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Glial cells and their role in amyotrophic lateral sclerosis
Vaňátko, Ondřej ; Anděrová, Miroslava (advisor) ; Černý, Jan (referee)
Amyotrophic lateral sclerosis (ALS, also known as Lou Gehrig's disease) is a progressive neurodegenerative disorder. It affects upper and lower motor neurons in the brain motor cortex, the brain stem and the spinal cord, causing their death, which results in denervation of voluntary muscles. Progressive muscle weakness and atrophy throughout the entire body gradually leads to worsening of the ability to move, speak, chew, swallow and eventually breath. Ultimately it results in affected individual's death due to respiratory muscle failure. Although first identified in 1869, no cure for ALS has been yet found. While early studies focused mainly on the research of motor neurons themselves, the attention has shifted towards glial cells in the past two decades. Glial cells are essential for proper neuron functioning and survival and it appears that they play a major role in ALS progression. The goal of this thesis is to review and summarize findings on the role of glial cells in ALS over the last years, focusing on four specific types of glial cells, namely astrocytes, microglia, oligodendrocytes and NG2-glia. Key words: amyotrophic lateral sclerosis, ALS, motor neuron, glia, astrocyte, microglia, oligodendrocyte, NG2-glia
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The effect of physiotherapy on respiratory muscle strength and functional capabilities in patients with amyotrophic lateral sclerosis
Vítek, Jiří ; Horáček, Ondřej (advisor) ; Kobesová, Alena (referee)
Bibliography VÍTEK, Jiří. The effect of physiotherapy on respiratory muscle strength and functional ability in patients with amyotrophic lateral sclerosis. Prague: Charles University, 2nd Faculty of Medicine, Department of Rehabilitation and Sports Medicine, 2017. 132 p. Supervisor prim. MUDr. Ondřej Horáček, Ph.D. Abstract Objectives To determine the effects of intensive physiotherapeutic care in patients with Amyotrophic lateral sclerosis (ALS), with the focus on objective measurement of respiratory functions and functional status. Methods Patients with ALS (n = 21) were non-randomly assigned into two groups. First group (group R) received a 3-week in-patient rehabilitation programme in the Rehabilitation Hospital Beroun. Second group, control group (group K), received a home exercise programme. Patients from both groups underwent spirometry examination and evaluation of functional skills via the Amyotrophic lateral sclerosis functional rating scale revised questionnaire (ALSFRS-R) three weeks apart. Among the spirometry parameters, forced vital capacity (FVC) and forced expiratory volume in 1 second (FEV1) were selected for further evaluation. These parameters are significantly associated with respiratory muscle strength and survival in patients with ALS. Results There were no significant differences in...
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Immunomodulatory properties of mesenchymal stem cells from patients with amyotrophic lateral sclerosis and healthy donors
Matějčková, Nicole ; Javorková, Eliška (advisor) ; Kanderová, Veronika (referee)
Mesenchymal stem cells (MSC) possess a multilineage differentiation potential and have the ability to regulate reactivity of the immune system. They are usually isolated and expanded from the bone marrow, adipose tissue or umbilical cord. MSC represent promising cell population for the treatment of some severe diseases, such as amyotrofic lateral sclerosis (ALS), due to the combination of regenerative and immunomodulatory properties. The aim of this study is to compare MSC from ALS patients and healthy donors in their phenotype, proliferative activity and mainly their immunomodulatory properties. The assessment of impact of the disease on the properties of MSC is important for their autologous use in clinical trials. In this study we used MSC isolated from bone marrow of 14 ALS patients and 15 patients undergoing mostly orthopedic surgery as control group. We also used MSC stimulated for 24 hours by poinflammatory cytokines. Cells were compared in terms of immunophenotype, differentiation in adipocytes and osteoblasts, metabolic activity, expression of selected genes for immunomodulatory molecules and for inhibition of lymphocyte proliferation. Further experiments were focused on evaluation of immunomodulatory properties of MSC. The effect of MSC on peripheral blood mononuclear cells stimulated...
