Turbulent convection in protoplanetary discs and its role in angular momentum transfer

We present a model for the transport of anisotropic turbulence in an accretion disc. The model uses the Reynolds stress tensor approach in the mean field approximation. To study the role of convection in a protoplanetary disc, we combine the turbulence model with a radiative transfer calculation, and also include convection using the mixing length approximation. We find that the turbulence generated by convection causes the angular momentum of the accretion disc to be directed outwards. We also confirm the conclusions of other authors that turbulent convection is unable to provide the observed disc accretion rates as well as a heat source sufficient for the convection to be self-sustaining. The reasons for the latter are the strong anisotropy of the turbulence together with the low efficiency of the energy transfer from the background velocity shear to the turbulent stress tensor.Comment: MNRAS accepted | 15 pages, 8 figure

Pathomechanisms of a CLCN1 Mutation Found in a Russian Family Suffering From Becker's Myotonia

Objective: Myotonia congenita (MC) is a rare muscle disease characterized by sarcolemma over-excitability inducing skeletal muscle stiffness. It can be inherited either as an autosomal dominant (Thomsen's disease) or an autosomal recessive (Becker's disease) trait. Both types are caused by loss-of-function mutations in the CLCN1 gene, encoding for ClC-1 chloride channel. We found a ClC-1 mutation, p.G411C, identified in Russian patients who suffered from a severe form of Becker's disease. The purpose of this study was to provide a solid correlation between G411C dysfunction and clinical symptoms in the affected patient. Methods: We provide clinical and genetic information of the proband kindred. Functional studies include patch-clamp electrophysiology, biotinylation assay, western blot analysis, and confocal imaging of G411C and wild-type ClC-1 channels expressed in HEK293T cells. Results: The G411C mutation dramatically abolished chloride currents in transfected HEK cells. Biochemical experiments revealed that the majority of G411C mutant channels did not reach the plasma membrane but remained trapped in the cytoplasm. Treatment with the proteasome inhibitor MG132 reduced the degradation rate of G411C mutant channels, leading to their expression at the plasma membrane. However, despite an increase in cell surface expression, no significant chloride current was recorded in the G411C-transfected cell treated with MG132, suggesting that this mutation produces non-functional ClC-1 chloride channels. Conclusion: These results suggest that the molecular pathophysiology of G411C is linked to a reduced plasma membrane expression and biophysical dysfunction of mutant channels, likely due to a misfolding defect. Chloride current abolition confirms that the mutation is responsible for the clinical phenotype

The Mass Function of Dark Halos in Superclusters and Voids

A modification of the Press-Schechter theory allowing for presence of a background large-scale structure (LSS) - a supercluster or a void, is proposed. The LSS is accounted as the statistical constraints in form of linear functionals of the random overdensity field. The deviation of the background density within the LSS is interpreted in a pseudo-cosmological sense. Using the constraints formalism may help us to probe non-trivial spatial statistics of haloes, e.g. edge and shape effects on boundaries of the superclusters and voids. Parameters of the constraints are connected to features of the LSS: its mean overdensity, a spatial scale and a shape, and spatial momenta of higher orders. It is shown that presence of a non-virialized LSS can lead to an observable deviation of the mass function. This effect is exploited to build a procedure to recover parameters of the background perturbation from the observationally estimated mass function.Comment: 23 pages, 6 figures; to be appeared in Astronomy Reports, 2014, Vol. 58, No. 6, pp. 386-39