• Molecules and Cells
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• v.42 no.3
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• pp.245-251
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• 2019

Ependymal cells constitute the multi-ciliated epithelium, which lines the brain ventricular lumen. Although ependymal cells originate from radial glial cells in the perinatal rodent brain, the exact mechanisms underlying the full differentiation of ependymal cells are poorly understood. In this report, we present evidence that the Anks1a phosphotyrosine binding domain (PTB) adaptor is required for the proper development of ependymal cells in the rodent postnatal brain. Anks1a gene trap targeted LacZ reporter analysis revealed that Anks1a is expressed prominently in the ventricular region of the early postnatal brain and that its expression is restricted to mature ependymal cells during postnatal brain development. In addition, Anks1a-deficient ependymal cells were shown to possess type B cell characteristics, suggesting that ependymal cells require Anks1a in order to be fully differentiated. Finally, Anks1a overexpression in the lateral wall of the neonatal brain resulted in an increase in the number of ependymal cells during postnatal brain development. Altogether, our results suggest that ependymal cells require Anks1a PTB adaptor for their proper development.

• Molecules and Cells
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• v.46 no.12
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• pp.757-763
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• 2023

In this study, we examine whether a change in the protein levels for FOP in Ankyrin repeat and SAM domain-containing protein 1A (ANKS1A)-deficient ependymal cells affects the intraflagellar transport (IFT) protein transport system in the multicilia. Three distinct abnormalities are observed in the multicilia of ANKS1A-deficient ependymal cells. First, there were a greater number of IFT88-positive trains along the cilia from ANKS1A deficiency. The results are similar to each isolated cilium as well. Second, each isolated cilium contains a significant increase in the number of extracellular vesicles (ECVs) due to the lack of ANKS1A. Third, Van Gogh-like 2 (Vangl2), a ciliary membrane protein, is abundantly detected along the cilia and in the ECVs attached to them for ANKS1A-deficient cells. We also use primary ependymal culture systems to obtain the ECVs released from the multicilia. Consequently, we find that ECVs from ANKS1A-deficient cells contain more IFT machinery and Vangl2. These results indicate that ANKS1A deficiency increases the entry of the protein transport machinery into the multicilia and as a result of these abnormal protein transports, excessive ECVs form along the cilia. We conclude that ependymal cells make use of the ECV-based disposal system in order to eliminate excessively transported proteins from basal bodies.

• Molecules and Cells
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• v.38 no.5
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• pp.426-431
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• 2015

Odin has been implicated in the downstream signaling pathway of receptor tyrosine kinases, such as the epidermal growth factor and Eph receptors. However, the physiologically relevant function of Odin needs to be further determined. In this study, we used Odin heterozygous mice to analyze the Odin expression pattern; the targeted allele contained a ${\beta}$-geo gene trap vector inserted into the 14t intron of the Odin gene. Interestingly, we found that Odin was exclusively expressed in ependymal cells along the brain ventricles. In particular, Odin was highly expressed in the subcommissural organ, a small ependymal glandular tissue. However, we did not observe any morphological abnormalities in the brain ventricles or ependymal cells of Odin null-mutant mice. We also generated BAC transgenic mice that expressed the PTB-deleted Odin (dPTB) after a floxed GFP-STOP cassette was excised by tissue-specific Cre expression. Strikingly, Odin-dPTB expression played a causative role in the development of the hydrocephalic phenotype, primarily in the midbrain. In addition, Odin-dPTB expression disrupted proper development of the subcommissural organ and interfered with ependymal cell maturation in the cerebral aqueduct. Taken together, our findings strongly suggest that Odin plays a role in the differentiation of ependymal cells during early postnatal brain development.

• Applied Microscopy
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• v.27 no.2
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• pp.177-187
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• 1997

Hydrocephalus is induced experimentally in prenatal and suckling animals following an injection of 6-aminonicotinamide (6-AN). The most remarkable characteristic of these animals is aqueduct stenosis caused by swellings of the ependymal cells and subependymal cells in the periaqueductal gray matter and the central canal of the spinal cord. The present study was undertaken to investigate the morphological changes of the ependymal cells in the central canal of the spinal cord of 3.5 months old hamster after treatment with 6-AN. Intraperitoneal administrations of 6-AN (10 mg/kg body weight) every two days gave rise to partial central canal stenosis of the spinal cord after 27-29 days (13-l4th injection), but cilia and microvilli were located in the strictural area of the con#rat canal. The vacuolations in the ependymal cells were not observed and degenerating changes of intracellular organelles of the ependymal cells did not occur, so that the ependymal cells lining the central canal of the hamster spinal cord were not affected by 6-AN. But the present study demonstrate that 6-AN causes to create numerous vacuoles in the subependymal area of the central canal. Although the vacuoles were well developed in the neuroglial cells and the neuropils of the subependymal area, the neurons were not affected by 6-AN. These results strongly suggests that partial central canal stenosis occurred by 6-AN was due to vacuolations and swellings of the neuroglial cells and nueropils in the subependymal area.

