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HnRNP Pathologies in Frontotemporal Lobar Degeneration
Xinwa Jiang1,2, Ariana Gatt1,2, Tammaryn Lashley1,2
1Queen Square Brain Bank for Neurological Disorders, UCL Queen Square Institute of Neurology, London WC1N 1PJ, UK.
Cells
|June 28, 2023
Summary
Frontotemporal dementia (FTD) involves abnormal proteins, including TDP-43 and FUS. Research shows other hnRNPs are linked to FTD pathology, suggesting a broader role in disease development.
Area of Science:
- Neuroscience
- Pathology
- Genetics
Background:
- Frontotemporal dementia (FTD) is a common cause of early-onset dementia.
- FTD clinically presents with behavioral, executive, and language impairments.
- Frontotemporal lobar degeneration (FTLD) is the primary pathological cause of FTD.
Purpose of the Study:
- To review recent studies on heterogeneous ribonucleic acid proteins (hnRNPs) and their association with FTLD pathology.
- To highlight the emerging role of multiple hnRNPs in FTLD pathogenesis.
Main Methods:
- Literature review of studies investigating hnRNP abnormalities in FTLD.
- Analysis of histological findings and protein mislocalization in FTLD cases.
Main Results:
- FTLD involves proteinopathies with key proteins like tau, TDP-43, and FUS.
- TDP-43 and FUS are members of the hnRNP family.
- Multiple hnRNPs show nuclear depletion and cytoplasmic mislocalization in FTLD neurons.
Conclusions:
- Abnormalities in various hnRNPs are associated with FTLD.
- hnRNPs play a significant role in the pathogenesis of FTLD.
- Future FTLD research should encompass the full spectrum of hnRNPs.
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