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  • About
  • The Global ETD Search service is a free service for researchers to find electronic theses and dissertations. This service is provided by the Networked Digital Library of Theses and Dissertations.
    Our metadata is collected from universities around the world. If you manage a university/consortium/country archive and want to be added, details can be found on the NDLTD website.
61

Genetic testing in Amyotrophic Lateral Sclerosis: A Survey of ALS Clinicians and Commercial Testing Laboratories

Klepek, Holly N. 04 September 2018 (has links)
No description available.
62

Oxidativer Stress als Biomarker für die (Neben-) Wirkungen von Strahlentherapie: Bestimmung von Isoprostanspiegeln und Genexpressionsprofilen in Patientenproben / Oxidative stress as a marker for effects and side effects of radiotherapy. Analysis of isoprostane levels and gene expression profiles in patients samples

Kluge, Friedrich 29 November 2011 (has links)
No description available.
63

Studies on bioactive lipid mediators involved in brain function and neurodegenerative disorders : the effect of ω-3PUFA supplementation and lithium treatment on rat brain sphingomyelin species and endocannabinoids formation : changes in oxysterol profiles in blood of ALS patients and animal models of ALS

Drbal, Abed Alnaser Anter Amer January 2013 (has links)
Lipids are important for structural and physiological functions of neuronal cell membranes. They exhibit a range of biological effects many are bioactive lipid mediators derived from polyunsaturated fatty acids such as sphingolipids, fatty acid ethanolamides (FA-EA) and endocannabinoids (EC). These lipid mediators and oxysterols elicit potent bioactive functions in many physiological and pathological processes of the brain and neuronal tissues. They have been investigated for biomarker discovery of ageing, neuroinflammation and neurodegenerative disorders. The n-3 fatty acids EPA and DPA are thought to exhibit a range of neuroprotective effects many of which are mediated through production of such lipid mediators. The aims of this study were to evaluate the effects of n-3 EPA and n-3 DPA supplementation on RBC membranes and in this way assess dietary compliance and to investigate brain sphingomyelin species of adult and aged rats supplemented with n-3 EPA and n-3 DPA to evaluate the effects and benefits on age-related changes in the brain. Furthermore, to study the effects of lithium on the brain FA-EAs and ECs to further understand the neuroprotective effects of lithium neuroprotective action on neuroinflammation as induced by LPS. Finally to examine if circulating oxysterols are linked to the prevalence of ALS and whether RBC fatty acids are markers of this action in relation to age and disease stages. These analytes were extracted from tissue samples and analysed with GC, LC/ESI-MS/MS and GC-MS. It was found that aged rats exhibited a significant increase in brain AA and decrease in Σn-3 and Σn-6 PUFAs when compared to adult animals. The observed increase of brain AA was reversed following n-3 EPA and n-3 DPA supplementation. Sphingomyelin was significantly increased when aged animals were supplemented with n-3 DPA. LPS treatment following lithium supplementation increased LA-EA and ALA-EA, while it decreased DHA-EA. Both oxysterols 24-OH and 27-OH increased in ALS patients and SOD1-mice. Eicosadienoic acid was different in ASL-patients compared to aged SOD1-mice. These studies demonstrated that dietary intake of n-3 EPA and n-3DPA significantly altered RBC fatty acids and sphingolipids in rat brain. They suggest that n-3 DPA can be a potential storage form for EPA, as shown by retro-conversion of n-3 DPA into EPA in erythrocyte membranes, ensuring supply of n-3 EPA. Also, n-3 EPA and n-3 DPA supplementation can contribute to an increase in brain sphingomyelin species with implications for age effects and regulation of brain development. Effects of lithium highlight novel anti-neuroinflammatory treatment pathways. Both 24-hydroxycholesterol and eicosadienoic acid may be used as biomarkers in ALS thereby possibly helping to manage the progressive stages of disease.
64

Dégénérescence locale et réparation anormale de la jonction neuromusculaire dans un modèle de la sclérose latérale amyotrophique

Martineau, Éric 12 1900 (has links)
No description available.
65

Vyšetření genů DNM2, GARS, MORC2, TRPV4 a SOD1 u českých pacientů s dědičnou neuropatií axonálního typu / Examination of the genes DNM2, GARS, MORC2, TRPV4 and SOD1 among Czech patients with hereditary neuropathy axonal type

