Characterization of the retinal degeneration and glial activation of neuronal ceroid lipofuscinosis mouse models Cln3 ex7-8 and Cln6 nclf and the beneficial effects of dietary supplementation:
Gespeichert in:
| 1. Verfasser: | |
|---|---|
| Format: | Abschlussarbeit Buch |
| Sprache: | English |
| Veröffentlicht: |
2013
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| Schlagworte: | |
| Online-Zugang: | https://epub.uni-regensburg.de/28892/ Volltext Inhaltsverzeichnis |
| Beschreibung: | IV, 109 S. Ill., graph. Darst. |
Internformat
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| 100 | 1 | |a Mirza, Myriam |d 1985- |e Verfasser |0 (DE-588)1043112952 |4 aut | |
| 245 | 1 | 0 | |a Characterization of the retinal degeneration and glial activation of neuronal ceroid lipofuscinosis mouse models Cln3 ex7-8 and Cln6 nclf and the beneficial effects of dietary supplementation |c vorgelegt von Myriam Mirza |
| 264 | 1 | |c 2013 | |
| 300 | |a IV, 109 S. |b Ill., graph. Darst. | ||
| 336 | |b txt |2 rdacontent | ||
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| 502 | |a Regensburg, Univ., Diss., 2013 | ||
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Datensatz im Suchindex
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TABLE OF CONTENTS I
TABLE OF CONTENTS
1. INTRODUCTION 1
1.1 STRUCTURE AND FUNCTION OF THE MAMMALIAN RETINA 1
1.2 MIILLER CELLS 2
1.3 MICROGLIA CELLS 3
1.3.1 MICROGLIA IN THE CNS AND RETINA 3
1.3.2 FUNCTION OF THE MICROGLIA CELLS 4
1.3.3 ACTIVATION AND MORPHOLOGICAL PLASTICITY OF MICROGLIA CELLS 4
1.4 INHERITED DISEASES OF THE EYE 6
1.4.1 MICROGLIA IN RETINAL DEGENERATION 7
1.4.2 MIILLER CELLS IN RETINAL DEGENERATION 8
1.5 NEURONAL CEROID LIPOFUSCINOSIS (NCL] 9
1.5.1 CHARACTERISTICS OF NCL 9
1.5.2 NCL AS A LYSOSOMAL STORAGE DISORDER 10
1.5.3 CHARACTERISTICS OF CLN3 MUTATIONS 11
1.5.4 CHARACTERISTICS OF CLN6 MUTATIONS 12
1.5.5 ANIMAL MODELS OF CLN3 AND CLN6 13
1.5.6 RETINAL DEGENERATION IN NCL 14
1.5.7 GLIAL ACTIVATION IN NCL 15
1.6 GLIAL ATTENUATION WITH NATURAL COMPOUNDS 16
1.6.1 CURUCMIN 16
1.6.2 LUTEOLIN 16
1.6.3 DHA 17
1.7 AIM OF THE THESIS 17
2. MATERIALS 19
HTTP://D-NB.INFO/1044564962
TABLE OF CONTENTS II
2.1 MOUSE MODELS 19
2.2 OLIGONUCLEOTIDES FOR REAL-TIME RT-PCR 19
2.3 ENZYMES 20
2.4 ANTIBODIES 20
2.4 CHEMICAL AND KIT SYSTEM 20
2.5 DIETARY SUPPLEMENTATION 22
2.6 ELECTRORETINOGRAMS 22
2.7 BUFFERS AND SOLUTIONS 22
2.8 BASIC MATERIALS 23
2.9 MACHINES AND SOFTWARE 24
3 METHODS 26
3.1 MOUSE LINES AND HUSBANDRY 26
3.2 MOUSE GENOTYPING 26
3.2.1 DNA EXTRACTIONS 26
3.2.2 PHOTOMETRIC DETERMINATION OF DNA CONCENTRATION 26
3.2.3. DNA AMPLIFICATION WITH PCR 26
3.2.4 DNA AMPLIFICATION FOR SEQUENCING 27
3.2.5 DNA SEPARATION AND ANALYSIS 28
3.2.6 SEQUENCE ANALYSIS 29
3.3 RETINAL PREPARATIONS FOR EXPERIMENTS 29
3.3.1 CRYO-EMBEDDING AND SECTIONS 29
3.3.2 WHOLE RETINAL FLAT MOUNTS 29
3.3.3 MORPHOMETRY EXPERIMENTS 30
3.4 MORPHOLOGICAL AND IMMUNOHISTOLOGICAL ANALYSES OF PREPARED RETINAE 30
3.4.1 HEMATOXYLIN AND EOSIN STAIN : 30
