ArticleBrain pathology (Zurich, Switzerland)2017
Fatal familial insomnia: mitochondrial and protein synthesis machinery decline in the mediodorsal thalamus.
Article in Brain pathology (Zurich, Switzerland), 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers.
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Who cites it
20 citing papers in PubMed, 33 citations in OpenAlex.
- Insomnia in Forensic Detainees: Is Salience Network the Common Pathway for Sleep, Neuropsychiatric, and Neurodegenerative Disorders?Journal of clinical medicine · 2024Review
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- Altered energy metabolism in Fatal Familial Insomnia cerebral organoids is associated with astrogliosis and neuronal dysfunction.PLoS genetics · 2023Article
- The eEF1 family of mammalian translation elongation factors.BBA advances · 2023Article
- Mitochondria as the Essence of Yang Qi in the Human Body.Phenomics (Cham, Switzerland) · 2022Review
- Early Stages of RNA-Mediated Conversion of Human Prions.The journal of physical chemistry. B · 2022Article
- Distinct translatome changes in specific neural populations precede electroencephalographic changes in prion-infected mice.PLoS pathogens · 2022Article
- Astrocyte in prion disease: a double-edged sword.Neural regeneration research · 2022Review
- Ginsenoside Rg1 Ameliorates Acute Renal Ischemia/Reperfusion Injury via Upregulating AMPKOxidative medicine and cellular longevity · 2022Article
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- Reduced SOD2 expression does not influence prion disease course or pathology in mice.PloS one · 2021Article
- Alterations of Subcortical Brain Structures in Paradoxical and Psychophysiological Insomnia Disorder.Frontiers in psychiatry · 2021Article
- Effects of Insomnia on Peptic Ulcer Disease Using Mendelian Randomization.Oxidative medicine and cellular longevity · 2021Article
- Combinatory FK506 and Minocycline Treatment Alleviates Prion-Induced Neurodegenerative Events via Caspase-Mediated MAPK-NRF2 Pathway.International journal of molecular sciences · 2019Article
- Recent advances in the histo-molecular pathology of human prion disease.Brain pathology (Zurich, Switzerland) · 2019Review
- Mutations Alter RNA-Mediated Conversion of Human Prions.ACS omega · 2018Article
- p62-Keap1-NRF2-ARE Pathway: A Contentious Player for Selective Targeting of Autophagy, Oxidative Stress and Mitochondrial Dysfunction in Prion Diseases.Frontiers in molecular neuroscience · 2018Review
- Sisyphus in Neverland.Journal of Alzheimer's disease : JAD · 2018Article
- Agrypnia excitata and obstructive apnea in a patient with fatal familial insomnia from China: A case report.Medicine · 2017Article
- Losing sleep over mitochondria: a new player in the pathophysiology of fatal familial insomnia.Brain pathology (Zurich, Switzerland) · 2017Article
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10 authors at 6 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
The expression of subunits of mitochondrial respiratory complexes and components of the protein synthesis machinery from the nucleolus to the ribosome was analyzed in the mediodorsal thalamus in seven cases of fatal familial insomnia (FFI) compared with age-matched controls. NDUFB8 (complex I subunit), SDHB (complex II subunit), UQCRC2 (complex III subunit), COX2 (complex IV subunit), and ATP50 (complex V subunit) expression levels, as revealed by western blotting, were reduced in FFI. Voltage-dependent anion channel (VDAC) and ATP5H were also reduced due to the marked depopulation of neurons. In contrast, a marked increase in superoxide dismutase 2 (SOD2) was found in reactive astrocytes thus suggesting that astrocytes are key factors in oxidative stress responses. The histone-binding chaperones nucleolin and nucleoplasmin 3, and histone H3 di-methylated K9 were markedly reduced together with a decrease in the expression of protein transcription elongation factor eEF1A. These findings show severe impairment in the expression of crucial components of mitochondrial function and protein synthesis in parallel with neuron loss in mediodorsal thalamus at terminal stages of FFI. Therapeutic measures must be taken long before the appearance of clinical symptoms to prevent the devastating effects of FFI.
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