Towards a TDP-43-based biomarker for ALS and FTLD
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...
| Principais autores: | , , , , |
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| Formato: | Journal article |
| Publicado em: |
Springer US
2018
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| _version_ | 1826287595988975616 |
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| author | Feneberg, E Gray, E Ansorge, O Talbot, K Turner, M |
| author_facet | Feneberg, E Gray, E Ansorge, O Talbot, K Turner, M |
| author_sort | Feneberg, E |
| collection | OXFORD |
| description | 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. |
| first_indexed | 2024-03-07T02:01:01Z |
| format | Journal article |
| id | oxford-uuid:9d5cc1db-5ce2-4b0e-9e82-8b59f512e91c |
| institution | University of Oxford |
| last_indexed | 2024-03-07T02:01:01Z |
| publishDate | 2018 |
| publisher | Springer US |
| record_format | dspace |
| spelling | oxford-uuid:9d5cc1db-5ce2-4b0e-9e82-8b59f512e91c2022-03-27T00:42:34ZTowards a TDP-43-based biomarker for ALS and FTLDJournal articlehttp://purl.org/coar/resource_type/c_dcae04bcuuid:9d5cc1db-5ce2-4b0e-9e82-8b59f512e91cSymplectic Elements at OxfordSpringer US2018Feneberg, EGray, EAnsorge, OTalbot, KTurner, MTDP-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. |
| spellingShingle | Feneberg, E Gray, E Ansorge, O Talbot, K Turner, M Towards a TDP-43-based biomarker for ALS and FTLD |
| title | Towards a TDP-43-based biomarker for ALS and FTLD |
| title_full | Towards a TDP-43-based biomarker for ALS and FTLD |
| title_fullStr | Towards a TDP-43-based biomarker for ALS and FTLD |
| title_full_unstemmed | Towards a TDP-43-based biomarker for ALS and FTLD |
| title_short | Towards a TDP-43-based biomarker for ALS and FTLD |
| title_sort | towards a tdp 43 based biomarker for als and ftld |
| work_keys_str_mv | AT feneberge towardsatdp43basedbiomarkerforalsandftld AT graye towardsatdp43basedbiomarkerforalsandftld AT ansorgeo towardsatdp43basedbiomarkerforalsandftld AT talbotk towardsatdp43basedbiomarkerforalsandftld AT turnerm towardsatdp43basedbiomarkerforalsandftld |