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101.
This study reports on a patient with Leigh syndrome with a T-to-C mutation at nucleotide 8993 of mitochondrial deoxyribonucleic acid (T8993C). The authors reviewed 10 Leigh syndrome patients, including ours, with T8993C. Compared with 18 reported patients with Leigh syndrome caused by a T-to-G mutation at nucleotide 8993 (T8993G), Leigh syndrome with T8993C was characterized by a significantly higher frequency of ataxia (P < 0.01). None of the reviewed T8993C-associated Leigh syndrome patients had retinitis pigmentosa, which is one of the characteristic findings in Leigh syndrome with T8993G. The milder symptoms of T8993C-Leigh syndrome can be explained by the milder complex V dysfunction; however, the higher frequency of ataxia in T8993C-Leigh syndrome requires more study. 相似文献
102.
M Urushitani S Shimohama T Kihara H Sawada A Akaike M Ibi R Inoue Y Kitamura T Taniguchi J Kimura 《Canadian Metallurgical Quarterly》1998,44(5):796-807
In this study, we analyzed the mechanism of selective motor neuronal death, a characteristic of amyotrophic lateral sclerosis, using embryonic rat spinal cord culture. When dissociated cultures were exposed to low-level glutamate (Glu) coadministered with the Glu transporter inhibitor L-trans-pyrrolidine-2,4-decarboxylate (PDC) for 24 hours, motor neurons were selectively injured through N-methyl-D-aspartate (NMDA) and alpha-amino-3-hydroxy-5-methylisoxazole-4-propionate (AMPA)/kainate receptors. Nitric oxide synthase (NOS) inhibitors attenuated this toxicity, and long-acting nitric oxide (NO) donors damaged motor neurons selectively. Nonmotor neurons survived after exposure to low-dose Glu/PDC, but Glu-induced toxicity was potentiated by coadministration of an NO-dependent guanylyl cyclase inhibitor. In addition, 8-bromo-cyclic GMP, a soluble cyclic GMP analogue, rescued nonmotor neurons, but not motor neurons, exposed to high-dose Glu/PDC. Twenty-four hours' incubation with PDC elevated the number of neuronal NOS-immunoreactive neurons by about twofold compared with controls, and a double-staining study, using the motor neuron marker SMI32, revealed that most of them were nonmotor neurons. These findings suggest that selective motor neuronal death caused by chronic low-level exposure to Glu is mediated by the formation of NO in nonmotor neurons, which inversely protects nonmotor neurons through the guanylyl cyclase-cyclic GMP cascade. Induction of neuronal NOS in nonmotor neurons might enhance both the toxicity of motor neurons and the protection of nonmotor neurons, which could explain the pathology of amyotrophic lateral sclerosis. 相似文献
103.
Micro‐porous copolymer hydrogels were prepared by γ‐ray irradiation of mixed solutions of N‐isopropylacrylamide (NIPAAm) and acrylic acid (AAc) above the lower critical solution temperature (LCST). From Cryo‐SEM observations, the gels were found to consist of three‐dimensional fibrous micro‐gels and micro‐pores. The copolymer gels swelled at temperatures below the LCST and shrunk at temperatures above it, and they showed rapid volume transitions on a time scale on the order of a minute when experiencing temperature changes between 10 and 40°C. The transition times for thermal shrinking were almost the same regardless of AAc composition, but the transition times for thermal swelling were increased with increasing AAc contents. The copolymer gels also showed rapid volume transitions with time constants on the order of an hour on experiencing pH changes between 2 and 12. The transition times for pH volume change at 10°C were within one hour, except for the gels containing only small amounts of AAc. On the other hand, the transition times for pH‐dependent volume change at 40°C were increased with increasing AAc content. The lower responsiveness of the transition results from an increase in hydrophobicity arising from the formation of inter‐ and intra‐molecular hydrogen bonds between the non‐ionized carboxylic acid groups and the amide groups. © 2003 Wiley Periodicals, Inc. J Appl Polym Sci 89: 75–84, 2003 相似文献
104.
I Nobuhisa S Inamasu M Nakai A Tatsui T Mimori T Ogawa Y Shimohigashi Y Fukumaki S Hattori H Kihara M Ohno 《Canadian Metallurgical Quarterly》1997,249(3):838-845
The proteins that bind phospholipase A2 (PLA2) isozymes of Trimeresurus flavoviridis (habu snake, crotalinae) venom were fractionated from sera on four columns, each conjugated with one of four PLA2 isozymes. Five proteins, termed PLA2 inhibitors (PLI) I-V, were obtained as the binding components. The combinations of the binding components differed depending on the PLA2 isozymes. PLI-IV and PLI-V correspond to PLI-A and PLI-B, respectively, which were known to bind to a major [Asp49]PLA2, PLA2, and contained a segment similar to the carbohydrate-recognition domain of C-type lectins. PLI-I, which is a major component of inhibitory proteins against three basic PLA2 isozymes, PLA-B (a basic [Asp49]PLA2) and basic proteins I and II (both [Lys49]PLA2s), has been isolated, and its partial amino acid sequence has been determined. A cDNA encoding PLI-I was isolated from a T. flavoviridis liver cDNA library and sequenced. PLI-I cDNA encoded 200 amino acid residues, including a signal peptide of 19 amino acid residues. One sugar chain was predicted to occur at position 157. A gene coding for PLI-I was isolated. It is 9.6-kb long and consists of five exons and four introns. Comparison of the exon-intron structure of the PLI-I gene with those of genes encoding urokinase-type-plasminogen-activator receptor (uPAR), Ly-6, CD59 and neurotoxins showed that they have characteristic unit encoding approximately 90 amino acid residues, which is divided over two exons. This strongly suggests that the PLI-I gene belongs to the uPAR, Ly-6, CD59 and neurotoxin gene family. There are two types of structurally different inhibitors against PLA2 isozymes in T. flavoviridis serum with different evolutionary origins. 相似文献
105.