• Applied Microscopy
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• v.29 no.1
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• pp.11-23
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• 1999

Ultrastructure of the ependymal cells of X-irradiated rats on their head were studied. Rats weighing $200\sim250gm$ were X-irradiated on their head and neck areas. Total exposures were 3,000 rads or 6,000 rads depending on experimental groups. And irradiated rats were sacrificed on 6 hours, 2 days and 6 days following the radiation exposures. Animals were perfused through the heart with 1% glutaraldehyde-1% paraformaldehyde solution, under ether-anesthesia. The tissues from the wall of lateral ventricles were fixed in the 2% osmium tetroxide solution. The results observed with electron microscope were as follow: 1. In 6 hours group, many ependymal cells were swelled, luminal portions of cytoplasms of some cells protruded into the ventricular lumen, and many cilia were lost or irregularly altered. 2. In 2 days group, ependymal cells were swelled more severely and subependymal edema were pronounced. 3. Protruded cytoplasm contained usually basal bodies of cilia, groups of mitochondria, endoplasmic reticula , etc. 4. Following X-irradiations, some protruded masses contained neural elements including the axon terminals with dense core vesicles. Axons and axon terminals were also found in the enlarged intercellular spaces among ependymal cells. From the above results, the heavy irradiation on the head area of the rat induced alteration of the ependymal cells lining the lateral ventricle. Hence the ependymal functions of selective barrier, protective barrier, and metabolic barrier could be altered following X-irradiation on the head.

• Molecules and Cells
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• v.46 no.12
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• pp.746-756
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• 2023

A recent study revealed that the loss of Deup1 expression does not affect either centriole amplification or multicilia formation. Therefore, the deuterosome per se is not a platform for amplification of centrioles. In this study, we examine whether gain-of-function of Deup1 affects the development of multiciliated ependymal cells. Our time-lapse study reveals that deuterosomes with an average diameter of 300 nm have two different fates during ependymal differentiation. In the first instance, deuterosomes are scattered and gradually disappear as cells become multiciliated. In the second instance, deuterosomes self-organize into a larger aggregate, called a deuterosome cluster (DC). Unlike scattered deuterosomes, DCs possess centriole components primarily within their large structure. A characteristic of DC-containing cells is that they tend to become primary ciliated rather than multiciliated. Our in utero electroporation study shows that DCs in ependymal tissue are mostly observed at early postnatal stages, but are scarce at late postnatal stages, suggesting the presence of DC antagonists within the differentiating cells. Importantly, from our bead flow assay, ectopic expression of Deup1 significantly impairs cerebrospinal fluid flow. Furthermore, we show that expression of mouse Deup1 in Xenopus embryos has an inhibitory effect on differentiation of multiciliated cells in the epidermis. Taken together, we conclude that the DC formation of Deup1 in multiciliated cells inhibits production of multiple centrioles.

• Journal of Korean Neurosurgical Society
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• v.52 no.1
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• pp.67-70
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• 2012

We report a rare case of a spinal intramedullary ependymal cyst in a 46-year-old female and review the 17 pathologically proven cases in the literature. The patient presented with a two-week history of gradually increasing tingling in her left posterior thigh and calf. A preoperative magnetic resonance image revealed a well-defined intramedullary cystic lesion on the ventral side of the spinal cord at the T11 to T12 levels. The lesion was hyper intense in T2-weighted images and hypointense in T1-weighted. The patient underwent a right-side hemilaminectomy at the T11 to T12 levels and fenestration of the cyst wall. After having the cyst wall partially removed and communication established between the cyst and the subarachnoid space, the patient improved neurologically. A histological study of the surgical specimens revealed that the cyst wall consisted of glial cells lined by a simple cuboidal to columnar epithelium. An immunohistochemical examination of the cells lining the cyst wall was positive for S-100 protein, glial fibrillary acidic protein, epithelial membrane antigen, and cytokeratin. We suggest that the optimal treatment of intramedullary ependymal cysts creates adequate communication between the cyst and the subarachnoid space.

