• 제목/요약/키워드: Nuclear envelope

검색결과 101건 처리시간 0.021초

Genetic Variations Leading to Familial Dilated Cardiomyopathy

  • Cho, Kae Won;Lee, Jongsung;Kim, Youngjo
    • Molecules and Cells
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    • 제39권10호
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    • pp.722-727
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    • 2016
  • Cardiomyopathy is a major cause of death worldwide. Based on pathohistological abnormalities and clinical manifestation, cardiomyopathies are categorized into several groups: hypertrophic, dilated, restricted, arrhythmogenic right ventricular, and unclassified. Dilated cardiomyopathy, which is characterized by dilation of the left ventricle and systolic dysfunction, is the most severe and prevalent form of cardiomyopathy and usually requires heart transplantation. Its etiology remains unclear. Recent genetic studies of single gene mutations have provided significant insights into the complex processes of cardiac dysfunction. To date, over 40 genes have been demonstrated to contribute to dilated cardiomyopathy. With advances in genetic screening techniques, novel genes associated with this disease are continuously being identified. The respective gene products can be classified into several functional groups such as sarcomere proteins, structural proteins, ion channels, and nuclear envelope proteins. Nuclear envelope proteins are emerging as potential molecular targets in dilated cardiomyopathy. Because they are not directly associated with contractile force generation and transmission, the molecular pathways through which these proteins cause cardiac muscle disorder remain unclear. However, nuclear envelope proteins are involved in many essential cellular processes. Therefore, integrating apparently distinct cellular processes is of great interest in elucidating the etiology of dilated cardiomyopathy. In this mini review, we summarize the genetic factors associated with dilated cardiomyopathy and discuss their cellular functions.

웅담(熊膽).우황(牛黃) 약침(藥鍼)의 효능(效能) 관찰(觀察)을 위한 미세구조적(微細構造的) 연구(硏究) (Ultrastructural Study to the Effects of Fel Ursi and Calculus Bovis Aqua-acupuncture)

  • 나창수;김정상;김희철;김병수;황우준
    • 대한한의학회지
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    • 제18권1호
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    • pp.430-445
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    • 1997
  • In order to know the effects of Calculus $Bovis{\cdot}F디$ Ursi aqua-acupuncture ($C{\cdot}F$ aqua-acupuncture) to the liver damage, we carried out the treatment of $C{\cdot}F$ aqua-acupuncture and injection of Kam-Du-Tang on the liver damaged rat which is induced by aconitine, and then ultrastructural study has been to the liver tissue. The results were as follows: 1. In the hepatic cell of normal group, the nuclear envelope is round and electro-density of nucleoplasm is relatively low, and have one or two large nucleolus. Cell organelle is developed rough endoplasmic reticulum(RER) and many mitochondria, glycogen granules, smooth endoplasmic reticulum(SER) and Golgi apparatus. 2. In 24 hours control group, nuclear envelope is very irregular, RER and most of cell organell is shown that destroyed by aconitine. This is similar to the 48 hours control group. In 72 hours control group, RER is recovered somewhat but cristae of mitochondria is not seen the shape 3.In 24 hours $C{\cdot}F$ aqua-acupuncture group, nuclear envelope is very irregular and cristernae is very dilatable and many mitochondria is dilationed and the cristae is not definite like the control group. In 48 hours $C{\cdot}F$ aqua-acupuncture group, nuclear envelope is relatively round and chromatin is regular. Cristernae of RER and ribosme is somewhat imperfect but is similar the normal group, and the cristae of mitchondria is shown definitely like the normal group. In 72 hours $C{\cdot}F$ aqua-acupuncture group, nuclear envelope is round and cell organell is similar to normal group. 4. In 24 hours Kam-Du-Tang group, nuclear envlope is very irregular and cell organelles are destroyed by the effects of aconitine. In 48 hours Kam-Du-Tang group, nuclear envelope is somewhat irregular but chromatin is relatively regular. RER is seperated regularly through the cytoplasm, but cannot make the structure of lamina, and cistennae is very dilationed. many of glycogen granules is seperated regularly. In 72 hours Kam-Du-Tang group, RER makes the lamina. Mitochondria is a little but inner space is dilationed so the shape of is not definite and a lot of glycogen granules are accumulated in several places. It see the above result, hepatic cell that is damaged by aconitine is effected chiefly by the $C{\cdot}F$ acupuncture and effect of acupuncture is similar to the detoxicated effect of Kam-Du-Tang.

