SPG10 is a rare cause of spastic paraplegia in European families

Background: SPG10 is an autosomal dominant form of hereditary spastic paraplegia (HSP), which is caused by mutations in the neural kinesin heavy chain KIF5A gene, the neuronal motor of fast anterograde axonal transport. Only four mutations have been identified to date.Objective: To determine the frequency of SPG10 in European families with HSP and to specify the SPG10 phenotype.Patients and methods: 80 index patients from families with autosomal dominant HSP were investigated for SPG10 mutations by direct sequencing of the KIF5A motor domain. Additionally, the whole gene was sequenced in 20 of these families.Results: Three novel KIF5A mutations were detected in German families, including one missense mutation (c.759G>T, p.K253N), one in frame deletion (c.768_770delCAA, p.N256del) and one splice site mutation (c.217G>A). Onset of gait disturbance varied from infancy to 30 years of age. All patients presented clinically with pure HSP, but a subclinical sensory--motor neuropathy was detected by neurophysiology studies.Conclusions: SPG10 accounts for approximately 3% of European autosomal dominant HSP families. All mutations affect the motor domain of kinesin and thus most likely impair axonal transport. Clinically, SPG10 is characterised by spastic paraplegia with mostly subclinical peripheral neuropathy

Muscle glycogen recovery after exercise measured by13C-magnetic resonance spectroscopy in humans: effect of nutritional solutions

The rate of glycogen resynthesis in human skeletal muscle after glycogen-depleting exercise is known to depend on carbohydrate intake and is reported to reach a platean after an adequate amount of carbohydrate (CHO) consumption. Efforts to maximize the rate of glycogen storage by changing the type and form of CHO, as well as by adding proteins or lipids have yielded inconsistent results. The objective of this study was to assess whether isocaloric addition of proteins and arginine to a CHO diet in the first 4 h after an endurance exercise would increase the rate of glycogen synthesis. The CHO solution, given twice at a 2 h interval according to earlier optimized protocols, contained 1.7 g CHO kgbody weight. The effects of this solution were compared to those of an isocaloric solution containing 1.2 g CHO/kgbody weight plus 0.5 g protein/kgbody weight (including 5 g arginine). Glycogen was measured in quadriceps muscle in vivo with natural abundance13C-magnetic resonance spectroscopy before exercise and twice after exercise, before and at the end of a 4-h period following the intake of one of the solutions. Eight subjects took part in a randomized cross-over trial separated by at least 1 week. Glycogen synthesis was found to be significantly increased with both regimes compared to a zero-caloric placebo diet, but no significant difference in glycogen resynthesis was found between the CHO-only diet and the one supplemented by proteins and arginine. It is estimated that significance would have been reached for an increase of 34%, while the effectively measured synthesis rates only differed by 5

Optimizing passive acoustic sampling of bats in forests

Passive acoustic methods are increasingly used in biodiversity research and monitoring programs because they are cost-effective and permit the collection of large datasets. However, the accuracy of the results depends on the bioacoustic characteristics of the focal taxa and their habitat use. In particular, this applies to bats which exhibit distinct activity patterns in three-dimensionally structured habitats such as forests. We assessed the performance of 21 acoustic sampling schemes with three temporal sampling patterns and seven sampling designs. Acoustic sampling was performed in 32 forest plots, each containing three microhabitats: forest ground, canopy, and forest gap. We compared bat activity, species richness, and sampling effort using species accumulation curves fitted with the clench equation. In addition, we estimated the sampling costs to undertake the best sampling schemes. We recorded a total of 145,433 echolocation call sequences of 16 bat species. Our results indicated that to generate the best outcome, it was necessary to sample all three microhabitats of a given forest location simultaneously throughout the entire night. Sampling only the forest gaps and the forest ground simultaneously was the second best choice and proved to be a viable alternative when the number of available detectors is limited. When assessing bat species richness at the 1-km(2) scale, the implementation of these sampling schemes at three to four forest locations yielded highest labor cost-benefit ratios but increasing equipment costs. Our study illustrates that multiple passive acoustic sampling schemes require testing based on the target taxa and habitat complexity and should be performed with reference to cost-benefit ratios. Choosing a standardized and replicated sampling scheme is particularly important to optimize the level of precision in inventories, especially when rare or elusive species are expected

Sex differences in age-related decline of urinary insulin-like growth factor-binding protein-3 levels in adult bonobos and chimpanzees

There is increasing interest in the characterization of normative senescence in humans. To assess to what extent aging patterns in humans are unique, comparative data from closely-related species, such as non-human primates, can be very useful. Here we use data from bonobos and chimpanzees, two closely-related species that share a common ancestor with humans, to explore physiological markers that are indicative of aging processes. Many studies on aging in humans focus on the somatotropic axis, consisting of growth hormone (GH), insulin-like growth factors (IGFs), and IGF binding proteins (IGFBPs). In humans, IGFBP-3 levels decline steadily with increasing age. We used urinary IGFBP-3 levels as an alternative endocrine marker for IGF-I, to identify the temporal pattern known to be related with age-related changes in cell proliferation, growth, and apoptosis. We measured urinary IGFBP-3 levels in samples from 71 bonobos and 102 chimpanzees. Focusing on samples from individuals aged ten years or older we found that urinary IGFBP-3 levels decline in both ape species with increasing age. However, in both species, females start with higher urinary IGFBP-3 levels than males, experience a steeper decline with increasing age, and converge with male levels around the age of 30 to 35 years. Our measurements of urinary IGFBP-3 levels indicate that bonobos and chimpanzees mirror human patterns of age-related decline in IGFBP-3 in older individuals (< 10 years) of both sexes. Moreover, like humans, both ape species show sex-specific differences in IGFBP-3 levels with females having higher levels than males, a result that correlates with sex-differences in life expectancy. Using changes in urinary IGFBP-3 levels as a proxy for changes in GH and IGF-I levels that mark age-related changes in cell proliferation, this approach provides an opportunity to investigate trade-offs in life history strategies in cross-sectional and in longitudinal studies, both in captivity and in the wild

