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Tanit Guitart Daria Picchioni David Pi?eyro Lluís Ribas de Pouplana 《Nucleic acids research》2013,41(13):6595-6608
The translation of genes encoded in the mitochondrial genome requires specific machinery that functions in the organelle. Among the many mutations linked to human disease that affect mitochondrial translation, several are localized to nuclear genes coding for mitochondrial aminoacyl-transfer RNA synthetases. The molecular significance of these mutations is poorly understood, but it is expected to be similar to that of the mutations affecting mitochondrial transfer RNAs. To better understand the molecular features of diseases caused by these mutations, and to improve their diagnosis and therapeutics, we have constructed a Drosophila melanogaster model disrupting the mitochondrial seryl-tRNA synthetase by RNA interference. At the molecular level, the knockdown generates a reduction in transfer RNA serylation, which correlates with the severity of the phenotype observed. The silencing compromises viability, longevity, motility and tissue development. At the cellular level, the knockdown alters mitochondrial morphology, biogenesis and function, and induces lactic acidosis and reactive oxygen species accumulation. We report that administration of antioxidant compounds has a palliative effect of some of these phenotypes. In conclusion, the fly model generated in this work reproduces typical characteristics of pathologies caused by mutations in the mitochondrial aminoacylation system, and can be useful to assess therapeutic approaches. 相似文献
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Jan Piťha 《Cell and tissue research》1968,90(4):563-569
Summary Paneth cells of rabbit small intestine almost constantly contain inclusions of variable size which consist of two components. The first consists of cylindrical bodies formed by concentric or spiral dense lamellae. The second is formed by tubules with triple-layered walls which may be arranged in a hexagonal pattern. The density of arrangement of both components is variable. The structure of inclusions is discussed in relation to their composition and derivation and lipid or lipoprotein composition is assumed. 相似文献
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Alexandrium taylori Balech is a phototrophic marine dinoflagellate. It produced recurrent blooms during the summer months (July and August) of 1994 to 1997 in La Fosca beach (NW Mediterranean). In addition to a motile vegetative form, A. taylori had two benthic forms: temporary cysts and resting cysts. Temporary cysts were a temporally quiescent stage produced from the ecdysis of the vegetative cell in both natural populations and laboratory cultures. Temporary cysts may divide to form motile cells. Resting cysts had a thicker wall than the temporary cysts and had a red accumulation body. Gametes and planozygotes were also observed in laboratory cultures. Alexandrium taylori showed in situ diurnal vertical migration with an increase of vegetative cells in the water column in the morning through midday, with concentrations peaking in the afternoon followed by lower levels at night. Most vegetative cells lost their thecae and flagella, and with them their motility, turning into temporary cysts that settled in the early evening. The number of temporary cysts in the water column rose in the evening and at night. The temporary cysts gave rise to motile cells the following morning. Synthesis of DNA occurred in vegetative cells at night, and a preferential period of cell division occurred at sunrise. The estimated division rate in the field was 0.4–0.5 vegetative cells·day−1 . Temporary cysts had twice the DNA of a G1 vegetative cell. The minimum in situ division rate of the temporary cysts was 0.14 day−1 . The role of the resting and temporary cyst population in the annual recurrence and maintenance of the A. taylori bloom is discussed. 相似文献