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000165352 041__ $$aEnglish
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000165352 1001_ $$00000-0003-4731-5002$$aBauer, Susanne$$b0
000165352 245__ $$aTranslatome profiling in fatal familial insomnia implicates TOR signaling in somatostatin neurons.
000165352 260__ $$aHeidelberg$$bEMBO Press$$c2022
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000165352 520__ $$aSelective neuronal vulnerability is common in neurodegenerative diseases but poorly understood. In genetic prion diseases, including fatal familial insomnia (FFI) and Creutzfeldt-Jakob disease (CJD), different mutations in the Prnp gene manifest as clinically and neuropathologically distinct diseases. Here we report with electroencephalography studies that theta waves are mildly increased in 21 mo old knock-in mice modeling FFI and CJD and that sleep is mildy affected in FFI mice. To define affected cell types, we analyzed cell type-specific translatomes from six neuron types of 9 mo old FFI and CJD mice. Somatostatin (SST) neurons responded the strongest in both diseases, with unexpectedly high overlap in genes and pathways. Functional analyses revealed up-regulation of neurodegenerative disease pathways and ribosome and mitochondria biogenesis, and down-regulation of synaptic function and small GTPase-mediated signaling in FFI, implicating down-regulation of mTOR signaling as the root of these changes. In contrast, responses in glutamatergic cerebellar neurons were disease-specific. The high similarity in SST neurons of FFI and CJD mice suggests that a common therapy may be beneficial for multiple genetic prion diseases.
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000165352 650_7 $$051110-01-1$$2NLM Chemicals$$aSomatostatin
000165352 650_7 $$0EC 2.7.11.1$$2NLM Chemicals$$aTOR Serine-Threonine Kinases
000165352 650_7 $$0EC 3.6.5.2$$2NLM Chemicals$$aMonomeric GTP-Binding Proteins
000165352 650_2 $$2MeSH$$aAnimals
000165352 650_2 $$2MeSH$$aCreutzfeldt-Jakob Syndrome: genetics
000165352 650_2 $$2MeSH$$aInsomnia, Fatal Familial: genetics
000165352 650_2 $$2MeSH$$aMice
000165352 650_2 $$2MeSH$$aMonomeric GTP-Binding Proteins: metabolism
000165352 650_2 $$2MeSH$$aNeurodegenerative Diseases
000165352 650_2 $$2MeSH$$aNeurons: metabolism
000165352 650_2 $$2MeSH$$aPrion Diseases: genetics
000165352 650_2 $$2MeSH$$aSomatostatin: genetics
000165352 650_2 $$2MeSH$$aSomatostatin: metabolism
000165352 650_2 $$2MeSH$$aTOR Serine-Threonine Kinases: genetics
000165352 650_2 $$2MeSH$$aTOR Serine-Threonine Kinases: metabolism
000165352 7001_ $$0P:(DE-2719)2811120$$aDittrich, Lars$$b1$$udzne
000165352 7001_ $$0P:(DE-2719)2810486$$aKaczmarczyk, Lech$$b2$$udzne
000165352 7001_ $$0P:(DE-2719)2810344$$aSchleif, Melvin$$b3$$udzne
000165352 7001_ $$aBenfeitas, Rui$$b4
000165352 7001_ $$0P:(DE-2719)2810253$$aJackson, Walker Scot$$b5$$eLast author$$udzne
000165352 773__ $$0PERI:(DE-600)2948687-7$$a10.26508/lsa.202201530$$gVol. 5, no. 11, p. e202201530 -$$n11$$pe202201530$$tLife science alliance$$v5$$x2575-1077$$y2022
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