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Exosome component 8 (EXOSC8)

Target
EXOSC8
Molecular classification
Other (Non-catalytic structural protein of the multi-protein RNA exosome complex)
01

Overview

Exosome component 8 (EXOSC8) is a non-catalytic, structural subunit of the RNA exosome complex, a multiprotein assembly essential for 3'–5' degradation and processing of diverse RNA species, including mRNA, rRNA, snRNA, and snoRNA[1][6]. EXOSC8 participates in both nuclear and cytoplasmic exosome activities: it supports ribonucleolytic processing and surveillance by facilitating the presentation and turnover of normal and aberrant RNAs, especially mRNAs with AU-rich elements in their 3′-UTRs[1][6]. In the nucleus, the exosome complex is required for rRNA and non-coding RNA maturation and for eliminating defective or pervasive transcripts; in the cytoplasm, it is key to general mRNA degradation and mRNA surveillance, preventing translation of aberrant transcripts[1][3][6]. Mutations in EXOSC8 disrupt normal RNA metabolism, resulting in severe neurodevelopmental syndromes such as pontocerebellar hypoplasia and spinal motor neuron disease, elucidating its critical role in human brain development and maintenance[3][4]. There are no current drugs targeting EXOSC8 for therapeutic purposes, and it is not considered a conventional drug target.

Other names
Exosome complex component RRP43OIP2RRP43OIP-2bA421P11.3Rrp43pEAP2p9CIP3CBP-interacting protein 3Opa-interacting protein 2Ribosomal RNA-processing protein 43
02

Biological functions

RNA degradationRNA processingmRNA surveillancematuration of rRNAsnRNAsnoRNAelimination of non-coding and defective RNAsgeneral mRNA turnoverdegradation of ARE-containing mRNAs
03

Disease associations

Neurodegenerative diseasespecifically pontocerebellar hypoplasia (types 1C, 1E)and spinal motor neuron disease
04

Safety considerations

Loss of function causes severe, early-onset neurodegeneration (pontocerebellar hypoplasia), highlighting critical CNS vulnerability to impaired exosome function

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