Anomalous Scleral Insertion of Superior Oblique in Axenfeld-Rieger Syndrome

Axenfeld-Rieger syndrome (ARS) is associated with ocular and systemic anomalies. PITX2 is known to be a major controlling gene in the pathogenesis of ARS and is associated with differentiation in both the neural crest and mesoderm during eye development

Neurotoxicity Screening in a Multipotent Neural Stem Cell Line Established from the Mouse Brain

Neural stem cells (NSCs) have mainly been applied to neurodegeneration in some medically intractable neurologic diseases. In this study, we established a novel NSC line and investigated the cytotoxic responses of NSCs to exogenous neurotoxicants, glutamates and reactive oxygen species (ROS). A multipotent NSC line, B2A1 cells, was established from long-term primary cultures of oligodendrocyte-enriched cells from an adult BALB/c mouse brain. B2A1 cells could be differentiated into neuronal, astrocytic and oligodendroglial lineages. The cells also expressed genotypic mRNA messages for both neural progenitor cells and differentiated neuronoglial cells. B2A1 cells treated with hydrogen peroxide and L-buthionine-(S,R)-sulfoximine underwent 30-40% cell death, while B2A1 cells treated with glutamate and kainate showed 25-35% cell death. Cytopathologic changes consisting of swollen cell bodies, loss of cytoplasmic processes, and nuclear chromatin disintegration, developed after exposure to both ROS and excitotoxic chemicals. These results suggest that B2A1 cells may be useful in the study of NSC biology and may constitute an effective neurotoxicity screening system for ROS and excitotoxic chemicals

Combined low dose cyclosporine and prednisone down-regulate natural killer cell-like effector functions of CD8brightCD56+ T cells in patients with active Behcet uveitis

PURPOSE: To investigate the changes of effector-related phenotypic markers and the natural killer (NK)-like effector functions of CD8(bright)CD56+ T cells in patients with Behcet uveitis after combined cyclosporine and prednisone (Cs/Pd) treatment. METHODS: Ten patients with active Behcet panuveitis and 10 healthy controls were prospectively recruited in this study. The effector-related surface markers (CD27, CD62L, CD11b, HLA-DR, CD94, NKG2D), chemokine receptors (CXCR1, CXCR3, CCR4, CCR5), and intracellular perforin of circulating CD8(bright)CD56+ T cells were determined by flow cytometric analysis before and after two months' treatment. NK-like cytotoxicity of ex vivo CD8(bright)CD56+ T cells against K562 was measured by standard 51Cr release assay. RESULTS: The expression levels of effector-related molecules on CD8(bright)CD56+ T cells normalized after treatment. The expression levels of CXCR1 and CCR5 were down-regulated on CD8(bright)CD56+ T cells after treatment. The amounts of preformed intracellular perforin of CD8(bright)CD56+ T cells were reduced to the normal levels. Furthermore, the NK-like cytolytic capacities of CD8(bright)CD56+ T cells were decreased after treatment. CONCLUSIONS: Our results suggest that the combined Cs/Pd treatments in active Behcet uveitis may downregulate the NK-like effector functions of CD8(bright)CD56+ T cells