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Altered localization and functionality of TAR DNA Binding Protein 43 (TDP-43) in niemann- pick disease type C

Overview of attention for article published in Acta Neuropathologica Communications, May 2016
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  • In the top 25% of all research outputs scored by Altmetric
  • High Attention Score compared to outputs of the same age (85th percentile)
  • Good Attention Score compared to outputs of the same age and source (70th percentile)

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Title
Altered localization and functionality of TAR DNA Binding Protein 43 (TDP-43) in niemann- pick disease type C
Published in
Acta Neuropathologica Communications, May 2016
DOI 10.1186/s40478-016-0325-4
Pubmed ID
Authors

A. Dardis, S. Zampieri, S. Canterini, K. L. Newell, C. Stuani, J. R. Murrell, B. Ghetti, M. T. Fiorenza, B. Bembi, E. Buratti

Abstract

Niemann-Pick type C (NPC) disease is a lysosomal storage disorder characterized by the occurrence of visceral and neurological symptoms. At present, the molecular mechanisms causing neurodegeneration in this disease are unknown. Here we report the altered expression and/or mislocalization of the TAR-DNA binding protein 43 (TDP-43) in both NPC mouse and in a human neuronal model of the disease. We also report the neuropathologic study of a NPC patient's brain, showing that while TDP-43 is below immunohistochemical detection in nuclei of cerebellar Purkinje cells, it has a predominant localization in the cytoplasm of these cells. From a functional point of view, the TDP-43 mislocalization, that occurs in a human experimental neuronal model system, is associated with specific alterations in TDP-43 controlled genes. Most interestingly, treatment with N-Acetyl-cysteine (NAC) or beta-cyclodextrin (CD) can partially restore TDP-43 nuclear localization. Taken together, the results of these studies extend the role of TDP-43 beyond the Amyotrophic lateral sclerosis (ALS)/frontotemporal dementia (FTD)/Alzheimer disease (AD) spectrum. These findings may open novel research/therapeutic avenues for a better understanding of both NPC disease and the TDP-43 proteinopathy disease mechanism.

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X Demographics

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Mendeley readers

Mendeley readers

The data shown below were compiled from readership statistics for 62 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Italy 1 2%
Unknown 61 98%

Demographic breakdown

Readers by professional status Count As %
Student > Ph. D. Student 11 18%
Student > Bachelor 10 16%
Researcher 8 13%
Other 6 10%
Professor 5 8%
Other 11 18%
Unknown 11 18%
Readers by discipline Count As %
Biochemistry, Genetics and Molecular Biology 13 21%
Agricultural and Biological Sciences 10 16%
Medicine and Dentistry 7 11%
Neuroscience 7 11%
Pharmacology, Toxicology and Pharmaceutical Science 4 6%
Other 8 13%
Unknown 13 21%
Attention Score in Context

Attention Score in Context

This research output has an Altmetric Attention Score of 11. This is our high-level measure of the quality and quantity of online attention that it has received. This Attention Score, as well as the ranking and number of research outputs shown below, was calculated when the research output was last mentioned on 13 February 2018.
All research outputs
#2,712,414
of 22,873,031 outputs
Outputs from Acta Neuropathologica Communications
#492
of 1,379 outputs
Outputs of similar age
#48,860
of 334,245 outputs
Outputs of similar age from Acta Neuropathologica Communications
#8
of 31 outputs
Altmetric has tracked 22,873,031 research outputs across all sources so far. Compared to these this one has done well and is in the 87th percentile: it's in the top 25% of all research outputs ever tracked by Altmetric.
So far Altmetric has tracked 1,379 research outputs from this source. They typically receive a lot more attention than average, with a mean Attention Score of 12.8. This one has gotten more attention than average, scoring higher than 63% of its peers.
Older research outputs will score higher simply because they've had more time to accumulate mentions. To account for age we can compare this Altmetric Attention Score to the 334,245 tracked outputs that were published within six weeks on either side of this one in any source. This one has done well, scoring higher than 85% of its contemporaries.
We're also able to compare this research output to 31 others from the same source and published within six weeks on either side of this one. This one has gotten more attention than average, scoring higher than 70% of its contemporaries.