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Article

Mitochondrial spongiotic brain disease: astrocytic stress and harmful rapamycin and ketosis effect.

Life science alliance - 1 Sept 2020

Ignatenko Olesia, Nikkanen Joni, Kononov Alexander, Zamboni Nicola, Ince-Dunn Gulayse, Suomalainen Anu

Abstract excerpt

Mitochondrial DNA (mtDNA) depletion syndrome (MDS) is a group of severe, tissue-specific diseases of childhood with unknown pathogenesis. Brain-specific MDS manifests as devastating spongiotic encephalopathy with no curative therapy. Here, we report cell type-specific stress responses and effects of rapamycin treatment and ketogenic diet (KD) in mice with spongiotic encephalopathy mimicking human MDS, as these...

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Identifiers

PMID: 32737078
DOI: 10.26508/lsa.202000797
PMCID: PMC7409372

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

Source receipt: pubmed, retrieved 6 Sept 2026.

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