• Applied Microscopy
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• v.23 no.1
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• pp.109-124
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• 1993

The prenatal development of thoracic spinal cord was studied by electron microscope in human embryos and fetuses ranging from 9mm to 260mm crown-rump length (5-30 weeks of gestational age). Ependymal cells in all fetal ages had conspicuous junctional complexes close to the lumen of the central canal into which microvilli and cilia projected. The ependymal cells contained numerous longitudinally arranged mitochondria, flattened cisternae of endoplasmic reticulum and Golgi complex. At 20 mm embryo, the floor and roof plates were composed of ependymoglial cells and undifferentiated neuroepithelial cells. The neuroepithelia of the sacral spinal cord were delineated from central medullary cord. By 100 mm fetus few undifferentiated neuroepithelial cells remained in the floor and roof plates. At 150 mm fetus, the whole central canal was formed by ciliated columnar epithelial cells containing cilia with basal bodies. The microvilli became tangled and club-shaped and formed a matted surface. The canal was filled with areas of dark and pale amorphous materials bounded by membrane-like structure. These two types of material were found throughout the whole central canal from 100 mm fetus onwards. By 260 mm fetus, microfibrils were first observed in the ependymal cells. In conclusion, it seems that early development and differentiation of central canal ependyma are simlar to that in other part of the brain ventricular system although ependymoglial cells are more prominent.

• Applied Microscopy
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• v.30 no.4
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• pp.377-387
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• 2000

There are a few tanycytes between the general ependymal cells lining the ependymal layer of the brain ventricle. These cells are considered as modified ependymal cells which possess a long basal process. Tanycytes are known to have an ability to communicate by absorbing substances from cerebrospinal fluid and transporting them to the blood vessels and/or to the neurons in the CNS. The third and fourth ventricular tanycytes were mainly studied as subjects but it's rare to find reports about the tanycytes of the area postrema. Glial fibrillary acidic protein is an intermediate filament protein that is expressed especially in astrocytes of the CNS. But GFAP is also found in filament of the tanycytes and its process. Therefore this study was carried out for the examination of the GFAP immunoreactive tanycytes lining the area postrema of the bat, and we also examined the ultrastructure of tanycytes using electron microscope. GFAP immunoreactive tanycytes were located in the caudal portion of the fourth ventricle, and especially mainly in the transitional zone between the floor of the caudal fourth ventricle and ependymal layer lining the area postrema. A few GFAP immunoreactive tanycytes were also found in the ependymal layer lining the area postrema, and some groups of tanycytes were found in the ependymal layer of the area postrema near the floor of the caudal fourth ventricle , The processes of tanycytes were stained deeply with anti-GFAP antibody. Especially the GFAP immunoreactive tanycytes lining the area postrema had very long processes that cross the whole width of the area postrema. In the electron microscope, the cell body of ependymal tanycyte was located on the ependymal layer and had a long basal process. Intermediate filaments were observed around the nucleus and well developed in the process of tanycrte. Longitudinal oriented long mitochondria and a few lipid droplets were also found in this process. After immunocytocheical staining, the gold particles were found only in the intermediate filaments. We could not determine the function of the tanycytes in the area postrema. Thus, further investigation is required to determine the functional relationship between the tanycytes and the area postrema in hibernating animal, the bat.

• The Korean Journal of Physiology and Pharmacology
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• v.1 no.5
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• pp.495-504
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• 1997

Water transport is mediated by two distinct pathways, diffusional and channel-mediated water transport. The first molecular water channel was identified from human erythrocytes in 1992. Genetically-related proteins from other mammalian tissues have subsequently been identified to transport water, and the group is referred to as th "Aquaporins". Aquaporin-4 (AQP4) is most abundant in the brain, which may be involved in CSF reabsorption and osmoregulation. However, ontogeny and regulatory mechanisms of AQP4 channels have not been reported. Northern blot analysis showed that AQP4 mRNA began to be expressed in the brain just before birth and that its expression gradually increased by PN7 and then decreased at adult level. AQP4 was expressed predominantly in the ependymal cells of ventricles in newborn rats. And then its expression decreased in ependymal cells and increased gradually in other regions including supraoptic and paraventricular nuclei. AQP4 is also expressed in the subfornical organ, in which the expression level is not changed after birth. Cryogenic brain injury did not affect expression of AQP4 mRNA, while ischemic brain injury decreased it. Osmotic water permeability of AQP4 channel expressed in Xenopus oocytes was inhibited by the pretreatment of BAPTA/AM and calmidazolium, a $Ca^{2+}/Calmodulin$ kinase inhibitor, in a dose-dependent manner. These results indicate that the expression and the function of AQP4 channel are regulated by developmental processes and various pathophysiological conditions. These results will contribute to the understanding of fluid balance in the central nervous system and the osmoregulatory mechanisms of the body.