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PMID: 19269181 Published · ppublish English Journal Article Research Support, N.I.H., Extramural Review

Huntingtin as an essential integrator of intracellular vesicular trafficking.

Trends in cell biology ·Vol. 19 ·No. 4 ·2009-04-00 ·Pages 147-55

Caviston JP, Holzbaur EL

Abstract

The neurodegenerative disorder Huntington's disease is caused by an expansion in the polyglutamine repeat region of the protein huntingtin. Multiple studies in cellular and animal model systems indicate that this mutation imparts a novel toxic function required for disease pathogenesis. However, the normal function of huntingtin, an essential cellular protein in higher vertebrates, is not yet well understood. Emerging data indicate an important role for wild-type huntingtin in the intracellular transport of vesicles and organelles. Here, we discuss current progress on the role of huntingtin in vesicular trafficking, focusing on the proposal that huntingtin might be a crucial regulator of organelle transport along the cellular cytoskeleton.

MeSH Terms
Animals Biological Transport Carrier Proteins/physiology Cytoplasmic Vesicles/physiology Cytoskeleton/physiology Dyneins/physiology Humans Huntingtin Protein Huntington Disease/genetics,metabolism Nerve Tissue Proteins/genetics,metabolism Nuclear Proteins/genetics,metabolism
Chemicals
Carrier Proteins HTT protein, human Htt protein, mouse Huntingtin Protein Nerve Tissue Proteins Nuclear Proteins Dyneins
Authors & Affiliations
2 authors, click to expand affiliations / ORCID
Caviston Juliane P
Department of Physiology, University of Pennsylvania School of Medicine, Philadelphia, 19104-6085, USA.
Holzbaur Erika L F
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Article Info
Journal
Trends in cell biology
Abbr.
Trends Cell Biol
ISSN
1879-3088
DOI
10.1016/j.tcb.2009.01.005
Published
2009-04-00
Epub
2009-00-05
Pages
147-55
Language
English
Region
England
NLM ID
9200566
PMCID
PMC2930405
Subset
IM
Grants
NIGMS NIH HHS · R01 GM048661 · United States
NIGMS NIH HHS · R01 GM048661-17 · United States
NIGMS NIH HHS · GM48661 · United States
External Links
PubMed source DOI: 10.1016/j.tcb.2009.01.005 PMC Full Text View journal details
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