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451.
452.
Thalia Salinas Samira El?Farouk-Ameqrane Elodie Ubrig Claude Sauter Anne-Marie Duchêne Laurence Maréchal-Drouard 《Nucleic acids research》2014,42(15):9937-9948
In plants, the voltage-dependent anion-selective channel (VDAC) is a major component of a pathway involved in transfer RNA (tRNA) translocation through the mitochondrial outer membrane. However, the way in which VDAC proteins interact with tRNAs is still unknown. Potato mitochondria contain two major mitochondrial VDAC proteins, VDAC34 and VDAC36. These two proteins, composed of a N-terminal α-helix and of 19 β-strands forming a β-barrel structure, share 75% sequence identity. Here, using both northwestern and gel shift experiments, we report that these two proteins interact differentially with nucleic acids. VDAC34 binds more efficiently with tRNAs or other nucleic acids than VDAC36. To further identify specific features and critical amino acids required for tRNA binding, 21 VDAC34 mutants were constructed and analyzed by northwestern. This allowed us to show that the β-barrel structure of VDAC34 and the first 50 amino acids that contain the α-helix are essential for RNA binding. Altogether the work shows that during evolution, plant mitochondrial VDAC proteins have diverged so as to interact differentially with nucleic acids, and this may reflect their involvement in various specialized biological functions. 相似文献
453.
Fukuyama-type congenital muscular dystrophy (FCMD, MIM#253800) is an autosomal recessive disorder characterized by severe muscular dystrophy associated with brain malformations. FCMD is the second most common form of muscular dystrophy after Duchenne muscular dystrophy and one of the most common autosomal recessive diseases among the Japanese population, and yet few patients outside of Japan had been reported with this disorder. We report the first known Egyptian patient with FCMD, established by clinical features of generalized weakness, pseudohypertrophy of calf muscles, progressive joint contractures, severe scoliosis, elevated serum creatine kinase level, myopathic electrodiagnostic changes, brain MRI with cobblestone complex, and mutation in the fukutin gene. In addition, our patient displayed primary microcephaly, not previously reported associated with fukutin mutations. Our results expand the geographic and clinical spectrum of fukutin mutations. 相似文献
454.
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456.
Gislaine T. Rezin Camila B. Furlanetto Giselli Scaini Samira S. Valvassori Cinara L. Gonçalves Gabriela K. Ferreira Isabela C. Jeremias Wilson R. Resende Mariane R. Cardoso Roger B. Varela João Quevedo Emilio L. Streck 《Molecular neurobiology》2014,49(2):877-892
Fenproporex (Fen) is converted in vivo into amphetamine, which is used to induce mania-like behaviors in animals. In the present study, we intend to present a new animal model of mania. In order to prove through face, construct, and predictive validities, we evaluated behavioral parameters (locomotor activity, stereotypy activity, and fecal boli amount) and brain energy metabolism (enzymes citrate synthase; malate dehydrogenase; succinate dehydrogenase; complexes I, II, II–III, and IV of the mitochondrial respiratory chain; and creatine kinase) in rats submitted to acute and chronic administration of fenproporex, treated with lithium (Li) and valproate (VPA). The administration of Fen increased locomotor activity and decreased the activity of Krebs cycle enzymes, mitochondrial respiratory chain complexes, and creatine kinase, in most brain structures evaluated. In addition, treatment with mood stabilizers prevented and reversed this effect. Our results are consistent with the literature that demonstrates behavioral changes and mitochondrial dysfunction caused by psychostimulants. These findings suggest that chronic administration of Fen may be a potential animal model of mania. 相似文献
457.
Prolonged root hypoxia effects on enzymes involved in nitrogen assimilation pathway in tomato plants 总被引:1,自引:0,他引:1
In order to investigate the effects of root hypoxia (1–2% oxygen) on the nitrogen (N) metabolism of tomato plants (Solanum lycopersicum L. cv. Micro-Tom), a range of N compounds and N-assimilating enzymes were performed on roots and leaves of plants submitted to root hypoxia at the second leaf stage for three weeks. Obtained results showed that root hypoxia led to a significant decrease in dry weight (DW) production and nitrate content in roots and leaves. Conversely, shoot to root DW ratio and nitrite content were significantly increased. Contrary to that in leaves, glutamine synthetase activity was significantly enhanced in roots. The activities of nitrate and nitrite reductase were enhanced in roots as well as leaves. The higher increase in the NH4+ content and in the protease activities in roots and leaves of hypoxically treated plants coincide with a greater decrease in soluble protein contents. Taken together, these results suggest that root hypoxia leaded to higher protein degradation. The hypoxia-induced increase in the aminating glutamate dehydrogenase activity may be considered as an alternative N assimilation pathway involved in detoxifying the NH4+, accumulated under hypoxic conditions. With respect to hypoxic stress, the distinct sensitivity of the enzymes involved in N assimilation is discussed.Key words: tomato, hypoxia, nitrogen, glutamine synthetase, protease, glutamate dehydrogenase 相似文献
458.
