Li Liu1, Jung-Hwa Tao-Cheng2, Stephen Dodd1, Nadia Bouraoud1, Hari Rallapalli1, and Alan P Koretsky1
1Laboratory of Functional and Molecular Imaging, National Institute of Neurological Disorders and Stroke, Bethesda, MD, United States, 2Electron Microscopy Facility, National Institute of Neurological Disorders and Stroke, Bethesda, MD, United States
Synopsis
Keywords: Neuroinflammation, Neuroinflammation, Brain iron, olfactory ensheathing cells, glia limitans, olfactory sensory neuron, lipofuscin
Motivation: Brain iron overload is a hallmark of neuroinflammatory diseases. Here, T2*-weighted MRI detected an increase of hypointensity at the olfactory nerve layer (ONL) in young mice from 3 to 12 weeks.
Goal(s): Find out the cellular origins of T2*-hypointensity.
Approach: MRI guided immunohistochemistry and electron-microscopy study.
Results: We found that the T2*-hypointensity is due to high iron in olfactory ensheathing cells (OECs). OECs form glia limitans, ensheath bundles of olfactory axons, and are the main phagocytic cells in olfactory system to aid regeneration of olfactory neurons. Near high-iron region, lipofuscin, a neuroinflammation marker was found in activated microglia as early as 6 weeks.
Impact: Guided by MRI, we found
that there is an extraordinarily high level of intrinsic iron in the olfactory
ensheathing cells in olfactory nerve layer in young mice, which indicates
their phagocytic and neuroprotection functions during rapid olfactory sensory
neuron turnover.
INTRODUCTION
The olfactory nervous system glia limitans is unique as it consists of
olfactory ensheathing cells (OECs), olfactory sensory neuron
(OSN) axon bundles, and astrocytes1,2. In
contrast, the glia limitans in the rest of nervous system consists only of
astrocytes and oligodendrocytes or Schwann cells. OSNs
at the nasal epithelium have a unique ability to continuously regenerate from
progenitor cells throughout life3,4. OECs
at the olfactory nerve layer (ONL) wrap bundles of OSN axons, support axonal
regeneration of OSNs, and show inflammatory responses making them a unique cell
type5. Here,
T2*-weighted MRI detected a rapid increase of hypointensity at ONL in normal
mouse from 3 to 12 weeks. We aim to find out the origins of MRI hypointensity.METHODS
MRI and quantification.
MRI experiments were
carried out on an 11.7-T animal MRI with a Bruker CryoProbe. T2*-weighted 3D-gradient-recalled-echo
(GRE) sequences were used for acquisitions. For in-vivo imaging, parameters were: isotropic-resolution=75 µm, TE/TR=10/30 ms, FA=10°, NA=3, scan-time=36m. For
multi-gradient-echo (MGE) imaging: TE=5 ms, TR=60 ms, Echo Spacing=5 ms, Echo
Images=4, FA=40°, NA=10, scan time=17h4m. For ex-vivo MRI: isotropic-resolution=50 µm,
TE/TR=20/40 ms, FA=15°, NA=12, scan-time=15h8m. A line voxel-of-interest (MIPAV)
was drawn from external
plexiform layer (EPL) through glomerular layer (GL) and ONL. The % decreased intensity was calculated by: % (IntensityONL
– IntensityEPL)/ IntensityEPL.
Immunohistochemistry
(IHC). Brain sections were stained using standard procedures for free-floating
slice immunohistochemistry.
Preembedding immunogold
labeling and electron microscopy (EM). Tissue slides were incubated with goat-anti-mouse-Sox10
to identify OECs for 1 hr, followed by incubation with donkey-anti-goat-Nanogold.
The size and appearance of iron-containing ferritin molecules were determined
as previous report6.RESULTS
In normal 12-week-old
mice, T2*-weighted MRI detected a significant hypointensity region from the
edge of the GL into the ONL (Fig 1A-B). This hypointensity was
identified at the ONL as shown by MGE MRI, in which GL can be identified as
hyperintense due to shorter T1 of this layer at a short TE (TE=5 ms, Fig 1C).
The change in the hypointense region was
studied as a function of age in mice and rats (Fig 2A,C). The decrease
of intensity from EPL to ONL was quantified (Fig 2B,D). In mice, the
decrease of intensity at 3 weeks, 6 weeks, 12 weeks, and 2 years were
16.5±4.9%, 30±5.5%, 48.2±11.5% and 73.1±8.4%, respectively. This rapid increase
of hypointensity in the ONL was not observed in young rats. 17.3±4.6% decrease
of intensity was detected in 12-week rats, comparing with 48.2±11.5% in mice. Only at 11 months was there a large decrease
of intensity (Fig 2D).
This hypointensity at the ONL was due to
iron accumulation, as shown by Prussian Blue iron staining and IHC staining of
ferritin in 12-week mice (Fig 3).
To determine the cellular origin of the
high iron, olfactory bulb tissues of 12-week-old mice were stained for OECs
(Sox10), astrocytes (GFAP), microglia (IBA1) and oligodendrocytes (Oligo2). We
found that OECs were the major cell type (84.2±6.2% of DAPI+ cells) at ONL (Fig
4A). Co-staining of OECs and ferritin showed that there was a high level of
ferritin in OECs (Fig 4C). EM images showed OECs wrapped blood vessel
and axon bundles (Fig 4D,E). High concentration of EM dark contrast
consistent with the size of ferritin were interpreted to be ferritin containing
iron particles (yellow circles) in the cytoplasm of OECs.
Lipofuscin,
a neuroinflammation marker, was observed in high iron areas in ONL and GL in
mice as early as 6 weeks (Fig 5A). Lipofuscin was not detected in 3-week
mice and 6-month rats (Fig 5B), in which hypointensity was not detected
by MRI. Lipofuscin was found to located in the CD68+ activated microglia (Fig
5C-D). Lipofuscin was found primarily in the GL, and not ONL, indicating
that OECs can safely store high iron. DISCUSSION
Brain iron overload leads to neuroinflammation
and neurodegeneration, which is evident in many neurological disorders. Oligodendrocytes
are a major source of iron in brain as are iron-laden activated macrophages and
microglia7. It is
not clear why the rat and mouse have such different age dependencies for gaining
iron in ONL and may have to do with a higher rate of OSNs regeneration or a
higher metabolic rate in the mouse.
Although the
mechanistic connection between iron accumulation and markers of inflammation
such as lipofuscin and activated glia is unclear, they are likely closely
associated. This model may help to understand the well-known links between high
iron and inflammation. CONCLUSION
We found that there is a
high level of intrinsic iron in the OECs at the ONL of young mice and,
interestingly, lipofuscin accumulate nearby at this olfactory
barrier.Acknowledgements
This research was supported by the intramural program at the National Institute of Neurological Disorders and Stroke (NINDS), National Institutes of Health (NIH).References
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