This study investigated, through survey methodology, the instructional practices of teachers of English Language Learners (ELLs) with disabilities in Preschool Programs for Children with Disabilities (PPCD). These practices were compared to best-practice recommendations made by a group of evaluators in the field of bilingual special education. Results indicated that teacher practices differed considerably from recommendations made by expert evaluators in the field. Specifically, teachers preferred English as the exclusive language of instruction while expert evaluators strongly recommended bilingual instruction. Also, teachers reported strong administrator support while expert evaluators did not. Furthermore, most teachers reported satisfaction with the instruction of ELLs in their schools while most expert evaluators reported dissatisfaction. Results also showed that when administrators at Individualized Education Program (IEP) meetings encouraged discussion about language of instruction, the likelihood of parent participation in these discussions increased. Language dominance and language proficiency testing of preschool aged ELLs, and representation of LPAC members at IEP meetings were major predictors of whether or not these children would receive referral to the bilingual or ESL programs in the future. Most of the results found in this study supported results found by Mueller, Singer, and Carranza in 2006. This study highlights research favoring the development of the primary language of ELLs in PPCD and Pre-K settings while underscoring the disconnect among teachers' beliefs, training, and instructional practices.

Two corneal dystrophies, posterior polymorphous corneal dystrophy (PPCD) and keratoconus, have been associated with missense mutations found in the transcription factor-encoding gene Visual System Homeobox 1 (VSX1). Despite this association, the pathogenic link between VSX1 and these diseases remains controversial. To address this issue, I utilized a variety of in vitro approaches to study how seven VSX1 missense mutations found in disease populations that span two highly conserved domains, the homeodomain (HD) and CVC domain affect VSX1 transcriptional activity, protein expression levels and subcellular localization. I also carried out an in vivo investigation by generating a mouse line carrying a mutation in Vsx1: P254R. Corneal morphology was examined through histology and ex vivo whole eye confocal imaging which was used to assess corneal thickness. Quantification of immunocytochemistry was used to characterize terminal marker expression in the inner retina compared to previously described phenotypes in Vsx1-null mice. My in vitro results showed that mutations found in both the HD and CVC domain alter the normal transcriptional repression activity in Vsx1. These changes were not due to changes to protein expression or subcellular localization. Characterization of corneal and retinal phenotypes in vivo revealed no significant differences in Vsx1 P254R mice when compared to wild-type and Vsx1-null controls. In conclusion, my work shows that Vsx1 P254R is not pathogenic for corneal dystrophies in a mouse model. However, my in vitro studies show that Vsx1 mutations have the ability to alter transcriptional activity and therefore still have the potential to be pathogenic in humans. Further investigation is needed to determine whether VSX1 mutations found in disease populations are, in fact, causative for corneal dystrophies. / Graduate / 2019-04-25

Over the past decade, fusion research has showed the potential of being a main candidate for energy production for future generations. Further advances in improved fusion performance are therefore vital. This thesis focuses on advanced fusion plasma scenarios and their diagnostic requirements. In particular the design of a motional Stark effect (MSE) diagnostic at the MAST spherical tokamak and the analysis of magneto-hydrodynamic mode feedback control and pulsed poloidal current drive (PPCD) at the reversed field pinch (RFP) experiment EXTRAP T2R are discussed. The MSE diagnostic is important for the determination of the plasma current profile, information that is necessary for studies in advanced confinement scenarios like reversed shear profiles or current holes. The MAST MSE system has two channels and selects the spectral components using 1Å FWHM interference filters. The diagnostic has been commissioned during the fall of 2006 and the results show the feasibility of the technique with rms-noise ~0.5° using a time resolution of 1 ms. Investigations of mirror labyrinths for the future ITER MSE diagnostic highlight the need for careful calibration considerations. Feedback control and PPCD are techniques for improved confinement. Feedback control dramatically decreases impurity influx at the end of discharges while transport in the bulk plasma is largely unaffected. During PPCD the transport is seen to decrease and it is demonstrated that PPCD and feedback control can be employed simultaneously. New and innovative techniques for fusion spectroscopy are furthermore described. This includes the use of correlations in line integrated signals to determine ion emission profiles in poloidally symmetric environments. Good agreement with other diagnostic methods is obtained. The assessment of electron temperature profiles using measured differences between Thomson scattering and vacuum ultra-violet spectroscopy is also shown. / QC 20100907