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Mutant huntingtin impairs axonal trafficking in mammalian neurons in vivo and in vitro

Trushina Eugenia, Dyer Roy B., Badger John D., Ure Daren, Eide Lars, Tran David D., Vrieze Brent T., Legendre-Guillemin Valérie, McPherson Peter S., Mandavilli Bhaskar S., Van Houten Bennett, Zeitlin Scott, McNiven Mark, Aebersold Ruedi, Hayden Michael R., Parisi Joseph E., Seeberg Erling, Dragatsis Ioannis, Doyle Kelly, Bender Anna, Chacko Celin et McMurray Cynthia T.. (2004). Mutant huntingtin impairs axonal trafficking in mammalian neurons in vivo and in vitro. Molecular and Cellular Biology, 24, (18), p. 8195-8209.

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URL officielle: http://dx.doi.org/doi:10.1128/MCB.24.18.8195-8209....

Résumé

Recent data in invertebrates demonstrated that huntingtin (htt) is essential for fast axonal trafficking. Here, we provide direct and functional evidence that htt is involved in fast axonal trafficking in mammals. Moreover, expression of full-length mutant htt (mhtt) impairs vesicular and mitochondrial trafficking in mammalian neurons in vitro and in whole animals in vivo. Particularly, mitochondria become progressively immobilized and stop more frequently in neurons from transgenic animals. These defects occurred early in development prior to the onset of measurable neurological or mitochondrial abnormalities. Consistent with a progressive loss of function, wild-type htt, trafficking motors, and mitochondrial components were selectively sequestered by mhtt in human Huntington's disease-affected brain. Data provide a model for how loss of htt function causes toxicity; mhtt-mediated aggregation sequesters htt and components of trafficking machinery leading to loss of mitochondrial motility and eventual mitochondrial dysfunction.

Type de document:Article publié dans une revue avec comité d'évaluation
ISSN:0270-7306
Volume:24
Numéro:18
Pages:p. 8195-8209
Version évaluée par les pairs:Oui
Date:2004
Sujets:Sciences de la santé > Sciences médicales > Biochimie
Sciences de la santé > Sciences médicales > Biologie cellulaire
Sciences de la santé > Sciences médicales > Biologie moléculaire
Département, module, service et unité de recherche:Départements et modules > Département des sciences fondamentales
Mots-clés:Huntington disease, mutation, axonal transport, nerve tissue proteins, neurons
Déposé le:02 juin 2016 18:05
Dernière modification:05 déc. 2016 20:47
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