Numerical study of multilayer adsorption on fractal surfaces

We report a numerical study of van der Waals adsoprtion and capillary condensation effects on self-similar fractal surfaces. An assembly of uncoupled spherical pores with a power-law distributin of radii is used to model fractal surfaces with adjustable dimensions. We find that the commonly used fractal Frankel-Halsey-Hill equation systematically fails to give the correct dimension due to crossover effects, consistent with the findings of recent experiments. The effects of pore coupling and curvature dependent surface tension were also studied.Comment: 11 pages, 3 figure

Mutations in the colony stimulating factor 1 receptor (CSF1R) gene cause hereditary diffuse leukoencephalopathy with spheroids.

Hereditary diffuse leukoencephalopathy with spheroids (HDLS) is an autosomal-dominant central nervous system white-matter disease with variable clinical presentations, including personality and behavioral changes, dementia, depression, parkinsonism, seizures and other phenotypes. We combined genome-wide linkage analysis with exome sequencing and identified 14 different mutations affecting the tyrosine kinase domain of the colony stimulating factor 1 receptor (encoded by CSF1R) in 14 families with HDLS. In one kindred, we confirmed the de novo occurrence of the mutation. Follow-up sequencing identified an additional CSF1R mutation in an individual diagnosed with corticobasal syndrome. In vitro, CSF-1 stimulation resulted in rapid autophosphorylation of selected tyrosine residues in the kinase domain of wild-type but not mutant CSF1R, suggesting that HDLS may result from partial loss of CSF1R function. As CSF1R is a crucial mediator of microglial proliferation and differentiation in the brain, our findings suggest an important role for microglial dysfunction in HDLS pathogenesis