Article

Loss of MTPAP disrupts mitochondrial RNA processing causing upregulation of type I interferon signalling

2026-05-07

Abstract excerpt

Mitochondrial poly-A polymerase (MTPAP) is essential for mitochondrial mRNA (mt-mRNA) polyadenylation, a critical step in mt-mRNA maturation. Mutations in MTPAP have been reported to cause a mitochondrial cytopathy. Here, we provide evidence of enhanced type I interferon (IFN) signalling in the blood of patients carrying mutations in MTPAP . Further, deletion of MTPAP in a fibroblast cell model led to abnormali...

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Literature Corpus work: a42570f7-cb0f-569f-8eea-2d971307521f
DOI: 10.64898/2026.05.04.722669

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