Regulation and expression of genes associated with neurodegeneration with brain iron accumulation
نویسنده
چکیده
Regulation and expression of genes associated with neurodegeneration with brain iron accumulation" (2010). Appendix A: Additional PLA2G6 in situ hybridization images 166 iii LIST OF FIGURES Figure 1.1 Sub-classification of neurodegeneration with brain iron 3 accumulation (NBIA) Figure 1.2 Radiographic features of PKAN and INAD 6 Figure 1.3 PANK2 geonomic structure and mRNA transcripts 9 Figure 1.4 PANK2 isoforms 11 Figure 1.5 Coenzyme A biosynthesis pathway 14 Figure 1.6 Coenzyme A biosynthesis pathway with chemical structures 15 Figure 1.7 Intronic microRNA biogenesis 18 Figure 1.8 miR-103/7 family 20 Figure 1.9 miR-103-2 conservation within PANK2 intron 5 21 Figure 1.10 iPLA 2-VIA splicing variants 28 Figure 1.11 Phospholipase A 2 activity 30 Figure 2.1 PANK2 structure and transcriptional activity 44 Figure 2.2 Mammalian conservation of PANK2 5'genomic sequence 46 Figure 2.3 Deletion and mutational analysis of the PANK2 minimal promoter 48 Figure 2.4 Binding of SH-SY5Y nuclear proteins to DNA probes EMSA4 and 50 EMSA5 Figure 2.5 Binding of SH-SY5Y nuclear proteins to DNA probes EMSA1, 51 EMSA2, EMSA3, EMSA4 and EMSA5 Figure 2.6 Binding of SH-SY5Y nuclear proteins to EMSA5 probe and supershift 53 by hnRNPA/B antibody Figure 2.7 PANK2 expression and PANK2 promoter activity following 2 hour 66 treatment with various doses of H 2 O 2 in SH-SY5Y cells Figure 2.8 PANK2 expression and PANK2 promoter activity following 100M 67 H 2 O 2 treatment for various lengths of time in SH-SY5Y cells Figure 2.9 PANK2 expression and PANK2 promoter activity following iron 69 depletion and overload in N2a cells Figure 2.10 PANK2 expression and PANK2 promoter activity and relative 71 expression following bezafibrate treatment in N2a cells Figure 2.11 PANK2 and PANK1 expression following treatment with PPAR 73 agonists in SH-SY5Y cells Figure 2.12 PANK2 promoter activity following transient transfection with 77 hnRNPA1 iv Figure 2.13 PANK2 promoter activity following transfection with hnRNPA1, 79 hnRNPA2 and hnRNPA3 shRNA Figure 2.14 Location of PANK2 primers for ChIP PCR within the PANK2 promoter 80 Figure 2.15 Summary of ChIP experiments to detect hnRNPA/B proteins bound to 81 the PANK2 promoter in SH-SY5Y cells Figure 3.1 Relative expression of miR-103, miR-107 and their host genes in 91 mouse tissues
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