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By Michael J. Strong

Over the last ten years, there was an expanding attractiveness that syndromes of frontotemporal disorder (FTD) are a standard prevalence in sufferers with amyotrophic lateral sclerosis (ALS). Such syndromes can be found in as many as 60% of sufferers with ALS. Conversely, the incidence of motor neuron disorder in sufferers with clinically natural frontotemporal dementia is more and more well-known. this implies that to a point there are overlapping syndromes during which either ALS and FTD ensue in the comparable person.

This quantity summarizes the advances in our figuring out of those problems, in addition to the aptitude courting among the 2. Key themes contain advances in our skill to clinically describe the frontotemporal syndromes, preclinical detection, neuroimaging, and genetics. The exploding box of recent markers in neuropathology is tested, as is the function of latest genetic mutations in DNA/RNA delivery platforms. This ebook is the basic reference textual content for this subject, and may be of curiosity to neurologists and neurological trainees with a medical or learn curiosity within the FTDs or ALS, neuropsychologists, neuropathologists, and researchers.

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Neurotoxicology, 28, 702–704. 85. Lobner D, Piana PMT, Salous AK, Peoples RW. (2007). β-N-methylamino-L-alanine enhances neurotoxicity through multiple mechanisms. Neurobiol Dis, 25, 360–366. 86. Rao SD, Banack SA, Cox PA, Weiss JH. (2006). BMAA selectively injures motor neurons via AMPA/kainate receptor activation. Exp Neurol, 201, 244–252. 87. Ly PTT, Singh S, Shaw CA. (2007). Novel environmental toxins: steryl glycosides as a potential etiological factor for age-related neurodegenerative diseases.

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Human, drosophila, and C. elegans TDP43: nucleic acid binding properties and splicing regulatory function. J Mol Biol, 348, 575–588. 128. Buratti E, Brindisi A, Giombi M, Tisminetzky S, Ayala YM, Baralle FE. (2005). TDP-43 binds heterogeneous nuclear ribonucleoprotein A/B through its C-terminal tail. J Biol Chem, 280, 37572–3758. 129. Strong MJ, Volkening K, Hammond R, et al. (2007). TDP43 is a human low molecular weight neurofilament (hNFL) mRNA-binding protein. Mol Cell Neurosci, 35, 320–327.

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