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Expert Opin Biol Ther. 2008 Jul;8(7):969-78. doi: 10.1517/14712598.8.7.969 .

TDP-43 in neurodegenerative disorders.

Author information

1
Mayo Clinic, 4500 San Pablo Road Jacksonville, Florida 32224, USA.

Abstract

BACKGROUND:

The number of neurodegenerative diseases associated with pathological aggregates of transactivation response element (TAR)-DNA-binding protein 43 (TDP-43) has increased, leading to the new designation 'TDP-43 proteinopathy.' Biochemically, TDP-43 proteinopathies are characterized by decreased solubility, hyperphosphorylation, and cleavage of TDP-43 into 25- and 35-kDa fragments, and by altered cellular localization.

OBJECTIVE:

This review summarizes research characterizing the distribution of TDP-43 pathology in human postmortem brain tissue and discusses possible therapeutic strategies based on genetic and in vitro studies.

METHODS:

We reviewed recent studies of TDP-43 proteinopathy.

RESULTS/CONCLUSION:

Given that several different mutations can lead to TDP-43 proteinopathies, including mutations in progranulin and valosin-containing protein, research is needed to decipher and potentially exploit the link between these mutations and TDP-43 pathology.

PMID:
18549326
PMCID:
PMC2855963
DOI:
10.1517/14712598.8.7.969
[Indexed for MEDLINE]
Free PMC Article

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