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Related Experiment Video

Updated: Jun 21, 2026

Abbiategrasso Brain Bank Protocol for Collecting, Processing and Characterizing Aging Brains
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TDP-43 and frontotemporal dementia.

William T Hu1, Murray Grossman

  • 1Department of Neurology, University of Pennsylvania School of Medicine, 3400 Spruce Street, Philadelphia, PA 19106, USA. huw@uphs.upenn.edu

Current Neurology and Neuroscience Reports
|August 12, 2009
PubMed
Summary

TAR DNA-binding protein of 43 kDa (TDP-43) is central to frontotemporal lobar degeneration (FTLD-TDP). Its aggregation and movement to the cytoplasm are key in disease, and TDP-43 may serve as a diagnostic biomarker.

Area of Science:

  • Neuroscience
  • Molecular Biology
  • Pathology

Background:

  • TAR DNA-binding protein of 43 kDa (TDP-43) is a primary ubiquitinated protein in tau-negative frontotemporal lobar degeneration (FTLD).
  • TDP-43 normally resides in the nucleus; its aggregation and translocation to the cytoplasm are implicated in FTLD-TDP pathogenesis.
  • TDP-43 pathology links FTLD-TDP with amyotrophic lateral sclerosis (ALS) and is associated with specific frontotemporal dementia clinical syndromes.

Purpose of the Study:

  • To investigate the role of TDP-43 in the pathogenesis of FTLD-TDP.
  • To explore TDP-43 as a potential biomarker for antemortem diagnosis of FTLD-TDP.

Main Methods:

  • Review of recent clinical, genetic, and pathologic studies on FTLD-TDP and ALS.
  • Analysis of TDP-43 aggregation and cellular localization in FTLD-TDP.

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Evaluation of LC3-II Release via Extracellular Vesicles in Relation to the Accumulation of Intracellular LC3-positive Vesicles
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Evaluation of LC3-II Release via Extracellular Vesicles in Relation to the Accumulation of Intracellular LC3-positive Vesicles

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Main Results:

  • TDP-43 aggregation and cytoplasmic translocation are critical steps in FTLD-TDP.
  • TDP-43 pathology serves as a molecular link between FTLD-TDP and ALS.
  • TDP-43 is identified as a candidate biomarker for diagnosing FTLD-TDP before death.

Conclusions:

  • TDP-43 plays a significant pathogenic role in FTLD-TDP and ALS.
  • TDP-43 holds promise as a biomarker for early diagnosis of FTLD-TDP.