106.
THREE DIMENSIONAL ELASTO-PLASTIC CONTACT BOUNDARY ELEMENT ANALYSIS FOR ROLLING WITH CONSIDERATION OF FRICTION 总被引:4,自引:0,他引:4
Xiao Hong Chen Yiming Shen Guangxian Yanshan University Aizawa Tatsuhiko Kihara Junji University of Tokyo Tokyo Japan 《机械工程学报(英文版)》1998,(1)
THREEDIMENSIONALELASTOPLASTICCONTACTBOUNDARYELEMENTANALYSISFORROLLINGWITHCONSIDERATIONOFFRICTION①XiaoHongChenYimingShenGuang... 相似文献
107.
AC Baruzzi E Knobel C Cirenza EN Kihara VC Souza A Massaro PP Porto Júnior PH Monzillo AA Gabbai 《Canadian Metallurgical Quarterly》1997,68(5):347-351
Recently, a new isoform of the type II transforming growth factor beta receptor (TGF-beta RII) was identified. This isoform (TGF-beta RII2) contains an insertion of 25 amino acids in the extracellular domain of the receptor. Using RT-PCR the authors demonstrated that both TGF-beta RII1 and TGF-beta RII2 are expressed by chondrocytes in murine and human articular cartilage. Bovine articular chondrocytes expressed TGF-beta RII1 mRNA but did not express detectable levels of TGF-beta RII2 mRNA, suggesting that the new isoform does not play an important role in normal bovine cartilage physiology. Because TGF-beta responses seem to be age related and differential TGF-beta responses have been described between normal cartilage and cartilage undergoing repair the authors studied if the relative mRNA expression between these isoforms is altered during cartilage repair and aging. No differences in the relative mRNA expression of the two isoforms of the type II TGF-beta receptor could be demonstrated in murine cartilage during aging or during the repair phase after mild PG depletion indicating that it is unlikely that age-related TGF-beta responses and differential TGF-beta responses between normal cartilage and cartilage undergoing repair are the result of differences in the relative expression of the two TGF-beta RII isoforms. 相似文献
108.
109.
The irradiation behavior of uranium-plutonium mixed oxide fuels containing a large amount of silicon impurity was examined by post-irradiation examination. Influences of Si impurity on fuel restructuring and cladding attack were investigated in detail. Si impurity, along with Am, Pu and O were transported by spherical pores and cylindrical tubular pores to the fuel center during fuel restructuring of the Np-Am-MOX fuel, where a eutectic reaction of fuel and Si-rich inclusions occurred. After fuel restructuring of the Np-Am-MOX fuel, Si-rich inclusions without fuel constituents were agglomerated at fuel crack openings where shallow attacks on the inner wall of the cladding were seen. Such shallow attacks on the inner wall of the cladding were likewise observed near the location of fuel cracks in long-term steady-state irradiated MOX fuels. Evidence of these shallow attacks on the inner wall of the cladding remained after fuel restructuring in normal MOX fuel. However, grain boundary corrosion of the cladding inner wall at the opening of the fuel cracks was selective and was marked in MOX fuel at higher oxygen potential by the release of reactive fission products such as Cs and Te in comparison with other regions of cladding wall. 相似文献
110.
The synthesis and formation of MoSi2, Mo5Si3, and Mo3Si compounds by the mechanical alloying of MoSi powder mixtures has been investigated. Ball-milling experiments were conducted for the composition range of 10–80 at.% Si. The formation of molybdenum silicides, especially MoSi2, during mechanical alloying and the relevant reaction rates markedly depended on the powder composition. The spontaneous formation of MoSi2 during mechanical alloying at 67 at.% Si (MoSi2 stoichiometry) proceeded by a mechanically-induced self-propagating reaction (MSR), the mechanism of which is analogous to that of the self-propagating high-temperature synthesis (SHS). At the compositions of 54 and 80 at.% Si, however, the formation of MoSi2 proceeded by the gradual formation of both the and /gb phases instead of the MSR mode. The formation of Mo5Si3 during mechanical alloying was characterized by a slow reaction rate as the reactants and product coexisted over a long period. The milling of Mo-rich powder mixtures up to 150 h did not lead to the direct formation of Mo3Si. The Mo3Si phase appeared only after brief annealing at temperatures of 800°C and above. 相似文献