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001     165352
005     20230915090618.0
024 7 _ |a 10.26508/lsa.202201530
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037 _ _ |a DZNE-2022-01629
041 _ _ |a English
082 _ _ |a 570
100 1 _ |a Bauer, Susanne
|0 0000-0003-4731-5002
|b 0
245 _ _ |a Translatome profiling in fatal familial insomnia implicates TOR signaling in somatostatin neurons.
260 _ _ |a Heidelberg
|c 2022
|b EMBO Press
336 7 _ |a article
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520 _ _ |a Selective neuronal vulnerability is common in neurodegenerative diseases but poorly understood. In genetic prion diseases, including fatal familial insomnia (FFI) and Creutzfeldt-Jakob disease (CJD), different mutations in the Prnp gene manifest as clinically and neuropathologically distinct diseases. Here we report with electroencephalography studies that theta waves are mildly increased in 21 mo old knock-in mice modeling FFI and CJD and that sleep is mildy affected in FFI mice. To define affected cell types, we analyzed cell type-specific translatomes from six neuron types of 9 mo old FFI and CJD mice. Somatostatin (SST) neurons responded the strongest in both diseases, with unexpectedly high overlap in genes and pathways. Functional analyses revealed up-regulation of neurodegenerative disease pathways and ribosome and mitochondria biogenesis, and down-regulation of synaptic function and small GTPase-mediated signaling in FFI, implicating down-regulation of mTOR signaling as the root of these changes. In contrast, responses in glutamatergic cerebellar neurons were disease-specific. The high similarity in SST neurons of FFI and CJD mice suggests that a common therapy may be beneficial for multiple genetic prion diseases.
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650 _ 7 |a Somatostatin
|0 51110-01-1
|2 NLM Chemicals
650 _ 7 |a TOR Serine-Threonine Kinases
|0 EC 2.7.11.1
|2 NLM Chemicals
650 _ 7 |a Monomeric GTP-Binding Proteins
|0 EC 3.6.5.2
|2 NLM Chemicals
650 _ 2 |a Animals
|2 MeSH
650 _ 2 |a Creutzfeldt-Jakob Syndrome: genetics
|2 MeSH
650 _ 2 |a Insomnia, Fatal Familial: genetics
|2 MeSH
650 _ 2 |a Mice
|2 MeSH
650 _ 2 |a Monomeric GTP-Binding Proteins: metabolism
|2 MeSH
650 _ 2 |a Neurodegenerative Diseases
|2 MeSH
650 _ 2 |a Neurons: metabolism
|2 MeSH
650 _ 2 |a Prion Diseases: genetics
|2 MeSH
650 _ 2 |a Somatostatin: genetics
|2 MeSH
650 _ 2 |a Somatostatin: metabolism
|2 MeSH
650 _ 2 |a TOR Serine-Threonine Kinases: genetics
|2 MeSH
650 _ 2 |a TOR Serine-Threonine Kinases: metabolism
|2 MeSH
700 1 _ |a Dittrich, Lars
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700 1 _ |a Kaczmarczyk, Lech
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700 1 _ |a Schleif, Melvin
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700 1 _ |a Benfeitas, Rui
|b 4
700 1 _ |a Jackson, Walker Scot
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773 _ _ |a 10.26508/lsa.202201530
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910 1 _ |a Deutsches Zentrum für Neurodegenerative Erkrankungen
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