976 research outputs found

    How well does Miscanthus ensile for use in an anaerobic digestion plant?

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    This study examined the ability for early-harvested Miscanthus (Miscanthus x giganteus and Miscanthus sacchariflorus) to be stored in silage for later use in anaerobic digestion. Two silage additives favouring a homo and hetero-fermentation pathway were examined. The results show that silage additives are necessary to effectively ensile Miscanthus, otherwise untreated Miscanthus grasses incurred dry matter losses of 4% during three months' storage. The silage additives improved the lactic and acetic acid production in the Miscanthus silages however did not have any effect on the biogas yield. On a ‘per tonne volatile solids’-basis, Miscanthus produces half the biogas yield of maize. The outlook for the use of Miscanthus AD therefore depends on the yield when harvested in autumn. A minimum yield of 19–26.5 t DM/ha is needed for Miscanthus to match the biogas production from a similar area of maize yielding 10–14 t DM/ha

    Hyperinsulinism-hyperammonaemia syndrome: novel mutations in the GLUD1 gene and genotype-phenotype correlations

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    Background: Activating mutations in the GLUD1 gene (which encodes for the intra-mitochondrial enzyme glutamate dehydrogenase, GDH) cause the hyperinsulinism–hyperammonaemia (HI/HA) syndrome. Patients present with HA and leucine-sensitive hypoglycaemia. GDH is regulated by another intra-mitochondrial enzyme sirtuin 4 (SIRT4). Sirt4 knockout mice demonstrate activation of GDH with increased amino acid-stimulated insulin secretion. Objectives: To study the genotype–phenotype correlations in patients with GLUD1 mutations. To report the phenotype and functional analysis of a novel mutation (P436L) in the GLUD1 gene associated with the absence of HA. Patients and methods: Twenty patients with HI from 16 families had mutational analysis of the GLUD1 gene in view of HA (n=19) or leucine sensitivity (n=1). Patients negative for a GLUD1 mutation had sequence analysis of the SIRT4 gene. Functional analysis of the novel P436L GLUD1 mutation was performed. Results: Heterozygous missense mutations were detected in 15 patients with HI/HA, 2 of which are novel (N410D and D451V). In addition, a patient with a normal serum ammonia concentration (21 µmol/l) was heterozygous for a novel missense mutation P436L. Functional analysis of this mutation confirms that it is associated with a loss of GTP inhibition. Seizure disorder was common (43%) in our cohort of patients with a GLUD1 mutation. No mutations in the SIRT4 gene were identified. Conclusion: Patients with HI due to mutations in the GLUD1 gene may have normal serum ammonia concentrations. Hence, GLUD1 mutational analysis may be indicated in patients with leucine sensitivity; even in the absence of HA. A high frequency of epilepsy (43%) was observed in our patients with GLUD1 mutations

    So That the People May Live (Hecel Lena Oyate Ki Nipi Kte): Lakota and Dakota Elder Women as Reservoirs of Life and Keepers of Knowledge about Health Protection and Diabetes Prevention

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    Around the world, Type 2 diabetes is on the rise, affecting adults and youth from societies in the throes of industrialization. Over time, uncontrolled diabetes can leave in its wake people facing renal failure, blindness, and heart disease, and communities daunted by new, chaotic phenomena. Westernized lifestyles are a recognized explanation for the escalating prevalence. The web of causation, however, may be broader and thicker, woven by complex interactions with environmental, sociological, and historical roots. The purpose of this participatory ethnographic study was to document, understand, and support Lakota and Dakota elder women’s beliefs and knowledge about health protection and diabetes prevention. In-depth interviews were conducted with nine elder women to learn: (1) about the factors attributable to diabetes, (2) about related narratives addressing health protection and diabetes prevention, and (3) how knowledge about health protection is shared. The elders saw diabetes as an outside, unnatural disorder, the contributing influences of which are external as well as internal. They offered narratives about chaos, restitution, testimony, and quests for cures and meaning. The elders connected health to traditional values and ways, the land, and memory. Reservoirs of wisdom reside in the knowledge systems of tribal elders who remember when diabetes was unknown. Health leaders at local and national levels would be wise to respect and draw upon this knowledge for guidance in program planning and policy development

    Diazoxide-responsive hyperinsulinemic hypoglycemia caused by HNF4A gene mutations

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    Objective: The phenotype associated with heterozygous HNF4A gene mutations has recently been extended to include diazoxide responsive neonatal hypoglycemia in addition to maturity-onset diabetes of the young (MODY). To date, mutation screening has been limited to patients with a family history consistent with MODY. In this study, we investigated the prevalence of HNF4A mutations in a large cohort of patients with diazoxide responsive hyperinsulinemic hypoglycemia (HH). Subjects and methods: We sequenced the ABCC8, KCNJ11, GCK, GLUD1, and/or HNF4A genes in 220 patients with HH responsive to diazoxide. The order of genetic testing was dependent upon the clinical phenotype. Results: A genetic diagnosis was possible for 59/220 (27%) patients. KATP channel mutations were most common (15%) followed by GLUD1 mutations causing hyperinsulinism with hyperammonemia (5.9%), and HNF4A mutations (5%). Seven of the 11 probands with a heterozygous HNF4A mutation did not have a parent affected with diabetes, and four de novo mutations were confirmed. These patients were diagnosed with HI within the first week of life (median age 1 day), and they had increased birth weight (median +2.4 SDS). The duration of diazoxide treatment ranged from 3 months to ongoing at 8 years. Conclusions: In this large series, HNF4A mutations are the third most common cause of diazoxide responsive HH. We recommend that HNF4A sequencing is considered in all patients with diazoxide responsive HH diagnosed in the first week of life irrespective of a family history of diabetes, once KATP channel mutations have been excluded

    The Optical Alignment System of the ZEUS MicroVertex Detector

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    The laser alignment system of the ZEUS microvertex detector is described. The detector was installed in 2001 as part of an upgrade programme in preparation for the second phase of electron-proton physics at the HERA collider. The alignment system monitors the position of the vertex detector support structure with respect to the central tracking detector using semi-transparent amorphous-silicon sensors and diode lasers. The system is fully integrated into the general environmental monitoring of the ZEUS detector and data has been collected over a period of 5 years. The primary aim of defining periods of stability for track-based alignment has been achieved and the system is able to measure movements of the support structure to a precision around 10μ10 \mum.Comment: 38 pages; 17 figure
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