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Margulis J, Finkbeiner S. Proteostasis in striatal cells and selective neurodegeneration in Huntington's disease. Front Cell Neurosci. 2014;8:218doi: 10.3389/fncel.2014.00218.
Margulis, J., & Finkbeiner, S. (2014). Proteostasis in striatal cells and selective neurodegeneration in Huntington's disease. Frontiers in cellular neuroscience, 8218. https://doi.org/10.3389/fncel.2014.00218
Margulis, Julia, and Finkbeiner, Steven. "Proteostasis in striatal cells and selective neurodegeneration in Huntington's disease." Frontiers in cellular neuroscience vol. 8 (2014): 218. doi: https://doi.org/10.3389/fncel.2014.00218
Margulis J, Finkbeiner S. Proteostasis in striatal cells and selective neurodegeneration in Huntington's disease. Front Cell Neurosci. 2014 Aug 07;8:218. doi: 10.3389/fncel.2014.00218. eCollection 2014. PMID: 25147502; PMCID: PMC4124811.
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Front Cell Neurosci. 2014 Aug 07;8:218. doi: 10.3389/fncel.2014.00218. eCollection 2014.

Proteostasis in striatal cells and selective neurodegeneration in Huntington's disease.

Frontiers in cellular neuroscience

Julia Margulis, Steven Finkbeiner

Affiliations

  1. Gladstone Institute of Neurological Disease, J. David Gladstone Institutes San Francisco, CA, USA ; Department of Neurology, University of California at San Francisco San Francisco, CA, USA ; Department of Physiology, University of California at San Francisco San Francisco, CA, USA.
  2. Gladstone Institute of Neurological Disease, J. David Gladstone Institutes San Francisco, CA, USA ; Department of Neurology, University of California at San Francisco San Francisco, CA, USA ; Department of Physiology, University of California at San Francisco San Francisco, CA, USA ; Taube/Koret Center for Huntington's Disease Research San Francisco, CA, USA.

PMID: 25147502 PMCID: PMC4124811 DOI: 10.3389/fncel.2014.00218

Abstract

Selective neuronal loss is a hallmark of neurodegenerative diseases, including Huntington's disease (HD). Although mutant huntingtin, the protein responsible for HD, is expressed ubiquitously, a subpopulation of neurons in the striatum is the first to succumb. In this review, we examine evidence that protein quality control pathways, including the ubiquitin proteasome system, autophagy, and chaperones, are significantly altered in striatal neurons. These alterations may increase the susceptibility of striatal neurons to mutant huntingtin-mediated toxicity. This novel view of HD pathogenesis has profound therapeutic implications: protein homeostasis pathways in the striatum may be valuable targets for treating HD and other misfolded protein disorders.

Keywords: Huntington’s disease; autophagy; proteasome; proteostasis; striatum

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