Dysregulation of Gene Expression in a Lysosomal Storage Disease Varies between Brain Regions Implicating Unexpected Mechanisms of Neuropathology

The characteristic neurological feature of many neurogenetic diseases is intellectual disability. Although specific neuropathological features have been described, the mechanisms by which specific gene defects lead to cognitive impairment remain obscure. To gain insight into abnormal functions occurring secondary to a single gene defect, whole transcriptome analysis was used to identify molecular and cellular pathways that are dysregulated in the brain in a mouse model of a lysosomal storage disorder (LSD) (mucopolysaccharidosis [MPS] VII). We assayed multiple anatomical regions separately, in a large cohort of normal and diseased mice, which greatly increased the number of significant changes that could be detected compared to past studies in LSD models. We found that patterns of aberrant gene expression and involvement of multiple molecular and cellular systems varied significantly between brain regions. A number of changes revealed unexpected system and process alterations, such as up-regulation of the immune system with few inflammatory changes (a significant difference from the closely related MPS IIIb model), down-regulation of major oligodendrocyte genes even though white matter changes are not a feature histopathologically, and a plethora of developmental gene changes. The involvement of multiple neural systems indicates that the mechanisms of neuropathology in this type of disease are much broader than previously appreciated. In addition, the variation in gene dysregulation between brain regions indicates that different neuropathologic mechanisms may predominate within different regions of a diseased brain caused by a single gene mutation

Trends and connections across the Antarctic cryosphere

Satellite observations have transformed our understanding of the Antarctic cryosphere. The continent holds the vast majority of Earth’s fresh water, and blankets swathes of the Southern Hemisphere in ice. Reductions in the thickness and extent of floating ice shelves have disturbed inland ice, triggering retreat, acceleration and drawdown of marine-terminating glaciers. The waxing and waning of Antarctic sea ice is one of Earth’s greatest seasonal habitat changes, and although the maximum extent of the sea ice has increased modestly since the 1970s, inter-annual variability is high, and there is evidence of longer-term decline in its extent

Effects of different dietary vitamin combinations on the egg quality and vitamin deposition in the whole egg of laying hens

The experiment was conducted to evaluate the effects of different dietary vitamin combinations on the egg quality and vitamin concentrations in the eggs of commercial laying hens. A total of 1,800 25-week-old Lohman pink-shell hens were randomly assigned to four dietary vitamin treatments as follows: NRC(1994) level, NRC (1994) level with Hy.D® (25-hydroxy-cholecalciferol), Local level (current average industry level in China) and OVN® level (optimum vitamin nutrition level), with 10 replicates per treatment and 45 layers per replicate. Hens were housed in commercial laying cages with three birds per cage and given ad libitum access to feed. Results showed the hens that received the fortified vitamin levels in the OVN® treatment had a significantly (p<0.05) lower number of cracked (.47%) and dirty eggs (.27%), and increased egg deposition of vitamin B12, folic acid, vitamin A, vitamin D, 25-OH-D3, vitamin E, vitamin B1, biotin and pantothenate (p<0.05). Treatments had no significant effect on egg-shape index, egg specific gravity, Haugh units and eggshell thickness. Hens fed the NRC-Hy.D® combination also experienced a significant decrease in cracked and dirty eggs (.70% and .44%, respectively) and an increased deposition of 25-OH-D3 in comparison with the NRC treatment. Results of the present study suggest that that the Local treatment was able to improve egg quality parameters of laying hens, but resulted in more cracked and dirty eggs. OVN® reduced the number of cracked eggs and dirty eggs, and improved the deposition of several vitamins in eggs. With the addition of Hy.D®, eggshell strength and 25-OH-D3 deposition in eggs were also improved, and cracked and dirty egg rates declined