Abstract:
:TDP-43 accumulates in nerve cells of nearly all cases of amyotrophic lateral sclerosis (ALS; the commonest form of motor neuron disease) and in the majority of Tau-negative frontotemporal lobar degeneration (FTLD). There is currently no biochemical test or marker of disease activity for ALS or FTLD, and the clinical diagnosis depends on the opinion of an experienced neurologist. TDP-43 has a key role in the pathogenesis of ALS/FTLD. Measuring TDP-43 in easily accessible biofluids, such as blood or cerebrospinal fluid, might reduce diagnostic delay and offer a readout for use in future drug trials. However, attempts at measuring disease-specific forms of TDP-43 in peripheral biofluids of ALS and FTLD patients have not yielded consistent results, and only some of the pathological biochemical features of TDP-43 found in human brain tissue have been detected in clinical biofluids to date. Reflecting on the molecular pathology of TDP-43, this review provides a critical overview on biofluid studies and future directions to develop a TDP-43-based clinical biomarker for ALS and FTLD.
journal_name
Mol Neurobioljournal_title
Molecular neurobiologyauthors
Feneberg E,Gray E,Ansorge O,Talbot K,Turner MRdoi
10.1007/s12035-018-0947-6subject
Has Abstractpub_date
2018-10-01 00:00:00pages
7789-7801issue
10eissn
0893-7648issn
1559-1182pii
10.1007/s12035-018-0947-6journal_volume
55pub_type
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