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Targeting of Nuclear Encoded Proteins to Chloroplasts: a New Insight into the Mechanism

  • Lee, Yong-Jik;Kim, Yong-Woo;Pih, Kyeong-Tae;Hwang, Inhwan
    • 식물조직배양학회지
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    • 제27권5호
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    • pp.407-409
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    • 2000
  • Outer envelope membrane proteins of chloroplasts encoded by the nuclear genome are transported without the N-terminal transit peptide. Here, we investigated the targeting mechanism of AtOEP7, an Arabidopsis homolog of small outer envelope membrane proteins in vivo. AtOEP7 was expressed transiently in protoplasts or stably in transgenic plants as fusion proteins with GFP. In both cases AtOEP7:GFP was targeted to the outer envelope membrane when assayed under a fluorescent microscope or by Western blot analysis. Except the transmembrane domain, deletions of the N- or C-terminal regions of AtOEP7 did not affect targeting although a region closed to the C-terminal side of the transmembrane domain affected the targeting efficiency. Targeting experiments with various hybrid transmembrane mutants revealed that the amino acid sequence of the transmembrane domain determines the targeting specificity The targeting mechanism was further studied using a fusion protein, AtOEP7:NLS:GFP, that had a nuclear localization signal. AtOEP7:NLS:GFP was efficiently targeted to the chloroplast envelope despite the presence of the nuclear localization signal. Taken together, these results suggest that the transmembrane domain of AtOEP7 functions as the sole determinant of targeting specificity and that AtOEP7 may be associated with a cytosolic component during translocation to the chloroplast envelope membrane.

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Sensitivity study of parameters important to Molten Salt Reactor Safety

  • Sarah Elizabeth Creasman;Visura Pathirana;Ondrej Chvala
    • Nuclear Engineering and Technology
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    • 제55권5호
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    • pp.1687-1707
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    • 2023
  • This paper presents a molten salt reactor (MSR) design parameter sensitivity study using a nodal dynamic modelling methodology with explicitly modified point kinetics equation and Mann's model for heat transfer. Six parameters that can impact MSR safety are evaluated. A MATLAB-Simulink model inspired by Thorcon's 550MWth MSR is used for parameter evaluations. A safety envelope was formed to encapsulate power, maximum and minimum temperature, and temperature-induced reactivity feedback. The parameters are perturbed by ±30%. The parameters were then ranked by their subsequent impact on the considered safety envelope, which ranks acceptable parameter uncertainty. The model is openly available on GitHub.

사철나무 엽맥 바이러스의 형상과 세포내출현 (Morphology and Intracellular Appearance of Euonymus Vein Clear Virus)

  • 장무웅
    • 한국식물병리학회지
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    • 제2권1호
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    • pp.1-11
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    • 1986
  • Dip-method에 의한 전자현미경적 관찰의 결과, 사철나무 이병엽에서 길이 230-280nm, 폭 70-80nm의 단간형(일단이 둥근 탄환형 : bullef-shaped, 양단이 둥근 간균형 : bacilliform)의 바이러스 입자가 다수 검출되었다. 이 바이러스 입자를 사철나무 엽맥황화 바이러스(Euonymus vein clear virus : EVCV)로 명명하였다. EVCV는 피막(두께 8-10nm)을 갖고 있고, 이 피막의 표면에는 5-6nm의 염주모양의 돌기가 있다. 핵단백(nucleocapsid)은 길이 200-220nm, 폭 50-55nm의 나선구조를 갖고 있다. 바이러스 입자는 절편시료의 각종세포의 세포질내 또는 핵내와 핵막간극내에 산재 혹은 집단으로 관찰되었다. 이들 바이러스 입자의 인체는 핵막으로 둘러싸여 있었다. 대부분의 절편상에서는 바이러스 입자의 집단이 핵막의 내막과 외막 사이의 간극에서 관찰되었는데, 이것은 바이러스 입자의 성숙장을 시사하고 있는 것으로 생각된다. 어떤 절편상에서는 바이러스 입자의 피막이 핵막의 내막과 연결되어 있는 것도 관찰되어, 이 피막이 핵막의 내막으로부터 유래된 것으로 시사되었다.

