TDP-43 and FUS: a nuclear affair

Publication type:
Journal article
Metadata:
Autoren
  • Dorothee Dormann
  • Christian Haass
Autoren-URL
https://www.webofscience.com/api/gateway?GWVersion=2&SrcApp=fis-test-1&SrcAuth=WosAPI&KeyUT=WOS:000293206200001&DestLinkType=FullRecord&DestApp=WOS_CPL
DOI
10.1016/j.tins.2011.05.002
eISSN
1878-108X
Externe Identifier
  • Clarivate Analytics Document Solution ID: 798KZ
  • PubMed Identifier: 21700347
ISSN
0166-2236
Ausgabe der Veröffentlichung
7
Zeitschrift
TRENDS IN NEUROSCIENCES
Paginierung
339 - 348
Datum der Veröffentlichung
2011
Status
Published
Titel
TDP-43 and FUS: a nuclear affair
Sub types
  • Review
Ausgabe der Zeitschrift
34

Data source: Web of Science (Lite)

Other metadata sources:
Autoren
  • Dorothee Dormann
  • Christian Haass
DOI
10.1016/j.tins.2011.05.002
ISSN
0166-2236
Ausgabe der Veröffentlichung
7
Zeitschrift
Trends in Neurosciences
Sprache
en
Paginierung
339 - 348
Datum der Veröffentlichung
2011
Status
Published
Herausgeber
Elsevier BV
Herausgeber URL
http://dx.doi.org/10.1016/j.tins.2011.05.002
Datum der Datenerfassung
2024
Titel
TDP-43 and FUS: a nuclear affair
Ausgabe der Zeitschrift
34

Data source: Crossref

Abstract
Misfolded TAR DNA binding protein 43 (TDP-43) and Fused-In-Sarcoma (FUS) protein have recently been identified as pathological hallmarks of the neurodegenerative disorders amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) characterized by the presence of ubiquitin-positive inclusions (FTLD-U). Although TDP-43 and FUS are normally located predominantly in the nucleus, pathological TDP-43 and FUS inclusions are mostly found in the cytosol. Cytosolic deposition is paralleled by a striking nuclear depletion of either protein. Based on a number of recent findings, we postulate that defects in nuclear import are an important step towards TDP-43 and FUS dysfunction. Failure of nuclear transport can arise from mutations within a nuclear localization signal or from age-related decline of nuclear import mechanisms. We propose that nuclear import defects in combination with additional hits, for example cellular stress and genetic risk factors, may be a central underlying cause of ALS and FTLD-U pathology.
Addresses
  • Adolf-Butenandt-Institute, Biochemistry, Ludwig-Maximilians-University and German Center for Neurodegenerative Diseases (DZNE) Munich, Schillerstr. 44, 80336 Munich, Germany.
Autoren
  • Dorothee Dormann
  • Christian Haass
DOI
10.1016/j.tins.2011.05.002
eISSN
1878-108X
Externe Identifier
  • PubMed Identifier: 21700347
Open access
false
ISSN
0166-2236
Ausgabe der Veröffentlichung
7
Zeitschrift
Trends in neurosciences
Schlüsselwörter
  • Inclusion Bodies
  • Cell Nucleus
  • Animals
  • Humans
  • Amyotrophic Lateral Sclerosis
  • RNA-Binding Protein FUS
  • DNA-Binding Proteins
  • Active Transport, Cell Nucleus
  • Protein Transport
  • Frontotemporal Lobar Degeneration
Sprache
eng
Medium
Print-Electronic
Online publication date
2011
Paginierung
339 - 348
Datum der Veröffentlichung
2011
Status
Published
Datum der Datenerfassung
2011
Titel
TDP-43 and FUS: a nuclear affair.
Sub types
  • Research Support, Non-U.S. Gov't
  • Journal Article
Ausgabe der Zeitschrift
34

Data source: Europe PubMed Central

Abstract
Misfolded TAR DNA binding protein 43 (TDP-43) and Fused-In-Sarcoma (FUS) protein have recently been identified as pathological hallmarks of the neurodegenerative disorders amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) characterized by the presence of ubiquitin-positive inclusions (FTLD-U). Although TDP-43 and FUS are normally located predominantly in the nucleus, pathological TDP-43 and FUS inclusions are mostly found in the cytosol. Cytosolic deposition is paralleled by a striking nuclear depletion of either protein. Based on a number of recent findings, we postulate that defects in nuclear import are an important step towards TDP-43 and FUS dysfunction. Failure of nuclear transport can arise from mutations within a nuclear localization signal or from age-related decline of nuclear import mechanisms. We propose that nuclear import defects in combination with additional hits, for example cellular stress and genetic risk factors, may be a central underlying cause of ALS and FTLD-U pathology.
Date of acceptance
2011
Autoren
  • Dorothee Dormann
  • Christian Haass
Autoren-URL
https://www.ncbi.nlm.nih.gov/pubmed/21700347
DOI
10.1016/j.tins.2011.05.002
eISSN
1878-108X
Ausgabe der Veröffentlichung
7
Zeitschrift
Trends Neurosci
Schlüsselwörter
  • Active Transport, Cell Nucleus
  • Amyotrophic Lateral Sclerosis
  • Animals
  • Cell Nucleus
  • DNA-Binding Proteins
  • Frontotemporal Lobar Degeneration
  • Humans
  • Inclusion Bodies
  • Protein Transport
  • RNA-Binding Protein FUS
Sprache
eng
Country
England
Paginierung
339 - 348
PII
S0166-2236(11)00073-7
Datum der Veröffentlichung
2011
Status
Published
Datum, an dem der Datensatz öffentlich gemacht wurde
2014
Titel
TDP-43 and FUS: a nuclear affair.
Sub types
  • Journal Article
  • Research Support, Non-U.S. Gov't
Ausgabe der Zeitschrift
34

Data source: PubMed

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