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000163450 037__ $$aDZNE-2022-00210
000163450 041__ $$aEnglish
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000163450 1001_ $$00000-0003-4804-7716$$aKettwig, Matthias$$b0
000163450 245__ $$aInterferon-driven brain phenotype in a mouse model of RNaseT2 deficient leukoencephalopathy.
000163450 260__ $$a[London]$$bNature Publishing Group UK$$c2021
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000163450 520__ $$aInfantile-onset RNaseT2 deficient leukoencephalopathy is characterised by cystic brain lesions, multifocal white matter alterations, cerebral atrophy, and severe psychomotor impairment. The phenotype is similar to congenital cytomegalovirus brain infection and overlaps with type I interferonopathies, suggesting a role for innate immunity in its pathophysiology. To date, pathophysiological studies have been hindered by the lack of mouse models recapitulating the neuroinflammatory encephalopathy found in patients. In this study, we generated Rnaset2-/- mice using CRISPR/Cas9-mediated genome editing. Rnaset2-/- mice demonstrate upregulation of interferon-stimulated genes and concurrent IFNAR1-dependent neuroinflammation, with infiltration of CD8+ effector memory T cells and inflammatory monocytes into the grey and white matter. Single nuclei RNA sequencing reveals homeostatic dysfunctions in glial cells and neurons and provide important insights into the mechanisms of hippocampal-accentuated brain atrophy and cognitive impairment. The Rnaset2-/- mice may allow the study of CNS damage associated with RNaseT2 deficiency and may be used for the investigation of potential therapies.
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000163450 650_7 $$0EC 3.1.-$$2NLM Chemicals$$aEndoribonucleases
000163450 650_2 $$2MeSH$$aAnimals
000163450 650_2 $$2MeSH$$aCD8-Positive T-Lymphocytes: metabolism
000163450 650_2 $$2MeSH$$aCognitive Dysfunction: genetics
000163450 650_2 $$2MeSH$$aCognitive Dysfunction: metabolism
000163450 650_2 $$2MeSH$$aDisease Models, Animal
000163450 650_2 $$2MeSH$$aEndoribonucleases: genetics
000163450 650_2 $$2MeSH$$aEndoribonucleases: metabolism
000163450 650_2 $$2MeSH$$aFemale
000163450 650_2 $$2MeSH$$aFlow Cytometry
000163450 650_2 $$2MeSH$$aGenotype
000163450 650_2 $$2MeSH$$aHumans
000163450 650_2 $$2MeSH$$aImmunohistochemistry
000163450 650_2 $$2MeSH$$aLeukoencephalopathies: genetics
000163450 650_2 $$2MeSH$$aLeukoencephalopathies: metabolism
000163450 650_2 $$2MeSH$$aLeukoencephalopathies: pathology
000163450 650_2 $$2MeSH$$aMagnetic Resonance Imaging
000163450 650_2 $$2MeSH$$aMale
000163450 650_2 $$2MeSH$$aMemory T Cells: metabolism
000163450 650_2 $$2MeSH$$aMice
000163450 650_2 $$2MeSH$$aMice, Knockout
000163450 650_2 $$2MeSH$$aNeuroglia: metabolism
000163450 650_2 $$2MeSH$$aReal-Time Polymerase Chain Reaction
000163450 7001_ $$00000-0003-3773-4625$$aTernka, Katharina$$b1
000163450 7001_ $$aWendland, Kristin$$b2
000163450 7001_ $$0P:(DE-2719)2812548$$aKrüger, Dennis Manfred$$b3$$udzne
000163450 7001_ $$00000-0003-0134-0974$$aZampar, Silvia$$b4
000163450 7001_ $$aSchob, Charlotte$$b5
000163450 7001_ $$00000-0001-7523-6622$$aFranz, Jonas$$b6
000163450 7001_ $$00000-0001-8331-9874$$aAich, Abhishek$$b7
000163450 7001_ $$aWinkler, Anne$$b8
000163450 7001_ $$0P:(DE-2719)2812054$$aSakib, M Sadman$$b9$$udzne
000163450 7001_ $$0P:(DE-2719)2812832$$aKaurani, Lalit$$b10$$udzne
000163450 7001_ $$0P:(DE-2719)2812061$$aEpple, Robert$$b11$$udzne
000163450 7001_ $$00000-0002-7710-5738$$aWerner, Hauke B$$b12
000163450 7001_ $$aHakroush, Samy$$b13
000163450 7001_ $$aKitz, Julia$$b14
000163450 7001_ $$00000-0002-0349-1955$$aPrinz, Marco$$b15
000163450 7001_ $$00000-0003-0556-1950$$aBartok, Eva$$b16
000163450 7001_ $$aHartmann, Gunther$$b17
000163450 7001_ $$aSchröder, Simone$$b18
000163450 7001_ $$aRehling, Peter$$b19
000163450 7001_ $$aHenneke, Marco$$b20
000163450 7001_ $$00000-0003-2792-7423$$aBoretius, Susann$$b21
000163450 7001_ $$aAlia, A.$$b22
000163450 7001_ $$00000-0002-4115-0334$$aWirths, Oliver$$b23
000163450 7001_ $$0P:(DE-2719)2000047$$aFischer, Andre$$b24$$udzne
000163450 7001_ $$0P:(DE-2719)9000922$$aStadelmann, Christine$$b25$$udzne
000163450 7001_ $$aNessler, Stefan$$b26
000163450 7001_ $$0P:(DE-2719)2811933$$aGärtner, Jutta$$b27$$udzne
000163450 773__ $$0PERI:(DE-600)2553671-0$$a10.1038/s41467-021-26880-x$$gVol. 12, no. 1, p. 6530$$n1$$p6530$$tNature Communications$$v12$$x2041-1723$$y2021
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