Neupauerová, Jana January 2019 (has links)
Examination of the genes DNM2, GARS, MORC2, TRPV4 and SOD1 among Czech patients with hereditary neuropathy axonal type For my PhD thesis I chose to work with patients with axonal form of CMT, because at that time axonal forms were less likely to be clarified by classical methods of molecular genetics. For further examination in patients with unclear cause of the axonal CMT, the genes DNM2, GARS and TRPV4 were selected. The aim was to determine the significance of pathogenic mutations in these genes as the cause of CMT2 in Czech patients. In the course, we identified causal variants in the genes MORC2 and SOD1 with WES. Therefore, we have tested additional CMT2 patients for the presence of these variants. Using Sanger sequencing, I examined a representative set of patients for the DNM2 (37), GARS (10) and TRPV4 (24) genes without finding a causal mutation, then we investigated genes SOD1 (43 patients) and MORC2 (161 patients). The cohort (50 patients) was also subjected to MLPA analysis using a P406-A1 CMT2 duplication and deletion detection kit for genes RAB7A, GARS, HSPB1, HSBP8 and SPTLC1 (kit P406-A1 CMT2). At that time, massively parallel sequencing (MPS) was becoming important. We compared the cost of classical sequencing versus MPS, and accordingly, we decided that the genes DNM2, GARS, MORC2, TRPV4...
66

Gene Therapy for Amyotrophic Lateral Sclerosis: An AAV Delivered Artifical MicroRNA Against Human SOD1 Increases Survival and Delays Disease Progression of the SOD1<sup>G93A</sup> Mouse Model: A Dissertation

Stoica, Lorelei I. 07 December 2015 (has links)
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by loss of motor neurons, resulting in progressive muscle weakness, atrophy, paralysis and death within five years of diagnosis. About ten percent of cases are inherited, of which twenty percent are due to mutations in the superoxide dismutase 1 (SOD1) gene. Since the only FDA approved ALS drug prolongs survival by just a few months, new therapies for this disease are needed. Experiments in transgenic ALS mouse models have shown that decreasing levels of mutant SOD1 protein alters and in some cases entirely prevents disease progression. We explored this potential therapeutic approach by using a single stranded AAV9 vector encoding an artificial microRNA against human SOD1 injected bilaterally into the cerebral lateral ventricles of neonatal SOD1G93A mice. This therapy extended median survival from 135 to 206 days (a 50% increase) and delayed hind limb paralysis. Animals remained ambulatory until endpoint, as defined by a sharp drop in body weight. Treated animals had a reduction of mutant human SOD1 mRNA levels in upper and lower motor neurons. As compared to untreated SOD1G93A mice, the AAV9 treated mice also had significant improvements in multiple parameters including the number of motor neurons, diameter of ventral root axons, and degree of neuroinflammation in the spinal cord. These studies clearly show that an AAV9-delivered artificial microRNA is a translatable therapeutic approach for ALS.
67

Studies on Bioactive Lipid Mediators Involved in Brain Function and Neurodegenerative Disorders. The effect of ¿-3PUFA supplementation and lithium treatment on rat brain sphingomyelin species and endocannabinoids formation; changes in oxysterol profiles in blood of ALS patients and animal models of ALS.

Drbal, Abed Alnaser A.A. January 2013 (has links)
Lipids are important for structural and physiological functions of neuronal cell membranes. They exhibit a range of biological effects many are bioactive lipid mediators derived from polyunsaturated fatty acids such as sphingolipids, fatty acid ethanolamides (FA-EA) and endocannabinoids (EC). These lipid mediators and oxysterols elicit potent bioactive functions in many physiological and pathological processes of the brain and neuronal tissues. They have been investigated for biomarker discovery of ageing, neuroinflammation and neurodegenerative disorders. The n-3 fatty acids EPA and DPA are thought to exhibit a range of neuroprotective effects many of which are mediated through production of such lipid mediators. The aims of this study were to evaluate the effects of n-3 EPA and n-3 DPA supplementation on RBC membranes and in this way assess dietary compliance and to investigate brain sphingomyelin species of adult and aged rats supplemented with n-3 EPA and n-3 DPA to evaluate the effects and benefits on age-related changes in the brain. Furthermore, to study the effects of lithium on the brain FA-EAs and ECs to further understand the neuroprotective effects of lithium neuroprotective action on neuroinflammation as induced by LPS. Finally to examine if circulating oxysterols are linked to the prevalence of ALS and whether RBC fatty acids are markers of this action in relation to age and disease stages. These analytes were extracted from tissue samples and analysed with GC, LC/ESI-MS/MS and GC-MS. It was found that aged rats exhibited a significant increase in brain AA and decrease in ¿n-3 and ¿n-6 PUFAs when compared to adult animals. The observed increase of brain AA was reversed following n-3 EPA and n-3 DPA supplementation. Sphingomyelin was significantly increased when aged animals were supplemented with n-3 DPA. LPS treatment following lithium supplementation increased LA-EA and ALA-EA, while it decreased DHA-EA. Both oxysterols 24-OH and 27-OH increased in ALS patients and SOD1-mice. Eicosadienoic acid was different in ASL-patients compared to aged SOD1-mice. These studies demonstrated that dietary intake of n-3 EPA and n-3DPA significantly altered RBC fatty acids and sphingolipids in rat brain. They suggest that n-3 DPA can be a potential storage form for EPA, as shown by retro-conversion of n-3 DPA into EPA in erythrocyte membranes, ensuring supply of n-3 EPA. Also, n-3 EPA and n-3 DPA supplementation can contribute to an increase in brain sphingomyelin species with implications for age effects and regulation of brain development. Effects of lithium highlight novel anti-neuroinflammatory treatment pathways. Both 24-hydroxycholesterol and eicosadienoic acid may be used as biomarkers in ALS thereby possibly helping to manage the progressive stages of disease. / Libyan Government
68

