• Molecules and Cells
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• v.19 no.3
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• pp.310-317
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• 2005

The Xenopus FGF-8a and FGF-8b isoforms have been reported to be neural crest and neuronal inducers, respectively. However, cloning of Xenopus FGF-8b (XFGF-8b) has not been reported previously and the two isoforms do not seem to have been clearly distinguished in Xenopus experiments. Here, we describe the cloning and expression of XFGF-8b and compare the effects of the two isoforms. XFGF-8b has an 11 amino acid insert in its N-terminal region compared with XFGF-8a. Both isoforms are expressed in the anterior neural regions of the early embryo, and in the apical ectodermal ridge of limb buds and tips of growing digits in the larval stages. However, XFGF-8b is more abundant than XFGF-8a throughout early development. The two isoforms are also regulated in similar fashion by retinoic acid in early development. However, although both XFGF-8a and XFGF-8b induce ectopic neurogenesis, only XFGF-8a appears to be involved in neural crest induction.

• Tuberculosis and Respiratory Diseases
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• v.40 no.6
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• pp.747-750
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• 1993

A 51-year-old male was admitted due to mediastinal mass. Chest PA showed a $4{\times}5$ cm sized round mass on right lower paratracheal area. The chest computerized tomogram showed cystic mass located between superior vena cava and trachea. This cystic mass was composed of variable internal architecture with Hounsfield unit(HU) from -44.4 to +25.5. The resected cystic mass revealed ectodermal, mesodermal, and endodermal structures. We report a case of anterior mediastinal cystic teratoma confirmed by open thoracotomy.

• Archives of Craniofacial Surgery
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• v.20 no.5
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• pp.336-340
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• 2019

Myoepithelioma was recognized as a histological distinct entity by the World Health Organization (WHO) in 1991. Myoepithelial cells are believed to be of ectodermal origin. In salivary glands, the myoepithelial cells that surround the intercalated ducts are spindled, which is in contrast to the large stellate ones that envelop the acini. Myoepithelioma is a benign salivary gland tumor that consists entirely of myoepithelial cells. A 53-year-old man presented with a 1-year history of a painless mass originating from the right parotid gland. The mass grew rapidly reaching a size of approximately 6 cm. The patient had no facial paralysis. The authors performed right parotidectomy. Immunohistochemistry study of this tumor showed that it was positive for vimentin, positive for S-100, focally positive for pancytokeratin, and focally positive for p63 and that it had a Ki-67 labeling index (below 10%). Additionally, the tumor was negative for epithelial membrane antigen, negative for actin, negative for desmin, negative for CD34 and negative for anaplastic lymphoma kinase. The authors present a case of benign spindle cell myoepithelioma of the parotid gland in a 53-year-old man diagnosed after immunohistochemistry study, describing its importance, along with a brief review of the literature.

• Journal of Genetic Medicine
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• v.16 no.1
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• pp.1-9
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• 2019

Noonan syndrome (NS) and NS-related disorders (cardio-facio-cutaneous syndrome, Costello syndrome, NS with multiple lentigines, or LEOPARD [lentigines, ECG conduction abnormalities, ocular hypertelorism, pulmonic stenosis, abnormal genitalia, retardation of growth and sensory neural deafness] syndrome) are collectively named as RASopathies. Clinical presentations are similar, featured with typical facial features, short stature, intellectual disability, ectodermal abnormalities, congenital heart diseases, chest & skeletal deformity and delayed puberty. During past decades, molecular etiologies of RASopathies have been growingly discovered. The functional perturbations of the RAS-mitogen-activated protein kinase pathway are resulted from the mutation of more than 20 genes (PTPN11, SOS1, RAF1, SHOC2, BRAF, KRAS, NRAS, HRAS, MEK1, MEK2, CBL, SOS2, RIT, RRAS, RASA2, SPRY1, LZTR1, MAP3K8, MYST4, A2ML1, RRAS2). The PTPN11 (40-50%), SOS1 (10-20%), RAF1 (3-17%), and RIT1 (5-9%) mutations are common in NS patients. In this review, the constellation of overlapping clinical features of RASopathies will be described based on genotype as well as their differential diagnostic points and management.

• Development and Reproduction
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• v.12 no.2
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• pp.159-168
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• 2008

Nanog is a newly identified member of the homeobox family of DNA binding transcription factors that functions to maintain the undifferentiated state of stem cells. However, molecular mechanisms underlying the function of Nanog remain largely unknown. To elucidate the regulatory roles of Nanog involved in maintenance of P19 embryonal carcinoma (EC) stem cells, we transfected three small interfering RNA (siRNA) duplexes targeted against different regions of the Nanog gene into P19 cells. The Nanog siRNA-100 duplexes effectively decreased the expression of Nanog up to 30.7% compared to other two Nanog siRNAs, the Nanog siRNA-400 (67.9 %) and -793 (53.0%). When examined by RT-PCR and real-time PCR, the expression of markers for pluripotency such as Fgf4, Oct3/4, Rex1, Sox1 and Yes was downregulated at 48 h after transfection with Nanog siRNA-100. Furthermore, expression of the ectodermal markers, Fgf5 and Isl1 was reduced by Nanog knockdown. By contrast, the expression of other markers for pluripotency such as Cripto, Sox2 and Zfp57 was not affected by Nanog knockdown at this time. On the other hand, the expression of Lif/Stat3 pathway molecules and of the endoderm markers including Dab2, Gata4, Gata6 and the germ cell nuclear factor was not changed by Nanog knockdown. The results of this study demonstrated that the knockdown of Nanog expression by RNA interference in P19 cells was sufficient to modulate the expression of pluripotent markers involved in the self-renewal of EC stem cells. These results provide the valuable information on potential downstream targets of Nanog and add to our understanding of the function of Nanog in P19 EC stem cells.

