WtF-Nano : One-Pot Dewatering and Water-Free Topochemical Modification of Nanocellulose in Ionic Liquids or gamma-Valerolactone

Ionic liquids are used to dewater a suspension of birch Kraft pulp cellulose nanofibrils (CNF) and as a medium for water-free topochemical modification of the nanocellulose (a process denoted as "WtF-Nano"). Acetylation was applied as a model reaction to investigate the degree of modification and scope of effective ionic liquid structures. Little difference in reactivity was observed when water was removed, after introduction of an ionic liquid or molecular co-solvent. However, the viscoelastic properties of the CNF suspended in two ionic liquids show that the more basic, but non-dissolving ionic liquid, allows for better solvation of the CNF. Vibrio fischeri bacterial tests show that all ionic liquids in this study were harmless. Scanning electron microscopy and wide-angle X-ray scattering on regenerated samples show that the acetylated CNF is still in a fibrillar form. 1D and 2D NMR analyses, after direct dissolution in a novel ionic liquid electrolyte solution, indicate that both cellulose and residual xylan on the surface of the nanofibrils reacts to give acetate esters.Peer reviewe

Autosomal recessive cerebellar ataxias

Autosomal recessive cerebellar ataxias (ARCA) are a heterogeneous group of rare neurological disorders involving both central and peripheral nervous system, and in some case other systems and organs, and characterized by degeneration or abnormal development of cerebellum and spinal cord, autosomal recessive inheritance and, in most cases, early onset occurring before the age of 20 years. This group encompasses a large number of rare diseases, the most frequent in Caucasian population being Friedreich ataxia (estimated prevalence 2–4/100,000), ataxia-telangiectasia (1–2.5/100,000) and early onset cerebellar ataxia with retained tendon reflexes (1/100,000). Other forms ARCA are much less common. Based on clinicogenetic criteria, five main types ARCA can be distinguished: congenital ataxias (developmental disorder), ataxias associated with metabolic disorders, ataxias with a DNA repair defect, degenerative ataxias, and ataxia associated with other features. These diseases are due to mutations in specific genes, some of which have been identified, such as frataxin in Friedreich ataxia, α-tocopherol transfer protein in ataxia with vitamin E deficiency (AVED), aprataxin in ataxia with oculomotor apraxia (AOA1), and senataxin in ataxia with oculomotor apraxia (AOA2). Clinical diagnosis is confirmed by ancillary tests such as neuroimaging (magnetic resonance imaging, scanning), electrophysiological examination, and mutation analysis when the causative gene is identified. Correct clinical and genetic diagnosis is important for appropriate genetic counseling and prognosis and, in some instances, pharmacological treatment. Due to autosomal recessive inheritance, previous familial history of affected individuals is unlikely. For most ARCA there is no specific drug treatment except for coenzyme Q10 deficiency and abetalipoproteinemia

Plasma rotation and momentum transport studies at JET

An experimental study in plasma rotation and momentum transport was carried out at JET. The toroidal rotation profile was found to scale approximately with that of the ion temperature. However, significant deviations from this were found in high density ELMy H-mode discharges, which had broader rotation profiles. A rotation database analysis showed the variation of the dimensionless Mach number with respect to the plasma scenario. For predominantly NBI heated discharges the Mach number was found to be in the range of 0.3-0.45. Larger Mach numbers were observed in type I ELMy H-modes, while scenarios exhibiting type III ELMs or L-mode had lower Mach numbers. Advanced scenarios often showed a significant increase in the central Mach number when an internal transport barrier formed. A detailed study was done to investigate the Prandtl number, P-r, defined as the ratio between momentum and ion heat diffusivity. Generally the Prandtl number was found to be significantly below unity, e. g. 0.18 < P-r < 0.35. Although it is often predicted that momentum and heat diffusivity are equal in ITG dominated plasma, also for high density ELMy H-mode discharges with temperature profiles close and above the ITG threshold, the Prandtl number was P-r similar to 0.3

Neuronal Abelson helper integration site-1 (Ahi1) deficiency in mice alters TrkB signaling with a depressive phenotype

Recent studies suggest that the human Abelson helper integration site-1 (AHI1) gene on chromosome 6 is associated with susceptibility to schizophrenia and autism, two common neuropsychological disorders with depression symptoms. Mouse Ahi1 protein is abundant in the hypothalamus and amygdala, which are important brain regions for controlling emotion. However, the neuronal function of Ahi1 remains unclear. With the Cre–loxP system, we created a mouse model that selectively reduces Ahi1 expression in neuronal cells. Mice with neuronal Ahi1 deficiency show reduced TrkB level in the brain and depressive phenotypes, which can be alleviated by antidepressant drugs or by overexpression of TrkB in the amygdala. Ahi1 deficiency promotes the degradation of endocytic TrkB and reduces TrkB signaling in neuronal cells. Our findings suggest that impaired endocytic sorting and increased degradation of TrkB can induce depression and that this impaired pathway may serve as a previously uncharacterized therapeutic target for depression

Marfan Database (third edition): new mutations and new routines for the software

The Marfan database is a software that contains routines for the analysis of mutations identified in the FBN1 gene that encodes fibrillin-1. Mutations in this gene are associated not only with Marfan syndrome but also with a spectrum of overlapping disorders. the third version of the Marfan database contains 137 entries. the software has been modified to accommodate four new routines and is now accessible on the World Wide Web at http://www.umd.necker.fr.Univ Paris 05, Hop Necker Enfants Malad, Clin Maurice Lamy, INSERM,U383, F-75743 Paris 15, FranceCHU Cochin Port Royal, Inst Cochin Genet Mol, F-75014 Paris, FranceHop Broussais, F-75014 Paris, FranceRoyal Alexandra Hosp Children New, Dept Med Genet, Sydney, NSW 2124, AustraliaRoyal Alexandra Hosp Children New, Dept Paediat & Child Hlth, Sydney, NSW 2124, AustraliaUniv Dundee, Ninewells Hosp & Med Sch, Dept Mol & Cellular Pathol, Mol Genet Lab, Dundee DD1 9SY, ScotlandInst Child Hlth, Unit Clin Genet & Fetal Med, N Thames E Clin Mol Genet Lab, London, EnglandUniv Edinburgh, Western Gen Hosp, Mol Med Ctr, Dept Med,Human Genet Unit, Edinburgh EH4 2XU, Midlothian, ScotlandSt George Hosp, Sch Med, London SW17 0RE, EnglandStanford Univ, Med Ctr, Howard Hughes Med Inst, Stanford, CA 94305 USAUniv Munich, Klinikm GroBhadern, Dept Cardiovasc Surg, D-81366 Munich, GermanyUniv Munich, Klinikm GroBhadern, Inst Clin Chem, D-81366 Munich, GermanyState Univ Ghent Hosp, Ctr Med Genet, B-9000 Ghent, BelgiumNatl Publ Hlth Inst, Dept Human Mol Genet, FIN-00300 Helsinki, FinlandUNIFESP, EPM, BR-04101000 São Paulo, BrazilHop Ambroise Pare, Lab Cent Biochim Hormonol & Genet Mol, F-92104 Boulogne, FranceStanford Univ, Med Ctr, Dept Genet, Stanford, CA 94305 USAUNIFESP, EPM, BR-04101000 São Paulo, BrazilWeb of Scienc