3.4.2 IMMUNOHISTOCHEMICAL STAIN OF RETINAL SECTIONS :. 30
TABLE OF CONTENTS III
3.4.3 IMMUNOHISTOCHEMICAL STAIN OF RETINAL FLAT MOUNTS 31
3.4.4 TUNEL ASSAY 31
3.4.5 MICROSCOPY 31
3.4.6 RETINAL MORPHOMETRY ANALYSIS 32
3.5 BEHAVIOUR AND RETINAL FUNCTION STUDIES . . 32
3.5.1 OPTOMOTRY 32
3.5.1 ROTAROD 34
3.5.2 ELECTRORETINOGRAMS 34
3.6 RNA GENE EXPRESSION ANALYSIS 34
3.6.1 RNA ISOLATION 35
3.6.2 PHOTOMETRIC DETERMINATION OF RNA CONCENTRATION 35
3.6.3 REVERSE TRANSCRIPTION 35
3.6.4 TAQMAN TECHNOLOGY 36
3.6.5 RELATIVE QUANTIFICATION 37
3.7 FOOD SUPPLEMENTATION STUDY 38
3.8 STATISTICS 38
4. RESULTS 39
4.1 CHARACTERIZATION OF THE CLNS^"
7 8
RETINA 39
4.1.1 HISTOLOGICAL CHARACTERIZATION OF THE CLN3
&EX7
'
8
RETINA AND IMMUNOHISTOLOGICAL EVALUATION
OF MIILLER AND MICROGLIA CELLS 39
4.1.2 FUNCTIONAL CHARACTERIZATION OF THE CLN3
6EX7
-
8
RETINA 41
4.1.3 CRB*
18
MUTATION PRESENT IN THE CLN3
AEX7
-
8
AND WILD TYPE BACKGROUND 44
4.1.4 END OF CLN3
AEX7
-
8
STUDY 44
4.2 CHARACTERIZATION OF THE CIN6
NCL
F RETINA 44
4.2.1 MORPHOLOGICAL CHARACTERIZATION OF THE CLN6
NCL
F RETINA 44
4.2.2 QUANTIFICATION OF RETINAL DEGENERATION 47
4.2.3 BEHAVIOURAL AND FUNCTIONAL CHARACTERIZATION OF THE CLN6
NC
'F
RETINA AND MOUSE MODEL 49
TABLE OF CONTENTS IV
4.2.4 TRANSCRIPTIONAL CHANGES IN STRESS AND INFLAMMATORY GENE MARKERS IN
THE CLN6
NC
'F
RETINA
51
4.3 CLN6
NC
'/DIETARY SUPPLEMENTATION STUDY 53
4.3.1 SUPPLEMENTATION EFFECT ON CLN6
NC
'F RETINAL HISTOLOGY AND MICROGLIA 54
4.3.2 RETINAL MORPHOMETRY OF SUPPLEMENTED RETINAS 55
4.3.3 RETINAL FUNCTION OF SUPPLEMENTED RETINAS 56
4.3.4 TRANSCRIPTIONAL CHANGES AFFECTED BY SUPPLEMENTATION 58
5. DISCUSSION 60
5.1 COMPARISON OF RETINAL DEGENERATION IN NCL MODELS 60
5.2 MICROGLIA AND MIILLER CELLS IN NCL RETINAL DEGENERATION 62
5.2.1 GLIAL ACTIVATION IN CLN3
AE
*
7A
RETINA 63
5.2.2 GLIAL ACTIVATION IN CLN6
NC,
F RETINA 64
5.3 IMMUNO-MODULATION AND NEURONAL DEGENERATION RESCUE VIA DIETARY
SUPPLEMENTS 66
5.4 PERSPECTIVE 70
5.4.1 CLN3 AND CLN6: DIFFERENT BUT SIMILAR? 70
5.4.2 MIILLER CELLS, MICROGLIA AND MODULATION VIA NATURAL COMPOUNDS 71
6. SUMMARY 73
7. ZUSAMMENFASSUNG 75
8. REFERENCES 77
LIST OF TABLES 93
LIST OF FIGURES 94
LIST OF ABBREVIATIONS 96
LIST OF PUBLICATIONS 101
CONFERENCE CONTRIBUTIONS 102
CURRICULUM VITAE 104
GRATEFUL ACKNOWLEDGEMENTS 107 |
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| physical | IV, 109 S. Ill., graph. Darst. |
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| spelling | Mirza, Myriam 1985- Verfasser (DE-588)1043112952 aut Characterization of the retinal degeneration and glial activation of neuronal ceroid lipofuscinosis mouse models Cln3 ex7-8 and Cln6 nclf and the beneficial effects of dietary supplementation vorgelegt von Myriam Mirza 2013 IV, 109 S. Ill., graph. Darst. txt rdacontent n rdamedia nc rdacarrier Regensburg, Univ., Diss., 2013 Mit einer Zssfassung in dt. Sprache Netzhautdegeneration (DE-588)4171515-9 gnd rswk-swf Nervendegeneration (DE-588)4300182-8 gnd rswk-swf Mikroglia (DE-588)4301913-4 gnd rswk-swf (DE-588)4113937-9 Hochschulschrift