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Background: Spinal muscular atrophy (SMA) is a children’s neuromuscular disorder. Although motor neuron loss is a major feature of the disease, we have identified fatty acid abnormalities in SMA patients and in preclinical animal models, suggesting metabolic perturbation is also an important component of SMA. Methods: Biochemical, histological, proteomic, and high resolution respirometry were used. Results: SMA patients are more susceptible to dyslipidemia than the average population as determined by a standard lipid profile in a cohort of 72 pediatric patients. As well, we observed a non-alcoholic liver disease phenotype in apreclinical mouse model. Denervation alone was not sufficient to induce liver steatosis, as a mouse model of ALS, did not develop fatty liver. Hyperglucagonemia in Smn2B/-mice could explain the hepatic steatosis by increasing plasma substrate availability via glycogen depletion and peripheral lipolysis. Proteomic analysis identified mitochondrion and lipid metabolism as major clusters. Alterations in mitochondrial function were revealed by high-resolution respirometry. Finally, low-fat diets led to increased survival in Smn2B/-mice. Conclusions: These results provide strong evidence for lipid metabolism defects in SMA. Further investigation will be required to establish the primary mechanism of these alterations and understand how they lead to additional co-morbidities in SMA patients.
The objective of the current experiment was to determine the effects of high-concentration phytase (5000 FTU/kg) feeding to diverse lines of chickens fed phosphorus (P) adequate maize–soybean meal diets (4.5 g/kg non-phytate P) on the performance and intestinal immune function. Performance was measured for outbred broiler (Ross 308) and inbred Fayoumi lines over 0–21 days, and duodenum and ileum were harvested for the determination of mucin-2, interleukin (IL)-1β and IgA mRNA by quantitative reverse transcription polymerase chain reaction. Over the 0–7-day period, there was a significant line × diet interaction, as high phytase supplementation increased broiler average daily gain (ADG), but had no effect on Fayoumi ADG. Treatment of diets with phytase increased expression of the mucin-2 gene in the duodenum mucosa. There were significant interactions between line and age, and line, diet and age on duodenal expression of the IL-1β gene as phytase supplementation of the broiler line reduced IL-1β in comparison to control fed broilers without change in the Fayoumi line. Overall, the addition of a high concentration of phytase to broilers fed adequate concentrations of non-phytate P resulted in improved growth performance early with a reduction in this effect over time. Mucosal mucin-2 expression was increased with high-concentration phytase feeding across both lines, but IL-1β mRNA expression was reduced in the duodenum of broilers fed high concentrations of phytase, suggesting that the increased performance noted might be related to decreased inflammation.
In today’s global climate of consumer preferences or legislation for reduced antibiotic use in animal production, genetic approaches to health provide a natural and sustainable method to help ensure the wholesomeness of the food supply. The tools of molecular genetics have recently enabled the search for molecular genetic markers for health (Lamont, 1994, 1998). Although there are a few notable examples of health traits controlled by single genes, the vast majority of health traits are controlled by multiple genes.
To examine whether weight history and weight transitions over adult lifespan contribute to physical impairment among postmenopausal women.
BMI categories were calculated among postmenopausal women who reported their weight and height at age 18 years. Multiple-variable logistic regression was used to determine the association between BMI at age 18 years and BMI transitions over adulthood on severe physical impairment (SPI), defined as scoring <60 on the Physical Functioning subscale of the Rand thirty-six-item Short-Form Health Survey.
Participants were part of the Women’s Health Initiative Observational Study (WHI OS), where participants’ health was followed over time via questionnaires and clinical assessments.
Postmenopausal women (n 76 016; mean age 63·5 (sd 7·3) years).
Women with overweight (BMI=25·0–29·9 kg/m2) or obesity (BMI≥30·0 kg/m2) at 18 years had greater odds (OR (95 % CI)) of SPI (1·51 (1·35, 1·69) and 2·14 (1·72, 2·65), respectively) than normal-weight (BMI=18·5–24·9 kg/m2) counterparts. Transitions from normal weight to overweight/obese or to underweight (BMI<18·5 kg/m2) were associated with greater odds of SPI (1·97 (1·84, 2·11) and 1·35 (1·06, 1·71), respectively) compared with weight stability. Shifting from underweight to overweight/obese also had increased odds of SPI (1·52 (1·11, 2·09)). Overweight/obese to normal BMI transitions resulted in a reduced SPI odds (0·52 (0·39, 0·71)).
Higher weight history and transitions into higher weight classes were associated with higher likelihood of SPI, while transitioning into lower weight classes for those with overweight/obesity was protective among postmenopausal women.
We present a systematic study of the low-temperature photoluminescence from undoped GaAs layers grown directly on Si substrates by MOCVD. GaAs layers from 100Å to 4 μm in thickness were deposited on Si substrates prepared with a variety of doping levels and orientations. The emission from thicker samples is dominated by pairs of lines in the band-edge region. Photoluminescence excitation measurements show that this multiplicity results from two regions in the material with different levels of strain. The stress-induced splitting of the valence band is also studied using excitation spectroscopy. In thinner samples we observe strong emission in the midgap range due to stoichiometric defects. The nature of the defects near the interface depends strongly on the character of the substrate.
Sulfide passivation of optically pumped GaAs/AlGaAs microdisks (at 77K) was examined. The microdisks had diameters between 2 and 10 µm and thicknesses of 0.2 µm. The layer structure was grown by organometallic vapor phase epitaxy. An ammonium sulfide solution was used for the passivation. The GaAs/AlGaAs microdisks without the sulfide treatment did not lase when optically pumped, even at the highest pump power densities of 10 kW/cm2. With sulfide treatment cw lasing was achieved with a lifetime of several seconds. A dramatic increase in the laser lifetime was obtained when the sulfide treated samples were encapsulated with silicon nitride in order to prevent loss of sulfur and surface oxidation. In addition to the lifetime increase, the laser output could be increased by nearly an order of magnitude by annealing at 400°C for 300 s or by cw operation over a period of hours at pumping powers near 10 kW/cm2.
Serum samples collected from swine and cattle in Great Britain at various times between July 1971 and July 1977 were examined by haemagglutination-inhibition or single radial haemolysis methods for evidence of infection with influenza A (H3N2) viruses. A small proportion of swine sera collected in each year reacted in the tests but there was no evidence of infection in cattle. The significance of the findings is discussed, with particular reference to the seasonal fluctuations in the prevalence of antibody in swine observed during the period of the study, and their possible relevance to influenzal events in the human population. None of the sera tested had antibody to swine influenza strains (HSw1N1).