Comparative analysis of bevacizumab and sorafenib on the survival of retinal ganglion cells in the treatment of retinal diseases.

This study investigates the effects of bevacizumab, a common vascular endothelial growth factor (VEGF) inhibitor, in treating ocular neovascular disorders, with a focus on its impact on retinal ganglion cell (RGC) survival. Given that bevacizumab has been associated with adverse effects on RGCs, we aimed to validate these reports, identify an alternative VEGF inhibitor with similar antiangiogenic efficacy but without detrimental effects on RGCs, and explore the underlying mechanisms. Using primary RGCs extracted from neonatal rats and human umbilical vascular endothelial cells (HUVECs), we compared the efficacy of bevacizumab with other VEGF inhibitors and assessed the apoptotic effects and cell survival pathways influenced by these treatments.

Effects of Bone Morphogenetic Protein-7 on Steroid-Induced Extracellular Matrix Accumulation in Human Trabecular Meshwork Cells.

Long-term steroid use, though essential for treating eye diseases, can cause increased intraocular pressure (IOP) in susceptible individuals and may lead to steroid-induced glaucoma in a subset of patients. This study investigated the effect of bone morphogenetic protein-7 (BMP-7) on steroid-induced extracellular matrix (ECM) synthesis in human trabecular meshwork (TM) cells. We sought to explore the potential of BMP-7 as a protective agent against steroid-induced ECM accumulation in the TM.

Neuroprotective Mechanisms of Ciliary Neurotrophic Factor in Retinal Ganglion Cells: Insights from Microarray Analysis.

Purpose: This study investigated the changes in gene expression in retinal ganglion cells (RGCs) following ciliary neurotrophic factor (CNTF) treatment to elucidate the underlying mechanisms contributing to its neuroprotective effects.

Methods: RGCs isolated from Sprague-Dawley rat pups were treated with recombinant CNTF. Gene expression was analyzed via microarray.

Ciliary Neurotrophic Factor Derived From Astrocytes Protects Retinal Ganglion Cells Through PI3K/AKT, JAK/STAT, and MAPK/ERK Pathways.

Purpose: The purpose of this study was to investigate the roles of ciliary neurotrophic factor (CNTF) on the protective effects of astrocytes on retinal ganglion cells (RGCs).

Methods: Primary RGCs were isolated from neonatal rats. Oxidative stress was induced, and the effects of co-culture with astrocytes and CNTF treatment on RGCs were evaluated.

[Brain MRI Findings of the Cri-Du-Chat Syndrome: A Case Report and Summary].

Cri-du-chat syndrome is a rare genetic disorder in which the patient presents with a characteristic high-pitched monotonous cry and recurrent aspiration pneumonia, attributed to abnormalities in the larynx, epiglottis, and nervous system. The most prominent brain MRI findings are the presence of pontine and cerebellar hypoplasia, which primarily involve posterior cranial fossa structures. Although atrophy of supratentorial structures were also a common radiological finding, it was considered to be a secondary change due to pontine hypoplasia.

[Delayed Post-Hypoxic Leukoencephalopathy Induced by an Overdose with Fentanyl Patches: A Case Report].

Fentanyl intoxication has occasionally been reported since fentanyl patches became available for medical use. Delayed post-hypoxic leukoencephalopathy is a complication of hypoxia. However, its neuropsychiatric symptoms can be delayed, and it can progress to leukoencephalopathy even after full recovery from coma due to acute intoxication.

A principled strategy for mapping enhancers to genes.

Mapping enhancers to genes is a fundamental goal of modern biology. We have developed an innovative strategy that maps enhancers to genes in a principled manner. We illustrate its power by applying it to Myrf.

Elucidating the transactivation domain of the pleiotropic transcription factor Myrf.

Myrf is a newly discovered membrane-bound transcription factor that plays an essential role in as diverse organisms as human, worm, and slime mold. Myrf is generated as a type-II membrane protein in the endoplasmic reticulum (ER). It forms homo-oligomers to undergo auto-cleavage that releases Myrf N-terminal fragment from the ER membrane as a homo-trimer.

Homo-trimerization is essential for the transcription factor function of Myrf for oligodendrocyte differentiation.

Myrf is a key transcription factor for oligodendrocyte differentiation and central nervous system myelination. We and others have previously shown that Myrf is generated as a membrane protein in the endoplasmic reticulum (ER), and that it undergoes auto-processing to release its N-terminal fragment from the ER, which enters the nucleus to work as a transcription factor. These previous studies allow a glimpse into the unusual complexity behind the biogenesis and function of the transcription factor domain of Myrf.

Characterization of Fabry mice treated with recombinant adeno-associated virus 2/8-mediated gene transfer.

Background: Enzyme replacement therapy (ERT) with alpha-galactosidase A (alpha-Gal A) is currently the most effective therapeutic strategy for patients with Fabry disease, a lysosomal storage disease. However, ERT has limitations of a short half-life, requirement for frequent administration, and limited efficacy for patients with renal failure. Therefore, we investigated the efficacy of recombinant adeno-associated virus (rAAV) vector-mediated gene therapy for a Fabry disease mouse model and compared it with that of ERT.

Tissue-specific activation of mitogen-activated protein kinases for expression of transthyretin by phenylalanine and its metabolite, phenylpyruvic acid.

Phenylketonuria is an autosomal recessive disorder caused by a deficiency of phenylalanine hydroxylase. Transthyretin has been implicated as an indicator of nutritional status in phenylketonuria patients. In this study, we report that phenylalanine and its metabolite, phenylpyruvic acid, affect MAPK, changing transthyretin expression in a cell- and tissue-specific manner.

Expression of genes and their responses to enzyme replacement therapy in a Fabry disease mouse model.

Fabry disease is a lysosomal storage disease caused by a deficiency of alpha-galactosidase A, which results in aberrant glycosphingolipid metabolism and accumulation of globotriaosylceramide (Gb3). Since a correlation between the level of Gb3 and clinical manifestations of Fabry disease has not been observed, we investigated potential diagnostic biomarkers. Hepatic and renal gene expression of male alpha-galactosidase A-deficient mice (Fabry mice) was compared with that of wild-type mice.

Protective effect of recombinant adeno-associated virus 2/8-mediated gene therapy from the maternal hyperphenylalaninemia in offsprings of a mouse model of phenylketonuria.

Phenylketonuria (PKU) is an autosomal recessively inherited metabolic disorder caused by a deficiency of phenylalanine hydroxylase (PAH). The accumulation of phenylalanine leads to severe mental and psychomotor retardation, and the fetus of an uncontrolled pregnant female patient presents with maternal PKU syndrome. We have reported previously on the cognitive outcome of biochemical and phenotypic reversal of PKU in a mouse model, Pahenu2, by the AAV serotype 2-mediated gene delivery of a human PAH transgene.

Migration of styrene monomer, dimers and trimers from polystyrene to food simulants.

Migration experiments with polystyrene were performed in two-sided contact with n-heptane and distilled water as the food simulants at temperatures of 10, 24 and 40, and 40, 60 and 90 degrees C, respectively. The surface/volume ratios in the migration cell were set at 8.04 and 10.

Migration of surrogate contaminants in paper and paperboard into water through polyethylene coating layer.

The migration of five surrogate contaminants, anthracene, benzophenone, dimethyl phthalate, methyl stearate and pentachlorophenol, from paper and paperboard into water through a polyethylene (PE) coating layer was investigated. Virgin paper and paperboard coated with PE films of 0.012 and 0.