Does Huntingtin play a role in selective macroautophagy?

The accumulation of protein aggregates in neurons appears to be a basic feature of neurodegenerative disease. In Huntington's Disease, a progressive and ultimately fatal neurodegenerative disorder caused by an expansion of the polyglutamine repeat within the protein Huntingtin (Htt), the immedi...

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Published inCell cycle (Georgetown, Tex.) Vol. 9; no. 17; pp. 3401 - 3413
Main Author Steffan, Joan S.
Format Journal Article
LanguageEnglish
Published United States Taylor & Francis 01.09.2010
Landes Bioscience
Subjects
Autophagy
Binding
Biology
Bioscience
Calcium
Cancer
Cell
Cycle
Extra View
Humans
Huntingtin Protein
Huntington Disease - metabolism
I-kappa B Kinase - metabolism
Landes
Nerve Tissue Proteins - metabolism
Nerve Tissue Proteins - physiology
Nuclear Proteins - metabolism
Nuclear Proteins - physiology
Organogenesis
Peptides - metabolism
Phosphorylation
Proteins
Saccharomyces cerevisiae - enzymology
Saccharomyces cerevisiae - metabolism
Saccharomyces cerevisiae Proteins - genetics
Saccharomyces cerevisiae Proteins - metabolism
Ubiquitin-Activating Enzymes - genetics
Ubiquitin-Activating Enzymes - metabolism
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Summary:The accumulation of protein aggregates in neurons appears to be a basic feature of neurodegenerative disease. In Huntington's Disease, a progressive and ultimately fatal neurodegenerative disorder caused by an expansion of the polyglutamine repeat within the protein Huntingtin (Htt), the immediate proximal cause of disease is well understood. However, the cellular mechanisms which modulate the rate at which fragments of Htt containing polyglutamine accumulate in neurons is a central issue in the development of approaches to modulate the rate and extent of neuronal loss in this disease.
ISSN:1538-4101
1551-4005
DOI:10.4161/cc.9.17.12718