Reaction: SUPV3L1 unwinds double-stranded mitochondiral RNA
- in pathway: Mitochondrial RNA degradation
A dimer of SUPV3L1 and three molecules of PNPT1 form a complex called the degradosome (Wang et al. 2009). SUPV3L1 (SUV3, hSUV3) is a RNA helicase that unwinds double-stranded RNA prior to degradation of the resulting single-stranded RNA by the exonuclease PNPT1 (PNPase) (Shu et al. 2004, Dhir et al. 2018, Jain et al. 2022). In addition to preventing accumulation of double-stranded RNA, the SUPV3L1:PNPT1 complex also plays a role in eliminating R-loop structures formed during the replication process in mitochondria (Silva et al. 2018). A mutation due to a premature stop codon in SUPV3L1 causes accumulation of double-stranded mitochondrial RNA in humans (van Esveld et al. 2022).
Reaction - small molecule participants:
Pi [mitochondrial matrix]
ADP [mitochondrial matrix]
ATP [mitochondrial matrix]
Reactome.org reaction link: R-HSA-9836927
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Reaction input - small molecules:
ATP(4-)
Reaction output - small molecules:
hydrogenphosphate
ADP(3-)
Reactome.org link: R-HSA-9836927