Environmental effects of James River sewage treatment plant outfall construction

In the spring of 1975 the Institute began a program to determine whether significant environmental changes would occur in the area of the new James River Plant outfall that might be related to its construction and/or initial operation. Parameters measured in the study were benthic animal and oyster populations, coliform levels and chlorine residuals. The primary emphasis of the study centered on the estimation of the impact of the construction activity on shellfish beds in the area. The results of the investigation are presented in three segments, the first dealing with shellfish populations, the second with other benthic animals and the third with coliforms and chlorine. Section I. Environmental Effects of James River Sewage Treatment Plant Outfall Construction on Oyster Beds in the James River by Dexter S. Haven and Paul C . Kendall Section II. Environmental Effects of the James River Sewage Treatment Plant Outfall Construction: Soft Bottom Macrobenthos by Robert J. Diaz and Donald F. Boesch Section III. Water Quality in the Vicinity of James River Sewage Treatment Plant Outfall by Michael E. Bende

Additive opportunistic capture explains group hunting benefits in African wild dogs

African wild dogs (Lycaon pictus) are described as highly collaborative endurance pursuit hunters based on observations derived primarily from the grass plains of East Africa. However, the remaining population of this endangered species mainly occupies mixed woodland savannah where hunting strategies appear to differ from those previously described. We used high-resolution GPS and inertial technology to record fine-scale movement of all members of a single pack of six adult African wild dogs in northern Botswana. The dogs used multiple short-distance hunting attempts with a low individual kill rate (15.5%), but high group feeding rate due to the sharing of prey. Use of high-level cooperative chase strategies (coordination and collaboration) was not recorded. In the mixed woodland habitats typical of their current range, simultaneous, opportunistic, short-distance chasing by dogs pursuing multiple prey (rather than long collaborative pursuits of single prey by multiple individuals) could be the key to their relative success in these habitats

Antibiofilm Efficacies of Flavonoid-Rich Sweet Orange Waste Extract against Dual-Species Biofilms

The current study evaluated the antibacterial properties of industrial sweet orange waste extracts (ISOWEs), which are a rich source of flavonoids. The ISOWEs exhibited antibacterial activity towards the dental cariogenic pathogens Streptococcus mutans and Lactobacillus casei with 13.0 ± 2.0 and 20.0 ± 2.0 mg/mL for MIC (minimum inhibitory concentration) and 37.7 ± 1.5 and 43.3 ± 2.1 mg/mL for MBC (minimum bactericidal concentration), respectively. When evaluated in a 7-day dual-species oral biofilm model, ISOWEs dose-dependently reduced the viable bacteria count, and demonstrated strong synergistic effects when combined with the anti-septic chlorhexidine (at 0.1 and 0.2%). Similarly, confocal microscopy confirmed the anti-cariogenic properties of ISOWEs, alone and in combination with chlorhexidine. The citrus flavonoids contributed differently to these effects, with the flavones (nobiletin, tangeretin and sinensetin) demonstrating significantly lower MICs and MBCs compared to the flavanones hesperidin and narirutin. In conclusion, our study demonstrated the potential of citrus waste as a currently underutilised source of flavonoids for antimicrobial applications, such as in dental health

Resonant steps and spatiotemporal dynamics in the damped dc-driven Frenkel-Kontorova chain

Kink dynamics of the damped Frenkel-Kontorova (discrete sine-Gordon) chain driven by a constant external force are investigated. Resonant steplike transitions of the average velocity occur due to the competitions between the moving kinks and their radiated phasonlike modes. A mean-field consideration is introduced to give a precise prediction of the resonant steps. Slip-stick motion and spatiotemporal dynamics on those resonant steps are discussed. Our results can be applied to studies of the fluxon dynamics of 1D Josephson-junction arrays and ladders, dislocations, tribology and other fields.Comment: 20 Plain Latex pages, 10 Eps figures, to appear in Phys. Rev.

Interplay of disorder and nonlinearity in Klein-Gordon models: Immobile kinks

We consider Klein-Gordon models with a $\delta$-correlated spatial disorder. We show that the properties of immobile kinks exhibit strong dependence on the assumptions as to their statistical distribution over the minima of the effective random potential. Namely, there exists a crossover from monotonically increasing (when a kink occupies the deepest potential well) to the non-monotonic (at equiprobable distribution of kinks over the potential minima) dependence of the average kink width as a function of the disorder intensity. We show also that the same crossover may take place with changing size of the system.Comment: 7 pages, 4 figure