Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines

Panov, Alexander V.; Gutekunst, Claire-Anne; Leavitt, Blair R.; Hayden, Michael R.; Burke, James R.; Strittmatter, Warren J.; Greenamyre, J. Timothy

doi:10.1038/nn884

Cited by 1,030Genetic Neurodegenerative DiseasesMitochondrial Function and PathologyHereditary Neurological Disorders

Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines

Panov, Alexander V., Gutekunst, Claire-Anne, Leavitt, Blair R., Hayden, Michael R., Burke, James R., Strittmatter, Warren J., Greenamyre, J. Timothy·2002·Nature Neuroscience

DOI 10.1038/nn884

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Abstract

Huntington's disease (HD) is caused by an expansion of exonic CAG triplet repeats in the gene encoding huntingtin protein (Htt), but the mechanisms by which this mutant protein causes neurodegeneration remain unknown. Here we show that lymphoblast mitochondria from patients with HD have a lower membrane potential and depolarize at lower calcium loads than do mitochondria from controls. We found a similar defect in brain mitochondria from transgenic mice expressing full-length mutant huntingtin, and this defect preceded the onset of pathological or behavioral abnormalities by months. By electron microscopy, we identified N-terminal mutant huntingtin on neuronal mitochondrial membranes, and by incubating normal mitochondria with a fusion protein containing an abnormally long polyglutamine repeat, we reproduced the mitochondrial calcium defect seen in human patients and transgenic animals. Thus, mitochondrial calcium abnormalities occur early in HD pathogenesis and may be a direct effect of mutant huntingtin on the organelle.

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Mutant Htt associates directly with the outer mitochondrial membrane.
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Authors: Panov, A. V., Gutekunst, C.-A., Leavitt, B. R., Hayden, M. R., Burke, J. R., Strittmatter, W. J., & Greenamyre, J. T.
Affiliations: Emory University (US)
University of British Columbia (CA)
Duke Medical Center (US)
Corresponding author: Greenamyre, J. Timothy
ORCID: Panov, Alexander V.
Gutekunst, Claire-Anne
Leavitt, Blair R.
Hayden, Michael R.
Burke, James R.
Greenamyre, J. Timothy
Venue: Nature Neuroscience
Publisher: Springer Science and Business Media LLC
Published: 2002-07-01
Volume: 5
Issue: 8
Pages: 731-736
ISSN: 1097-6256 (ISSN-L)
1546-1726
DOI: 10.1038/nn884
OpenAlex: W1972771185
Cited by: 1,030
References: 35
Citations by year: 2026: 16 · 2025: 23 · 2024: 26 · 2023: 27 · 2022: 30 · 2021: 49 · 2020: 29 · 2019: 35 · 2018: 39 · 2017: 23 · 2016: 45 · 2015: 35 · 2014: 48 · 2013: 33 · 2012: 68
FWCI: 19.24
Type: journal article
Language: en
Open access: closed
Licence: www.springer.com/tdm (tdm)
Locations: Nature Neuroscience (publishedVersion)
PubMed (publishedVersion)
Funders: National Institutes of Health (5r01ag014648-05)
National Institute on Aging (AG14648)
Topics: Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Genetic Neurodegenerative Diseases
Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Hereditary Neurological Disorders
Keywords: Huntington's disease, mitochondrial calcium handling, polyglutamine expansion, mutant huntingtin, mitochondrial membrane potential, mitochondrial depolarization, neurodegeneration, transgenic mice, early pathogenesis
MeSH terms: Huntingtin Protein, Animals, Brain, Brain Chemistry, Calcium, Cell Line, Disease Models, Animal, Humans, Huntington Disease, Intracellular Membranes, Lymphocytes, Membrane Potentials, Mice, Transgenic, Microscopy, Electron, Mitochondria, Nerve Tissue Proteins, Nuclear Proteins, Recombinant Fusion Proteins, Trinucleotide Repeat Expansion, Mice

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Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines

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