Hippocampal Neurogenesis In Amyotrophic Lateral Sclerosis Like Mice

Ma, Xiaoxing 10 1900 (has links)
<p> G93A SODI mice (G93A mice) are a transgenic model over-expressing a mutant human Cu/Zn-SOD gene, and are a model for amyotrophic lateral sclerosis (ALS), a predominantly motor neurodegenerative disease. Hippocampal neurogenesis in the subgranular zone (SGZ) of dentate gyms (DG) occurs throughout the life. It is regulated by many pathological and physiological processes. There is controversy with respect to the basal level of hippocampal neurogenesis and its response to exercise in neurodegenerative diseases and their mouse models. Little information regarding hippocampal neurogenesis is available in G93A mice. The present study was designed to study the impact of treadmill exercise and sex differences on hippocampal neurogenesis in this model. In addition, potential molecular mechanisms regulating hippocampal neurogenesis including growth factors (BDNF and IGFl) and oxidative stress (SOD2, catalase, 8-0Hdg, and 3-NT) were also addressed in the study. Bromodeoxyuridine (BrdU) was used to label newly generated cells. G93A and wild type (WT) mice were subjected to treadmill exercise (EX) or a sedentary (SEO) lifestyle. Immunohistochemistry was used to detect BrdU labeled newly proliferating cells, surviving cells, and their phenotype, as well as for determination of oxidative stress. BDNF and IGFl mRNA expression was assessed by in situ hybridization. Results showed that (1) G93A mice had an elevated basal level of hippocampal neurogenesis for both cell survival and neuronal differentiation, a growth factor (BDNF mRNA), and an oxidative stress marker (NT), as compared to wild type sedentary mice. (2) Treadmill running did not show any further effect on hippocampal neurogenesis, growth factors, oxidative stress, and antioxidant enzymes in G93A mice, while treadmill running promoted hippocampal neurogenes1s and expression of the growth factor (BDNF mRNA), and lowered oxidative stress (8-0Hdg) in WT mice. (3) There also were sex differences in hippocampal neurogenesis in G93A mice, whereby male G93A mice had a significant higher level of cell proliferation but a lower level of survival than female G93A mice. (4) The DG BDNF mRNA was associated with cell survival and neuronal differentiation in sedentary G93A mice, suggesting that BDNF is associated with a higher basal level of hippocampal neurogenesis in G93A mice. We conclude that G93A mice are more permissive in the context of hippocampal neurogenesis, which is associated with elevated DG BDNF mRNA expression. Running did not have impact on hippocampal neurogenesis and BDNF mRNA expression in G93A mice, probably due to a 'ceiling effect' of the already heightened basal levels of hippocampal neurogenesis and BDNF mRNA in this model. In addition, sex differences also affect hippocampal neurogenes1s, but the further study is needed to clarify the underlying molecular mechanisms. </p> / Thesis / Doctor of Philosophy (PhD)
69

Therapeutic suppression of mutant SOD1 by AAV9-mediated gene therapy approach in Amyotrophic Lateral Sclerosis

Likhite, Shibi B. January 2014 (has links)
No description available.
70

Optical Analysis of [Ca<sup>2+</sup>]i and Mitochondrial Signaling Pathways: Implications for the Selective Vulnerability of Motoneurons in Amyotrophic Lateral Sclerosis (ALS) / Optische Analysen von [Ca<sup>2+</sup>]i und mitochondrialen Signalwegen: Untersuchungen zur selektiven Verwundbarkeit von Motoneuronen in der amyotrophen Lateralsklerose (ALS)

Jaiswal, Manoj Kumar 23 January 2008 (has links)
No description available.

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