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

By A. Panov, C. Gutekunst, B. Leavitt, Michael R. Hayden, J. Burke, W. Strittmatter, J. Greenamyre. Nature Neuroscience, 2002

1,067 citations recorded by the source corpus.

Subfields: Cellular and Molecular Neuroscience, Molecular Biology, Neurology

View Paper: https://www.semanticscholar.org/paper/b74144440315ad18c71bd63be46c7cb6c0f915ca

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Citations in the corpus

Oxidative Stress in Neurodegenerative Diseases: From Molecular Mechanisms to Clinical Applications (2017, background): “In addition, mHtt has direct inhibitory effects on respiratory complex II [76, 77].”
Molecular mechanisms and potential therapeutical targets in Huntington's disease. (2010, background): “Although HD mice are born with no apparent defects, a recent study from Mark Mehler’s group at the Albert Einstein College of Medicine in New York (398) suggests that developmental abnormalities occur in a knock-in mouse model of HD (HdhQ111) compared with a knock-in mouse model expressing only 18 C”
Mitochondria take center stage in aging and neurodegeneration (2005, background): “In mitochondria isolated from YAC transgenic mice expressing full-length huntingtin containing 72 CAG repeats, there is reduced capacity for calcium uptake and an increased susceptibility to activation of the MPT.(76) This abnormality was mimicked by incubating isolated brain mitochondria with prote”
Variable tandem repeats accelerate evolution of coding and regulatory sequences. (2010, background): “Decreased axonal transport of BDNF-containing vesicles and altered Ca+2 signaling at the mitochondrial membrane also contribute to pathogenesis (40, 97).”
Measuring in vivo mitophagy (2015, background): “Evidence suggests that in Huntington’s disease, mutant Huntingtin protein has a direct, deleterious effect on mitochondrial function, perhaps by impairing mitochondrial calcium homeostasis (Panov et al., 2002; Zuccato et al., 2010).”
A Mitochondrial Paradigm of Metabolic and Degenerative Diseases, Aging, and Cancer: A Dawn for Evolutionary Medicine (2005, background): “Studies on Huntington disease have implicated a mitochondrial dysfunction in its etiology as well (172, 195, 241), and mtDNA rearrangements are elevated in the basal ganglia of patients with Huntington disease (76).”
Oxidative Stress, Mitochondrial Dysfunction, and Aging (2011, background): “In addition, expression of mutant HTT leads to impaired energy metabolism, abnormal Ca2+ signaling and mitochondrial membrane potential, and drastic changes in mitochondrial ultrastructures [191, 192].”
Synaptic energy use and supply. (2012, neutral): “Disrupted mitochondrial Ca2+ buffering (Panov et al., 2002) may pose a further problem at synapses, making neurons more susceptible to excitotoxicity upon mhtt-enhanced or even normal activation of NMDA receptors (Fan and Raymond, 2007).”
Diseases of Unstable Repeat Expansion: Mechanisms and Common Principles (2005, background): “Morphological and functional data support a role for mitochondrial dysfunction in polyglutamine disease”
Neuronal calcium signaling: function and dysfunction (2014, background): “direct interaction of mutant Htt with mitochondria [165],”
Molecular pathways to neurodegeneration (2004, background): “Htt is reported in membranes, cytosol, nuclei, and mitochondria, and in association with microtubule”
Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease. (2003, background): “These mice have helped elucidate different pathways involved in the pathogenesis of HD including increased susceptibility to excitotoxic cell death of neurons with mutant huntingtin (11–13), the discovery of decreased BDNF production in HD (14), the presence of mitochondrial dysfunction (15), and th”