• Biomedical Science Letters
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• v.12 no.4
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• pp.413-418
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• 2006

Charcot-Marie-Tooth disease (CMT) is blown as one of the inherited disorder of peripheral nervous system. Recently, it was found that point mutations in the neurofilament light subunit (NF-L) gene cause CMT. Neurofilaments (NFs) are heteropolymers consist of NF-L, NF-M and NF-H. To assess the relationship between CMT and NF-L mutation in cellular level, we performed phenotypic analysis of the mutant NF-L (E397K) using cultured cell lines. Vimentin-deficient human adrenal carcinoma SW13 (Vim-) cells have a potential to form the intermediate filaments when the cells are expressing both NF-L and NF-M. Our results show that co-expression of wild type NF-L with NF-M showed intermediate filament formation in SW13 (Vim-) cells, while E397K with NF-M did not. This result means that E397K mutant lost its ability to form the intermediate filament in vivo, and further suggests that the E397K mutation is closely related to CMT.

• BMB Reports
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• v.51 no.7
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• pp.327-337
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• 2018

Lamin A and its alternative splicing product Lamin C are the key intermediate filaments (IFs) of the inner nuclear membrane intermediate filament. Lamin A/C forms the inner nuclear mesh with Lamin B and works as a frame with a nuclear shape. In addition to supporting the function of nucleus, nuclear lamins perform important roles such as holding the nuclear pore complex and chromatin. However, mutations on the Lamin A or Lamin B related proteins induce various types of human genetic disorders and diseases including premature aging syndromes, muscular dystrophy, lipodystrophy and neuropathy. In this review, we briefly overview the relevance of genetic mutations of Lamin A, human disorders and laminopathies. We also discuss a mouse model for genetic diseases. Finally, we describe the current treatment for laminopathies.

• Journal of The Korean Astronomical Society
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• v.42 no.1
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• pp.9-15
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• 2009

A necessary condition for the formation of a filament is magnetic helicity. In the present paper we seek the origin of magnetic helicity of intermediate filaments. We observed the formation of a sinistral filament at the boundary of a decaying active region using full-disk $H_{\alpha}$ images obtained from Bi Bear Solar Observatory. We have measured the rate of helicity injection during the formation of the filament using full-disk 96 minute-cadence magnetograms taken by SOHO MDI. As a result we found that 1) no significant helicity was injected around the region (polarity inversion line; PIL) of filament formation and 2) negative helicity was injected in the decaying active region. The negative sign of the injected helicity was opposite to that of the filament helicity. On the other hand, at earlier times when the associated active region emerged and grew, positive helicity was intensively injected. Our results suggest that the magnetic helicity of the intermediate filament may have originated from the helicity accumulated during the period of the growth of its associated active region.

• Korean Journal of Metals and Materials
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• v.50 no.12
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• pp.931-937
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• 2012

The microstructure of Cu-15 wt%Ag composites fabricated by equal channel angular pressing (ECAP) with intermediate heat treatment at $320^{\circ}C$ was investigated by transmission electron microscopy (TEM) observations. Ag precipitates with a thickness of 20-40 nm were observed in the eutectic region of the Cu-15 wt%Ag composite solution treated at $700^{\circ}C$ before ECAP. The Cu matrix and Ag precipitates had a cube on cube orientation relationship. ECAPed composites exhibited ultrafine-grained microstructures with the shape and distribution dependent on the processing routes. For route A in which the sample was pressed without rotation between each pass, the Cu and Ag grains were elongated along the shear direction and many micro-twins were observed in elongated Cu grains as well as in Ag filaments. The steps were observed on coherent twin boundaries in Cu grains. For route Bc in which the sample was rotated by 90 degrees after each pass, a subgrain structure with misorientation of 2-4 degree by fragmentation of the large Cu grains were observed. For route C in which the sample was rotated by 180 degrees after each pass, the microstructure was similar to that of the route A sample. However, the thickness of the elongated grains along the shear direction was wider than that of the route A sample and the twin density was lower than the route A sample. It was found that more microtwins were formed in ECAPed Cu-15 wt%Ag than in the drawn sample. Grain boundaries were observed in relatively thick and long Ag filaments in Cu-15 wt%Ag ECAPed by route C, indicating the multi-crystalline nature of Ag filaments.

• Proceedings of the Korean Institute of Electrical and Electronic Material Engineers Conference
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• 2003.07a
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• pp.597-600
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• 2003

Bi-2223/Ag HTS wires have been fabricated by the PIT(powder in tube)process. Intermediate annealing was carried out to increase the homogenization and uniformity of the superconducting filaments embedded in the silver matrix during the deformation process that is important to sustain the engineering critical current density in long superconducting wire. Intermediate annealing act to release the deformation hardening of the superconducting wires during drawing process. Rolling parameters were investigated to roll the superconducting tapes with uniform thickness, width and winding tensions. Critical current of 60 m long superconducting tapes was measured 54.3 A continuously after final sintering heat treatment. The phase analysis of Bi-2223/Ag superconducting tapes are examined by the XRD.

