor cells, is a marker of islet
precursor cells.
PMID: 15297605,
PMID: 11973276
Neuropeptide Y (NPY)
known to occur in the autonomic nervous
system, including the pancreatic islet innervation, is also
expressed in endocrine islet cells in hamster pancreas.
PMID: 7644704
Neuropilin-2
a novel
marker expressed in pancreatic islet cells and endocrine
pancreatic tumours.
PMID: 12210066
NKX6.1
play roles in suppression of glucagon
expression and control of GSIS in islet beta cells.
PMID: 15883383
NSE (neuron-specific enolase)
a good marker for islet cell tumor, and is
valuable for examining islets in pancreas with various disorders
both alone and in combination with other tumor markers.
PMID: 3015709
serum NSE levels can aid in the diagnosis and
monitoring of the course of nonfunctining islet cell carcinoma.
PMID: 6295198
Pancreatic Polypeptide
a
useful plasma marker for radiographically evident pancreatic
islet cell tumors in patients with multiple endocrine neoplasia
type 1.
PMID: 9426414
presence of a markedly elevated fasting
plasma human pancreatic polypeptide (hPP) level in patients with
MEN 1 is 95% sensitive and 88% specific for the presence of
radiographically detectable pancreatic islet cell tumors.
PMID: 9426414
A small population of pancreatic islet cells
showed strong immunofluorescence after staining with anti-HPP
serum.
PMID: 1091353
Pax4
the mouse Pax-4 gene is expressed in
pancreatic islets, cultured islet beta cell lines of MIN6, beta
TC, and NIT-1 cells, but not detectable in any of 13 adult mouse
organs examined.
PMID: 9439631
Pax6
Pax-6 protein(s) expressed in adult
pancreatic islets function differently from the ones in the eye
and cerebellum.
PMID: 10319322
a key regulator of pancreatic islet hormone
gene transcription and is required for normal islet development.
PMID: 9224716
PDX-1 (Pancreatic
duodenal homeobox factor-1)
normally mediate pancreatic development are
capable of inducing hepatic progenitor cells to differentiate
into cells with pancreatic islet characteristics.
PMID: 17239820
highly enriched in beta and delta cells.
PMID: 9115263
exogenous expression of Pdx-1 should provide
a promising approach for efficiently producing islet-like cells
from BMSCs for the future therapeutic use in diabetic patients.
PMID: 17172056
regulates transcription of genes involved in
islet beta cell function and pancreas development.
PMID: 16147997
a transcription factor required for normal
pancreatic development, also plays a key role in normal insulin
secretion by islets.
PMID: 15562255
a critical regulator of beta cell replication
and is required for the compensatory response to insulin
resistance.
PMID: 15372107
also known as IDX-1/STF-1/IPF1, a homeodomain-containing
transcription factor, plays a central role in regulating
pancreatic development and insulin gene transcription.
PMID: 12829640
required for pancreas development, islet cell
differentiation, and the maintenance of beta cell function.
PMID: 12551916
PEK
only detected in human pancreatic islets, and
co-localized with somatostatin, a pancreatic delta cell-specific
hormone. Thus PEK is believed to play an important role in
regulating protein synthesis in the pancreatic islet, especially
in islet delta cells.
PMID: 10026192
Rel B
an
early marker of autoimmune islet inflammation in the biobreeding
(BB) rat.
PMID: 10630383
Somatostatin/Receptors
a pancreatic delta cell-specific hormone.
PMID: 10026192
a peptide synthesized in the pancreatic
islets, nervous system, gastrointestinal tract, and thyroid
gland.
PMID: 2566613
a novel ionic mechanism involved in
somatostatin-inhibition of glucagon-secretion from pancreatic
alpha cells.
PMID: 10050772
Although there is no absolute specificity of
any SSTR for an islet cell type, SSTR1 is beta-cell selective,
and SSTR2 is alpha-cell selective. SSTR5 is well expressed in
beta-cells and delta-cells and moderately well expressed in
alpha-cells, and thereby it lacks the islet cell selectivity
displayed by SSTR1 and SSTR2.
PMID: 9892225
chicken islet D-cells contain only
SST-14-like peptide, but not SST-28-like peptide, and that this
phenomenon may be common to the avian species.
PMID: 8760862
STF-1 (somatostatin
transactivating factor-1)
a novel
homeobox factor that stimulates somatostatin expression in
pancreatic islet cells.
PMID: 7505393
Synaptophysin
In all ontogenetic
stages, synaptophysin was demonstrated in all
pancreatic islet
cell types and granules.
PMID: 12826904
Tyrosine Hydroxylase
co-expressed with islet hormones in the fetal
mouse pancreas. In the adult animal, the enzyme has been
considered as a marker of ageing beta-cells.
PMID: 11813861
Other Islet Cell
Markers
Trypan
Blue as a marker of plasma membrane permeability in alloxan-treated
mouse islet cells.
PMID: 226577