Electrophysiological evaluation of lower motor neuron loss in amyotrophic lateral sclerosis, Jeremy M. Shefner Ludolph Using neuroimaging to understand brain-behaviour relationships in the context of motor neuron disease, Sandra E. Black and Yana Yunusova Amyotrophic lateral sclerosis and the frontotemporal dementias: using neuroimaging to quantitate disease progression, S. Kalra Differentiating normal from pathological atrophy: when is frontotemporal atrophy normal?
Chow Cerebrospinal fluid-based biomarkers for amyotrophic lateral sclerosis, Robert Bowser, James Connor, and Martin Turner Neuropathology of frontotemporal lobar degeneration, Nigel J. Cairns The neuropathology of the motor neurone diseases, J. Robin Highley and Paul G. Ince Genetics of frontotemporal dementia, Bryan J. Traynor and Stuart Pickering-Brown Strong Alterations in tau metabolism in amyotrophic lateral sclerosis with cognitive impairment, Michael J. PubMed ID: Byrne et al. PubMed ID: Couthouis et al. PubMed ID: Cruts et al.
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ALS and Frontotemporal Degeneration
Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration: Vol 14, No 1
Moreover, chronic inflammation is a common feature observed in ALS and FTD, indicating an essential role of microglia, the resident immune cells of the central nervous system, in disease development and progression. Specifically, we will focus on the role of impaired phagocytosis and increased inflammatory responses and their impact on microglial function.
Several genes associated with the disorders can directly be linked to microglial activation, phagocytosis and neuroinflammation. Other genes associated with the disorders are implicated in biological pathways involved in protein degradation and autophagy. In general such mutations have been shown to cause abnormal protein accumulation and impaired autophagy. These impairments have previously been linked to affect the innate immune system in the central nervous system through inappropriate activation of microglia and neuroinflammation, highlighted in this review.
Although it has been well established that microglia play essential roles in neurodegenerative disorders, the precise underlying mechanisms remain to be elucidated.