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Cited by 555

The DOI matches its Crossref record; 8 of the 8 references it cites were found in a registry or an index.

Mutant huntingtin binds the mitochondrial fission GTPase dynamin-related protein-1 and increases its enzymatic activity

Open access
Song, Wenjun, Chen, Jin, Petrilli, Alejandra, Liot, Geraldine, Klinglmayr, Eva, Zhou, Yue, Poquiz, Patrick, Tjong, Jonathan, Pouladi, Mahmoud A, Hayden, Michael R, Masliah, Eliezer, Ellisman, Mark, Rouiller, Isabelle, Schwarzenbacher, Robert, Bossy, Blaise, Perkins, Guy, Bossy-Wetzel, Ella·2011·Nature Medicine
DOI 10.1038/nm.2313
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Abstract

Huntington's disease is an inherited and incurable neurodegenerative disorder caused by an abnormal polyglutamine (polyQ) expansion in huntingtin (encoded by HTT). PolyQ length determines disease onset and severity, with a longer expansion causing earlier onset. The mechanisms of mutant huntingtin-mediated neurotoxicity remain unclear; however, mitochondrial dysfunction is a key event in Huntington's disease pathogenesis. Here we tested whether mutant huntingtin impairs the mitochondrial fission-fusion balance and thereby causes neuronal injury. We show that mutant huntingtin triggers mitochondrial fragmentation in rat neurons and fibroblasts of individuals with Huntington's disease in vitro and in a mouse model of Huntington's disease in vivo before the presence of neurological deficits and huntingtin aggregates. Mutant huntingtin abnormally interacts with the mitochondrial fission GTPase dynamin-related protein-1 (DRP1) in mice and humans with Huntington's disease, which, in turn, stimulates its enzymatic activity. Mutant huntingtin–mediated mitochondrial fragmentation, defects in anterograde and retrograde mitochondrial transport and neuronal cell death are all rescued by reducing DRP1 GTPase activity with the dominant-negative DRP1 K38A mutant. Thus, DRP1 might represent a new therapeutic target to combat neurodegeneration in Huntington's disease.

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Metadata

Authors
Song, W., Chen, J., Petrilli, A., Liot, G., Klinglmayr, E., Zhou, Y., Poquiz, P., Tjong, J., Pouladi, M. A., Hayden, M. R., Masliah, E., Ellisman, M., Rouiller, I., Schwarzenbacher, R., Bossy, B., Perkins, G., & Bossy-Wetzel, E.
Venue
Nature Medicine
Publisher
Springer Science and Business Media LLC
Published
2011-02-20
Volume
17
Issue
3
Pages
377-382
ISSN
  • 1078-8956 (ISSN-L)
  • 1546-170X
DOI
10.1038/nm.2313
OpenAlex
W2008584420
Cited by
555
References
8
FWCI
17.39
Type
journal article
Language
en
Open access
green

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