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N-Acetyl-D-Glucosamine Kinase Interacts with Dynein-Lis1-NudE1 Complex and Regulates Cell Division

  • Sharif, Syeda Ridita;Islam, Md. Ariful;Moon, Il Soo
    • Molecules and Cells
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    • 제39권9호
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    • pp.669-679
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    • 2016
  • N-acetyl-D-glucosamine kinase (GlcNAc kinase or NAGK) primarily catalyzes phosphoryl transfer to GlcNAc during amino sugar metabolism. Recently, it was shown NAGK interacts with dynein light chain roadblock type 1 (DYNLRB1) and upregulates axo-dendritic growth, which is an enzyme activity-independent, non-canonical structural role. The authors examined the distributions of NAGK and NAGK-dynein complexes during the cell cycle in HEK293T cells. NAGK was expressed throughout different stages of cell division and immunocytochemistry (ICC) showed NAGK was localized at nuclear envelope, spindle microtubules (MTs), and kinetochores (KTs). A proximity ligation assay (PLA) for NAGK and DYNLRB1 revealed NAGK-dynein complex on nuclear envelopes in prophase cells and on chromosomes in metaphase cells. NAGK-DYNLRB1 PLA followed by Lis1/NudE1 immunostaining showed NAGK-dynein complexes were colocalized with Lis1 and NudE1 signals, and PLA for NAGK-Lis1 showed similar signal patterns, suggesting a functional link between NAGK and dynein-Lis1 complex. Subsequently, NAGK-dynein complexes were found in KTs and on nuclear membranes where KTs were marked with CENP-B ICC and nuclear membrane with lamin ICC. Furthermore, knockdown of NAGK by small hairpin (sh) RNA was found to delay cell division. These results indicate that the NAGK-dynein interaction with the involvements of Lis1 and NudE1 plays an important role in prophase nuclear envelope breakdown (NEB) and metaphase MT-KT attachment during eukaryotic cell division.

An improved time-domain approach for the spectra-compatible seismic motion generation considering intrinsic non-stationary features

  • Feng Cheng;Jianbo Li;Zhixin Ding;Gao Lin
    • Nuclear Engineering and Technology
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    • 제55권3호
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    • pp.968-980
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    • 2023
  • The dynamic structural responses are sensitive to the time-frequency content of seismic waves, and seismic input motions in time-history analysis are usually required to be compatible with design response spectra according to nuclear codes. In order to generate spectra-compatible input motions while maintaining the intrinsic non-stationarity of seismic waves, an improved time-domain approach is proposed in this paper. To maintain the nonstationary characteristics of the given seismic waves, a new time-frequency envelope function is constructed using the Hilbert amplitude spectrum. Based on the intrinsic mode functions (IMFs) obtained from given seismic waves through variational mode decomposition, a new corrective time history is constructed to locally modify the given seismic waves. The proposed corrective time history and time-frequency envelope function are unique for each earthquake records as they are extracted from the given seismic waves. In addition, a dimension reduction iterative technique is presented herein to simultaneously superimpose corrective time histories of all the damping ratios at a specific frequency in the time domain according to optimal weights, which are found by the genetic algorithm (GA). Examples are presented to show the capability of the proposed approach in generating spectra-compatible time histories, especially in maintaining the nonstationary characteristics of seismic records. And numerical results reveal that the modified time histories generated by the proposed method can obtain similar dynamic behaviors of AP1000 nuclear power plant with the natural seismic records. Thus, the proposed method can be efficiently used in the design practices.