|
|
Etiology and mechanisms of pathogenesis of congenital neural crest disorders
Poštulková, Adéla ; Fábik, Jaroslav (advisor) ; Krylov, Vladimír (referee)
The neural crest is an extensively migrating population of cells found only in vertebrates. It is formed in the dorsal part of the neural tube during the early embryonic development by a process called neurulation, in which the neural plate forms and the neuroepithelium subsequently sinks into the embryo. Neural crest cells detach during development and then migrate through the body to their final destinations, where they differentiate into various mesenchymal and non-mesenchymal cell types (derivatives) under the influence of signals. These derivatives include tissues of the peripheral nervous system, tissues of the endocrine system, pigment cells, facial bones, and other connective tissues. Defective migration of neural crest cells and disruption of their differentiation leads to maldevelopment of the neural crest cells derivatives, and to development a group of syndromes known as neurocristopathies, which include a wide range of malformations. The aim of this thesis is to summarize the current knowledge about individual neural crest diseases and syndromes such as Hirschsprung's disease, Treacher-Collins syndrome, piebaldism and neurofibromatosis, their causes, mechanisms of pathogenesis and possible treatment options. Key words: Neural crest, migration, differentiation, neurocristopathies,...
|
guest ::
