TDP-43 in neurodegenerative disorders

Research output: Contribution to journalArticle

18 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)969-978
Number of pages10
JournalExpert Opinion on Biological Therapy
Volume8
Issue number7
DOIs
StatePublished - Jul 2008

Fingerprint

DNA-Binding Proteins
Neurodegenerative Diseases
Pathology
Mutation
Neurodegenerative diseases
Response Elements
Research
Solubility
Transcriptional Activation
Brain
Tissue

Keywords

  • Amyotropic lateral sclerosis
  • Frontotemporal dementia
  • Progranulin
  • TAR DNA binding protein-43 (TDP-43)
  • Valosin-containing protein

ASJC Scopus subject areas

  • Pharmacology
  • Biochemistry, Genetics and Molecular Biology(all)
  • Genetics
  • Immunology

Cite this

TDP-43 in neurodegenerative disorders. / Cook, Casey; Zhang, Yongjie; Xu, Ya Fei; Dickson, Dennis W; Petrucelli, Leonard.

In: Expert Opinion on Biological Therapy, Vol. 8, No. 7, 07.2008, p. 969-978.

Research output: Contribution to journalArticle

@article{082ae9e63a8646df9436b87452b80129,
title = "TDP-43 in neurodegenerative disorders",
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.",
keywords = "Amyotropic lateral sclerosis, Frontotemporal dementia, Progranulin, TAR DNA binding protein-43 (TDP-43), Valosin-containing protein",
author = "Casey Cook and Yongjie Zhang and Xu, {Ya Fei} and Dickson, {Dennis W} and Leonard Petrucelli",
year = "2008",
month = "7",
doi = "10.1517/14712598.8.7.969",
language = "English (US)",
volume = "8",
pages = "969--978",
journal = "Expert Opinion on Biological Therapy",
issn = "1471-2598",
publisher = "Informa Healthcare",
number = "7",

}

TY - JOUR

T1 - TDP-43 in neurodegenerative disorders

AU - Cook, Casey

AU - Zhang, Yongjie

AU - Xu, Ya Fei

AU - Dickson, Dennis W

AU - Petrucelli, Leonard

PY - 2008/7

Y1 - 2008/7

N2 - 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.

AB - 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.

KW - Amyotropic lateral sclerosis

KW - Frontotemporal dementia

KW - Progranulin

KW - TAR DNA binding protein-43 (TDP-43)

KW - Valosin-containing protein

UR - http://www.scopus.com/inward/record.url?scp=47549085411&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=47549085411&partnerID=8YFLogxK

U2 - 10.1517/14712598.8.7.969

DO - 10.1517/14712598.8.7.969

M3 - Article

C2 - 18549326

AN - SCOPUS:47549085411

VL - 8

SP - 969

EP - 978

JO - Expert Opinion on Biological Therapy

JF - Expert Opinion on Biological Therapy

SN - 1471-2598

IS - 7

ER -