gnd-content Netzhautdegeneration (DE-588)4171515-9 s Nervendegeneration (DE-588)4300182-8 s Mikroglia (DE-588)4301913-4 s b DE-604 Erscheint auch als Online-Ausgabe urn:nbn:de:bvb:355-epub- https://epub.uni-regensburg.de/28892/ Resolving-System https://nbn-resolving.org/urn:nbn:de:bvb:355-epub-288922 Verlag kostenfrei Volltext DNB Datenaustausch application/pdf http://bvbr.bib-bvb.de:8991/F?func=service&doc_library=BVB01&local_base=BVB01&doc_number=026814957&sequence=000001&line_number=0001&func_code=DB_RECORDS&service_type=MEDIA Inhaltsverzeichnis |
| spellingShingle | Mirza, Myriam 1985- Characterization of the retinal degeneration and glial activation of neuronal ceroid lipofuscinosis mouse models Cln3 ex7-8 and Cln6 nclf and the beneficial effects of dietary supplementation Netzhautdegeneration (DE-588)4171515-9 gnd Nervendegeneration (DE-588)4300182-8 gnd Mikroglia (DE-588)4301913-4 gnd |
| subject_GND | (DE-588)4171515-9 (DE-588)4300182-8 (DE-588)4301913-4 (DE-588)4113937-9 |
| title | Characterization of the retinal degeneration and glial activation of neuronal ceroid lipofuscinosis mouse models Cln3 ex7-8 and Cln6 nclf and the beneficial effects of dietary supplementation |
| title_auth | Characterization of the retinal degeneration and glial activation of neuronal ceroid lipofuscinosis mouse models Cln3 ex7-8 and Cln6 nclf and the beneficial effects of dietary supplementation |
| title_exact_search | Characterization of the retinal degeneration and glial activation of neuronal ceroid lipofuscinosis mouse models Cln3 ex7-8 and Cln6 nclf and the beneficial effects of dietary supplementation |
| title_full | Characterization of the retinal degeneration and glial activation of neuronal ceroid lipofuscinosis mouse models Cln3 ex7-8 and Cln6 nclf and the beneficial effects of dietary supplementation vorgelegt von Myriam Mirza |
| title_fullStr | Characterization of the retinal degeneration and glial activation of neuronal ceroid lipofuscinosis mouse models Cln3 ex7-8 and Cln6 nclf and the beneficial effects of dietary supplementation vorgelegt von Myriam Mirza |
| title_full_unstemmed | Characterization of the retinal degeneration and glial activation of neuronal ceroid lipofuscinosis mouse models Cln3 ex7-8 and Cln6 nclf and the beneficial effects of dietary supplementation vorgelegt von Myriam Mirza |
| title_short | Characterization of the retinal degeneration and glial activation of neuronal ceroid lipofuscinosis mouse models Cln3 ex7-8 and Cln6 nclf and the beneficial effects of dietary supplementation |
| title_sort | characterization of the retinal degeneration and glial activation of neuronal ceroid lipofuscinosis mouse models cln3 ex7 8 and cln6 nclf and the beneficial effects of dietary supplementation |
| topic | Netzhautdegeneration (DE-588)4171515-9 gnd Nervendegeneration (DE-588)4300182-8 gnd Mikroglia (DE-588)4301913-4 gnd |
| topic_facet | Netzhautdegeneration Nervendegeneration Mikroglia Hochschulschrift |
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