A Drosophila model of Pontocerebellar Hypoplasia reveals a critical role for the RNA exosome in neurons.

doi:10.1371/journal.pgen.1008901

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Article

A Drosophila model of Pontocerebellar Hypoplasia reveals a critical role for the RNA exosome in neurons.

PLoS genetics - 1 Jul 2020

Morton Derrick J, Jalloh Binta, Kim Lily, Kremsky Isaac, Nair Rishi J, Nguyen Khuong B, Rounds J Christopher, Sterrett Maria C, Brown Brianna, Le Thalia, Karkare Maya C, McGaughey Kathryn D, Sheng Shaoyi, Leung Sara W, Fasken Milo B, Moberg Kenneth H, Corbett Anita H

Abstract excerpt

The RNA exosome is an evolutionarily-conserved ribonuclease complex critically important for precise processing and/or complete degradation of a variety of cellular RNAs. The recent discovery that mutations in genes encoding structural RNA exosome subunits cause tissue-specific diseases makes defining the role of this complex within specific tissues critically important. Mutations in the RNA exosome component 3...

Topics

Amino Acid Substitution
Animals
CRISPR-Cas Systems
Cerebellar Diseases
Cerebellum
Cytoskeletal Proteins
Disease Models, Animal
Drosophila melanogaster
Exosome Multienzyme Ribonuclease Complex

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Exosomes

Humans

Mutation

Nerve Tissue Proteins

Neurons

RNA

RNA-Binding Proteins

Source

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Identifiers

PMID: 32645003
DOI: 10.1371/journal.pgen.1008901
PMCID: PMC7373318

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