• International Journal of Oral Biology
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• v.36 no.1
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• pp.31-35
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• 2011

Teeth develop via a reciprocal induction between the ectomesenchyme originating from the neural crest and the ectodermal epithelium. During complete formation of the tooth morphology and structure, many cells proliferate, differentiate, and can be replaced with other structures. Apoptosis is a type of genetically-controlled cell death and a biological process arising at the cellular level during development. To determine if apoptosis is an effective mechanism for eliminating cells during tooth development, this process was examined in the rat mandible including the developing molar teeth using the transferase-mediated dUTP-biotin nick labeling (TUNEL) method. The tooth germ of the mandibular first molar in the postnatal rat showed a variety of morphological appearances from the bell stage to the crown stage. Strong TUNEL-positive reactivity was observed in the ameloblasts and cells of the stellate reticulum. Odontoblasts near the prospective cusp area also showed a TUNEL positive reaction and several cells in the dental papilla, which are the forming pulp, were also stained intensively in this assay. Our results thus show that apoptosis may take place not only in epithelial-derived dental organs but also in the mesenchyme-derived dental papilla. Hence, apoptosis may be an essential biological process in tooth development.

• Clinical and Experimental Pediatrics
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• v.46 no.6
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• pp.606-609
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• 2003

Goltz syndrome(focal dermal hypoplasia) is a rare disorder characterized by ectodermal and mesodermal dysplasia described in 1962 by Goltz. In Korea, one case of Goltz syndrome was reported in 1994. The inheritance mode is mostly X-linked dominant. Skin abnormality is the most common manifestation including hypoplasia of the dermis. Skeletal involvement such as syndactyly, polydactyly, scoliosis, kyphosis and spina bifida occulta may be present, also ocular and dental abnormalities are reported. Radiologic findings are the osteopathy and striation of the long bone. We experienced a case of Goltz syndrome in a 9-year old female who was presented with right side hypotrophy, focal dermal hypoplasia, ocular(anidria, microcornea), dental(oligodontia, amelogenesis) and skeletal(syndactyly) abnormalities. Skin biopsy was performed and showed decreased expression of type I collagen gene with Northern blotting.

• Journal of the korean academy of Pediatric Dentistry
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• v.41 no.4
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• pp.322-327
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• 2014

Regional odontodysplasia (RO) is a rare and nonhereditary dental malformation. It is a dental alteration of unknown etiology, involving both mesodermal and ectodermal dental components, which is characterized by clinical, radiographic, and histologic features. The maxilla is more often involved than the mandible (especially the left side), and there is no racial predilection, but females are affected twice as often as males. The affected teeth are clinically hypoplastic and hypocalcified, presenting a "ghost-like" appearance radiographically. The present case features a male patient aged 4 years and 6 months who was diagnosed with regional odontodysplasia in the maxilla on the right side, confirmed by clinical and radiographic examination, with a follow up of 5 years. Since teeth affected by RO have a poor prognosis due to the fragile tooth surface and open apices, the long-term treatment strategy depends on periodic clinical and radiological observations.

• Journal of the korean academy of Pediatric Dentistry
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• v.39 no.1
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• pp.97-102
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• 2012

Odontoma is a mixed tumor formed by the overgrowth of ectodermal epithelial cells and mesodermal cells. It arises during normal tooth development and only grows to a certain level. It is not a true neoplasm and therefore it is considered as hamartomas. Odontomas are classified into compound and complex types based on morphology. Compound odontoma is commonly found in the anterior region of the maxilla, and has a radiopaque lesion that resembles small teeth. Usually, odontoma is treated by conservative surgical removal and there is little probability of recurrence. This paper describes three cases of compound odontoma diagnosed in children with 1 year of follow-up. These case reports present patients having eruption disturbance of primary and permanent teeth due to the presence of compound odontoma. In order to induce spontaneous eruption, conservative surgical removal was performed showing favorable results.

• Tuberculosis and Respiratory Diseases
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• v.47 no.3
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• pp.400-405
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• 1999

Mediastinal teratomas are rare and represent less than 10 per cent of all mediastinal tumors. Almost all arise in the anterosuperior mediastinal compartment, and most symptoms, when present, result from compression of adjacent structures. They contain different tissues derived from all three germinal layers, with the prevalence of ectodermal elements which can include hair, teeth and sebaceous material. Benign teratomas may rupture into adjacent organs. Up to 36% of all mediastinal teratomas rupture, most frequently into the lung and bronchial tree, followed by the pleural space, pericardial space, or great vessels. The signs and symptoms of a ruptured teratoma vary with the structures involved. We report a case of mediastinal teratoma ruptured spontaneously in a 18 year old female who experienced 4 or 5 times of hemoptysis for 1 year and sudden onset of pleural effusion, pericardial effusion and pneumonia.