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Article

Defective myelination in an RNA polymerase III mutant leukodystrophic mouse.

Proceedings of the National Academy of Sciences of the United States of America - 5 Oct 2021

Merheb Emilio, Cui Min-Hui, DuBois Juwen C, Branch Craig A, Gulinello Maria, Shafit-Zagardo Bridget, Moir Robyn D, Willis Ian M

Abstract excerpt

RNA polymerase (Pol) III synthesizes abundant short noncoding RNAs that have essential functions in protein synthesis, secretion, and other processes. Despite the ubiquitous functions of these RNAs, mutations in Pol III subunits cause Pol III-related leukodystrophy, an early-onset neurodegenerative disease. The basis of this neural sensitivity and the mechanisms of disease pathogenesis are unknown. Here we show...

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Identifiers

PMID: 34583988
DOI: 10.1073/pnas.2024378118
PMCID: PMC8501794

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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