Development of neurodegeneration in amyotrophic lateral sclerosis: from up or down?

Autor: Felix Geser, Gregor K. Wenning, Johannes Haybaeck, Lisa Fellner
Rok vydání: 2020
Předmět:
Zdroj: Journal of neural transmission (Vienna, Austria : 1996). 127(8)
ISSN: 1435-1463
Popis: Amyotrophic lateral sclerosis (ALS) is a fatal neurological disease associated with neurodegeneration and intracellular pathological 43-kDa transactive response sequence DNA-binding protein (TDP-43) positive inclusions. The various clinical symptoms, such as motor disorders and cognitive impairment, reflect the degeneration of certain areas of the nervous system. Since the discovery of the significance of pathological TDP-43 for human disease including ALS, there has been an increasing number of studies reporting on the distribution and severity of neurodegeneration. These have rekindled the old debate about whether the first or second motor neuron is the primary site of degeneration in ALS. To shed light on this question, the following is a review of the relevant neuropathological studies.
Databáze: OpenAIRE