• Korean Journal of Materials Research
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• v.10 no.1
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• pp.41-48
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• 2000

In this study, microstructure and mechanical properties and electrical conductivities of in situ Cu-Fe-Xi(Xi=Ag, Cr or Co) alloy wires obtained by cold drawing combined with intermediate heat treatments have investigated. During cold working the primary and secondary dendrite arms are aligned along the drawing direction and elongated into filaments after deformation processing. The addition of Ag was found to be more effective in reducing the microstructural scale at the given draw ratio than that of Co or Cr throughout the drawing processing. The ultimate tensile strength and the conductivity of the Cu-Fe based composites containing Ag were higher than those of Cu-Fe composites containing Co or Cr. The good mechanical and electrical properties of Cu-Fe-Ag wires may be associated with the more uniform distribution of the finer filaments in the wires containing silver. The strength of Cu-Fe-Xi composites is dependent on the spacing of the Fe filaments in accord with a Hall-Petch relationship. The fracture surfaces of all the specimens showed ductile-type fracture and iron filaments occasionally observed on the fracture surfaces.

• Applied Microscopy
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• v.29 no.4
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• pp.437-450
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• 1999

Bile canaliculi are the structure delivering bile secreted by hepatocytes into the bile passage. Bile secretion is mainly controlled by the cytoskeletal elements, mainly of actin in the microvilli, pericanalicular web. Most studies on the bile secretion have been done in viva situation, however, to control the various parameters in vitro culture system seem to be more useful. To set up an in vitro experimental system, the investigator isolated hepatocytes with an enzymatic method using a mixture of collagenase and hyaluronidase from normal Sprague-Dawley rat liver and cultured. Isolated hepatocytes were round and formed cords in culture. Microvilli covered the whole surface of hepatocytes. Bile canaliculi were formed between hepatocytes and were characterized by the presence of microvilli of various lengths and shapes mainly arising from small surface mounds. Actin filament core in the microvilli and pericanalicular actin web were incomplete. After cytochalasin D treatment, cultured hepatocytes were round but the surface were irregular with surfacen blebs, folds and grooves. Microvilli on the surface were scarce. Bile canaliculi were markedly dilated often with the detached junctional complexes. Bile canaliculi lacks microvilli almost completely and extended into the pericanalirular cytoplasm showing complex vacuolar and tubular structures by transmission electron mciroscopy. Pericanalicular actin web, intermediate filaments were hardly identified. Subsurface actin filaments were scattered scarcely under the cell membranes. These results suggest that hepatocytes isolated from rats can survive and form bile canaliculi in culture and the actin filaments are involved in the formation and/or maintenance of the bile canaliculi.

• 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.

• Progress in Superconductivity and Cryogenics
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• v.6 no.1
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• pp.1-5
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• 2004

Long length of Bi-2223/Ag superconducting wives were fabricated by stacking and drawing process with advanced heat-treatment schedules. Intermediate annealing was carried out to increase the homogeneity and uniformity of the superconducting filaments embedded in the silver matrix. Phase modification from tetragonal to orthorhombic Bi-2212 by pre heat treatment(PHT) was executed to improve the texture and phase transformation of Bi-2223. Drawing stress was measured to predict the sausaging and stress limit. Rolling Parameters such as thickness. width and winding tension were investigated to roll the tape with uniformity. 1 km length of Bi-2223/Ag superconducting wires were fabricated without any breakage. Critical current (Je) of 270 m length of superconducting tapes was measured over than $70 A/cm^2$ continuously after final sintering.

• The Bulletin of The Korean Astronomical Society
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• v.42 no.2
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• pp.79.1-79.1
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• 2017

The B-fields In STar-forming Region Observations (BISTRO) is the 3-year large program of the James Clerk Maxwell Telescope (JCMT) using SCUBA-2 and POL-2, started in 2016. We aim to study the roles of magnetic fields in star formation by observing 16 fields of nearby star forming regions, e.g., Orion and Ophiuchus molecular clouds. The angular resolution and wavelength provided by JCMT (14 arcsecond at 850 micrometer) is ideal to investigate the intermediate scales of magnetic fields (1000-10000 au) associated in cold dense cores and filaments. This year, moreover, we were awarded JCMT time for additional 16 fields (BISTRO-2), which allows us to cover broader physical properties of star forming regions. We report the current status of BISTRO and introduce BISTRO-2.