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121.
122.
TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis   总被引:1,自引:0,他引:1  
Amyotrophic lateral sclerosis (ALS) is a fatal motor neuron disorder characterized pathologically by ubiquitinated TAR DNA binding protein (TDP-43) inclusions. The function of TDP-43 in the nervous system is uncertain, and a mechanistic role in neurodegeneration remains speculative. We identified neighboring mutations in a highly conserved region of TARDBP in sporadic and familial ALS cases. TARDBPM337V segregated with disease within one kindred and a genome-wide scan confirmed that linkage was restricted to chromosome 1p36, which contains the TARDBP locus. Mutant forms of TDP-43 fragmented in vitro more readily than wild type and, in vivo, caused neural apoptosis and developmental delay in the chick embryo. Our evidence suggests a pathophysiological link between TDP-43 and ALS.  相似文献   
123.
Double-chambered right ventricle (DCRV) is possibly an emerging congenital cardiac anomaly in dogs. The defect causes clinical and pathophysiologic signs similar to those of congenital pulmonic stenosis in dogs but has distinct diagnostic features, breed predilections, and implications for treatment. The defect is often associated with clinical signs early in life. Surgical correction of DCRV can be undertaken with the aid of cardiopulmonary bypass and offers the prospect of an improved clinical outcome.  相似文献   
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Fuel hazards have increased in forests across the United States because of fire exclusion during the 20th century. Treatments used to reduce fuel buildup may affect wildlife, such as shrews, living on the forest floor, especially when treatments are applied repeatedly. From mid-May to mid-August 2006 and 2007, we used drift fences with pitfall traps to capture shrews in western North Carolina in 3 fuel reduction treatment areas [(1) twice-burned (2003 and 2006), (2) mechanical understory cut (2002), and (3) mechanical understory cut (2002) followed by 2 burns (2003 and 2006)] and a control. We captured 77% fewer southeastern shrews (Sorex longirostris) in mechanical + twice-burned treatment areas than in mechanical treatment areas in 2006, but southeastern shrew captures did not differ among treatment areas in 2007. Total shrew captures did not differ among treatment areas in either year. Decreases in leaf litter, duff depth, and canopy cover in mechanical + twice-burned treatment areas may have decreased ground-level moisture, thereby causing short-term declines in southeastern shrew captures. Prescribed fire or mechanical fuel reduction treatments in the southern Appalachian Mountains did not greatly affect shrew populations, though the combination of both treatments may negatively affect some shrew species, at least temporarily.  相似文献   
126.
Alginate, a natural polysaccharide derived from brown seaweed, is finding multiple applications in biomedicine via its transformation through chemical, physical, and, increasingly, enzymatic processes. In this study a novel alginate lyase, AlyDS44, was purified and characterized from a marine actinobacterium, Streptomyces luridiscabiei, which was isolated from decomposing seaweed. The purified enzyme had a specific activity of 108.6 U/mg, with a molecular weight of 28.6 kDa, and was composed of 260 amino acid residues. AlyDS44 is a bifunctional alginate lyase, active on both polyguluronate and polymannuronate, though it preferentially degrades polyguluronate. The optimal pH of this enzyme is 8.5 and the optimal temperature is 45 °C. It is a salt-tolerant alginate lyase with an optimal activity at 0.6 M NaCl. Metal ions Mn2+, Co2+, and Fe2+ increased the alginate degrading activity, but it was inhibited in the presence of Zn2+ and Cu2+. The highly conserved regions of its amino acid sequences indicated that AlyDS44 belongs to the polysaccharide lyase family 7. The main breakdown products of the enzyme on alginate were disaccharides, trisaccharides, and tetrasaccharides, which demonstrated that this enzyme acted as an endo-type alginate lyase. AlyDS44 is a novel enzyme, with the potential for efficient production of alginate oligosaccharides with low degrees of polymerization.  相似文献   
127.
  1. Detecting rare species is often a necessity for conservation and management, yet challenging for many field survey methods. Environmental DNA (eDNA) is a highly promising solution that has been shown to outperform many established survey methods.
  2. Macquarie perch (Macquaria australasica) is an endangered native species that has declined significantly in range and abundance. Detection of M. australasica was compared with an abundant alien fish species (Oncorhynchus mykiss) using eDNA and three conventional survey methods: gill nets, electrofishing and fyke nets.
  3. eDNA occupancy estimates for both fish species were compared using four different models to investigate what effect these differences have on false positives and false negatives for the rare and common fish species. These models used unadjusted eDNA detections in water samples, eDNA detections that have been screened using a limit of detection method to remove potential false positives, eDNA data supplemented with a second survey method, or eDNA data augmented with sequencing of positive polymerase chain reaction replicates.
  4. eDNA surveying as a single detection method was found to be more efficient and sensitive compared with each capture method separately and combined. Occupancy estimates for the common and rare species did not vary significantly between the four site occupancy-detection models, suggesting that supplementary data may not have as much effect on occupancy estimates compared with other approaches such as temporal or spatial sampling.
  5. We conclude that eDNA outperforms the three established survey methods for both a rare and common freshwater fish species. Although there was no significant effect of augmenting eDNA survey methods with other survey data, additional data may improve confidence in detection, and provide confirmatory evidence for unexpected or new detections of a species.
  相似文献   
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There is an urgent need for primary data that can be used to quantify the value of marine aquaculture facilities that also describe influences on the surrounding natural ecosystem and its wild fish communities. Divers completed 360 transect replicates below a net‐cage and at nearby and distant rocky reefs off Catalina Island, California, estimating the species abundance and size class of all conspicuous fishes in the water column. We observed 10,234 fishes aggregating below the net‐cage with a mean annual density of 142 (SE ± 30) per 100 m2 and diversity H′ 2.29. At the adjacent reference reef, we counted 8452 fishes with a mean annual density of 117 (SE ± 20) and H′ 1.45 and at Reference Reef 2, located 500 m away, there were 8958 fishes with a density of 124 (SE ± 20) and H′ 1.13. The total density of fishes was the same between the study sites (ANOVA; P = 0.24); however, the spatial density of fishes in the water column was different between the net‐cage and reference reefs. Additionally, the density of juvenile fish was significantly lower at the net‐cage (ANOVA; P = 0.02), while the density of subadult and adult fishes was higher (ANOVA; P = 0.03). Feeding guild analysis ranked the net‐cage highest (3507), while Reference Reef 1 (2149), and Reference Reef 2 (2241), had lower values, a relationship often found between artificial and natural reefs. This suggests the importance of available trophic resources to the adult population of wild fishes, and has implications in the artificial reef attraction–production debate. The results of this study not only reproduce a pattern of fish attraction to net‐cages in the marine environment reported in previous studies but also demonstrate a relative habitat value compared in both time and space, of the fish community at a net‐cage compared with vibrant kelp bed communities.  相似文献   
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