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Modelovanie ochorenia a štúdium regeneračných procesov v Huntingtonovej chorobe a ALS in vivo
Hruška-Plocháň, Marián
Neurological disorders affect more than 14% of the population worldwide and together with traumatic brain and spinal cord injuries represent major health, public and economic burden of the society. Incidence of inherited and idiopathic neurodegenerative disorders and acute CNS injuries is growing globally while neuroscience society is being challenged by numerous unanswered questions. Therefore, research of the CNS disorders is essential. Since animal models of the CNS diseases and injuries represent the key step in the conversion of the basic research to the clinics, we focused our work on generation of new animal models and on their use in pre-clinical research. We generated and characterized transgenic minipig model of Huntington's disease (HD) which represents the only successful establishment of a transgenic model of HD in minipig which should be valuable for testing of long term safety of HD therapeutics. Next, we crossed the well characterized R6/2 mouse HD model with the gad mouse model which lacks the expression of UCHL1 which led to results that support the theory of "protective" role of mutant huntingtin aggregates and suggest that UCHL1 function(s) may be affected in HD disturbing certain branches of Ubiquitin Proteasome System. Traumatic spinal cord injury and Amyotrophic Lateral...
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Huntington's disease modeling and stem cell therapy in spinal cord disorders and injury
Hruška-Plocháň, Marián ; Motlík, Jan (advisor) ; Bjarkam, Carsten (referee) ; Roth, Jan (referee)
Neurological disorders affect more than 14% of the population worldwide and together with traumatic brain and spinal cord injuries represent major health, public and economic burden of the society. Incidence of inherited and idiopathic neurodegenerative disorders and acute CNS injuries is growing globally while neuroscience society is being challenged by numerous unanswered questions. Therefore, research of the CNS disorders is essential. Since animal models of the CNS diseases and injuries represent the key step in the conversion of the basic research to the clinics, we focused our work on generation of new animal models and on their use in pre-clinical research. We generated and characterized transgenic minipig model of Huntington's disease (HD) which represents the only successful establishment of a transgenic model of HD in minipig which should be valuable for testing of long term safety of HD therapeutics. Next, we crossed the well characterized R6/2 mouse HD model with the gad mouse model which lacks the expression of UCHL1 which led to results that support the theory of "protective" role of mutant huntingtin aggregates and suggest that UCHL1 function(s) may be affected in HD disturbing certain branches of Ubiquitin Proteasome System. Traumatic spinal cord injury and Amyotrophic Lateral...
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Nursing diagnosis of a client with Amyotrophic lateral sclerosis
ŠVECOVÁ, Karolína
This bachelor thesis deals with the disease Amyotrophic lateral sclerosis (ALS). The theoretical part is focused on the anatomy of motor system, disease characteristics, etiology, symptoms, diagnosis, treatment and specific nurse roles in this issue are also included. It also deals with basic areas of nursing process for the client with this disease. The empirical part was based on research goals and questions, regarding nursing diagnoses and interventions occurring in case of clients with ALS. Qualitative research was used to obtain results. Clients with Amyotrophic lateral sclerosis were selected as a research group. The informations were investigated system of collecting data inspired by D. Orem´s self-care deficit theory. Subsequently, the open style for coding "pencil-paper" method for processing an anamnestic file was used. This method helped to received interventions and specific problems of individual clients, which were subsequently defined as a nursing diagnosis with the help of NANDA Taxonomy II. The publication Nursing Interventions Classification (NIC) was served for comparing of the above-mentioned interventions within discussion. The results provided answers to research questions. The basic nursing diagnoses were find out by the performing and processing of research survey. It dependent on the current stage of disease. The most affected region are universal self-care requirements for clients with ALS. The requirements for this category includes sufficient supply of air, food, fluids, toileting or balance of activity and rest. Subsequently, related interventions were mentioned in the individual nursing diagnoses.
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