Benhamouche S Decaens T Godard C Chambrey R Rickman DS Moinard C Vasseur-Cognet M Kuo CJ Kahn A Perret C Colnot S 《Developmental cell》2006,10(6):759-770
The molecular mechanisms by which liver genes are differentially expressed along a portocentral axis, allowing for metabolic zonation, are poorly understood. We provide here compelling evidence that the Wnt/beta-catenin pathway plays a key role in liver zonation. First, we show the complementary localization of activated beta-catenin in the perivenous area and the negative regulator Apc in periportal hepatocytes. We then analyzed the immediate consequences of either a liver-inducible Apc disruption or a blockade of Wnt signaling after infection with an adenovirus encoding Dkk1, and we show that Wnt/beta-catenin signaling inversely controls the perivenous and periportal genetic programs. Finally, we show that genes involved in the periportal urea cycle and the perivenous glutamine synthesis systems are critical targets of beta-catenin signaling, and that perturbations to ammonia metabolism are likely responsible for the death of mice with liver-targeted Apc loss. From our results, we propose that Apc is the liver "zonation-keeper" gene. 相似文献
459.
Ivan Rwomushana Fathiya M. Khamis Tim G. Grout Samira A. Mohamed Mamoudou Sétamou Christian Borgemeister Hellen M. Heya Chrysantus M. Tanga Peterson W. Nderitu Zuberi S. Seguni Christopher L. Materu Sunday Ekesi 《Biological invasions》2017,19(10):2777-2787
The Asian citrus psyllid Diaphorina citri Kuwayama (Hemiptera: Liviidae) is a damaging pest of citrus globally and has recently been detected in Tanzania. Although direct damage by the pest is seldom of economic importance, the insect is more notorious for its ability to vector the fastidious phloem-limited bacterium Candidatus Liberibacter asiaticus (CLas), the putative causal bacterium of Huanglongbing or Asian citrus greening disease. For many years, Trioza erytreae (Del Guercio) (Hemiptera: Triozidae) was known to be the main vector of the African citrus greening disease caused by Candidatus Liberibacter africanus (CLaf), but the recent arrival of D. citri on the continent adds to the dynamics of infection and spread of both diseases on mainland Africa. Following the recent report of the presence of D. citri in Tanzania, an additional delimiting survey was carried out in the region, focusing on Kenya, mainland Tanzania and Zanzibar to detect the presence and ascertain the extent of spread of D. citri. We employed molecular tools based on the use of DNA barcoding to confirm the identity of D. citri. In addition to D. citri, the occurrence of T. erytreae in the same sampling locations is also reported. Adults and nymphs of either D. citri or T. erytreae were collected from citrus at many of the surveyed sites ranging from 19 to 668 m above sea level (masl) in Tanzania, 20–1666 masl in Kenya, and 42–48 masl in Zanzibar. Diaphorina citri was sympatric with T. erytreae at the mid to higher elevations of 1375–1666 masl and no T. erytreae or its open-gall symptoms were detected below 523 masl. Sequences obtained were queried via BLAST and all linked to D. citri of different accession numbers already available on GenBank. This is the first report of the presence of D. citri in Kenya and Zanzibar. The potential implication of the detection and spread of the two pathogens, CLaf and CLas to the citrus industry in East Africa and movement of suitable host plants is discussed. 相似文献
460.
Mutations in MDH2, Encoding a Krebs Cycle Enzyme,Cause Early-Onset Severe Encephalopathy 总被引:1,自引:0,他引:1
Samira Ait-El-Mkadem Manal Dayem-Quere Mirjana Gusic Annabelle Chaussenot Sylvie Bannwarth Bérengère François Emmanuelle C. Genin Konstantina Fragaki Catharina L.M. Volker-Touw Christelle Vasnier Valérie Serre Koen L.I. van Gassen Françoise Lespinasse Susan Richter Graeme Eisenhofer Cécile Rouzier Fanny Mochel Anne De Saint-Martin Véronique Paquis-Flucklinger 《American journal of human genetics》2017,100(1):151-159