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51.
52.
Metabolic Dysfunction in Familial, but Not Sporadic, Amyotrophic Lateral Sclerosis 总被引:12,自引:2,他引:12
Susan E. Browne †Allen C. Bowling M. Jay Baik ‡Mark Gurney †Robert H. Brown Jr. M. Flint Beal 《Journal of neurochemistry》1998,71(1):281-287
Abstract: Autosomal dominant familial amyotrophic lateral sclerosis (FALS) is associated with mutations in the gene encoding Cu/Zn superoxide dismutase (SOD1). Previous studies have implicated the involvement of metabolic dysfunction in ALS pathogenesis. To further investigate the biochemical features of FALS and sporadic ALS (SALS), we examined SOD activity and mitochondrial oxidative phosphorylation enzyme activities in motor cortex (Brodmann area 4), parietal cortex (Brodmann area 40), and cerebellum from control subjects, FALS patients with and without known SOD mutations, SALS patients, and disease controls (Pick's disease, progressive supranuclear palsy, diffuse Lewy body disease). Cytosolic SOD activity, predominantly Cu/Zn SOD, was decreased ∼50% in all regions in FALS patients with SOD mutations but was not significantly altered in other patient groups. Marked increases in complex I and II–III activities were seen in FALS patients with SOD mutations but not in SALS patients. We also measured electron transport chain enzyme activities in a transgenic mouse model of FALS. Complex I activity was significantly increased in the forebrain of 60-day-old G93A transgenic mice overexpressing human mutant SOD1, relative to levels in transgenic wild-type animals, supporting the hypothesis that the motor neuron disorder associated with SOD1 mutations involves a defect in mitochondrial energy metabolism. 相似文献
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Sarah E. Gutowsky Yann Tremblay Michelle A. Kappes Elizabeth N. Flint John Klavitter Leona Laniawe Dan P. Costa Maura B. Naughton Marc D. Romano Scott A. Shaffer 《Ibis》2014,156(1):60-72
Past tracking studies of marine animals have primarily targeted adults, biasing our understanding of at‐sea habitat use toward older life stages. Anthropogenic threats persist throughout the at‐sea ranges of all life stages and it is therefore of interest to population ecologists and managers alike to understand spatiotemporal distributions and possible niche differentiation between age‐classes. In albatrosses, particularly little is known about the juvenile life stage when fledglings depart the colonies and venture to sea with no prior experience or parental guidance. We compared the dispersal of 22 fledgling Black‐footed Albatross Phoebastria nigripes between 2006 and 2008 using satellite telemetry and 16 adults between 2008 and 2009 using geolocaters from Midway Atoll National Wildlife Refuge, Northwest Hawaiian Islands. Following tag deployment, all fledglings spent several days within the calm atoll waters, then travelled northward until reaching 750–900 km from the colony. At this point, fledgling distributions approached the productive North Pacific Transition Zone (NPTZ). Rather than reaching the high chlorophyll a densities on the leading edge of this zone, however, fledglings remained in areas of low productivity in the subtropical gyre. In contrast, adult albatrosses from the same breeding colony did not utilize the NPTZ at this time of year but rather ranged throughout the highly productive northern periphery of the Pacific Ocean Basin among the shelf regions off Japan and the Aleutian Islands. The dichotomy in habitat use between fledglings and adults from Midway Atoll results in complete spatial segregation between age‐classes and suggests ontogenetic niche separation in this species. This research fills a large knowledge gap in at‐sea habitat use during a little known yet critical life stage of albatrosses, and contributes to a more comprehensive understanding of differential mortality pressure between age‐classes and overall conservation status for the vulnerable Black‐footed Albatross. 相似文献
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Vita Rozman Tomaž Accetto Sylvia H. Duncan Harry J. Flint Maša Vodovnik 《Environmental microbiology》2021,23(3):1527-1540
Type IV pili (T4P) are bacterial surface-exposed appendages that have been extensively studied in Gram-negative pathogenic bacteria. Despite recent sequencing efforts, little is known regarding these structures in non-pathogenic anaerobic Gram-positive species, particularly commensals of the mammalian gut. Early studies revealed that T4P in two ruminal Gram-positive species are associated with growth on cellulose, suggesting possible associations of T4P with substrate utilization patterns. In the present study, genome sequences of 118 taxonomically diverse, mainly Gram-positive, bacterial strains isolated from anaerobic (gastrointestinal) environments, have been analysed. The genes likely to be associated with T4P biogenesis were analysed and grouped according to T4P genetic organization. In parallel, consortia of Carbohydrate Active enZYmes (CAZymes) were also analysed and used to predict carbohydrate utilization abilities of selected strains. The predictive power of this approach was additionally confirmed by experimental assessment of substrate-related growth patterns of selected strains. Our analysis revealed that T4P systems with diverse genetic organization are widespread among Gram-positive anaerobic non-pathogenic bacteria isolated from different environments, belonging to two phylogenetically distantly related phyla: Firmicutes and Actinobacteria. 相似文献
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Fischer-344 (F-344) rats differ from other common rat strains in that they
fail to show any preference for NaCl at any concentration in two- bottle
preference tests. Because 100 microM amiloride partially blocks the
NaCl-evoked chorda tympani (CT) response in electrophysiological studies,
we tested NaCl preference (0.068-0.273 M) in F-344 rats with and without
100 microM amiloride solution as the solvent. A third group was tested with
unadulterated NaCl solutions following CT transection. Amiloride had no
significant effect on the NaCl preference-aversion function, whereas CT
transection significantly reduced NaCl avoidance. These results suggest
that the amiloride-sensitive component of the NaCl response is not
necessary for F-344 rats to display avoidance of NaCl, but the entire CT
input is.
相似文献
59.
Cross-pathway control of ornithine carbamoyltransferase synthesis in Neurospora crassa 总被引:1,自引:0,他引:1
The pattern of cross-pathway regulation of the arginine synthetic enzyme ornithine carbamoyltransferase was investigated in Neurospora crassa, using single and double mutant auxotrophic strains starved for their required amino acids. These experiments show that starvation for histidine, tryptophan, isoleucine, valine or arginine can result in derepression of ornithine carbamoyltransferase. Methionine starvation also gave slight derepression, but starvation for lysine or leucine gave little or no effect. 相似文献
60.
Mary K. Yates Mithun R. Raje Payel Chatterjee Christina F. Spiropoulou Sina Bavari Mike Flint Veronica Soloveva Katherine L. Seley-Radtke 《Bioorganic & medicinal chemistry letters》2017,27(12):2800-2802
Fleximers, a novel type of flexible nucleoside that have garnered attention due to their unprecedented activity against human coronaviruses, have now exhibited highly promising levels of activity against filoviruses. The Flex-nucleoside was the most potent against recombinant Ebola virus in Huh7 cells with an EC50 = 2 μM, while the McGuigan prodrug was most active against Sudan virus-infected HeLa cells with an EC50 of 7 μM. 相似文献