SUPV3L1 unwinds double-stranded mitochondiral RNA

Stable Identifier
R-HSA-9836927
Type
Reaction [transition]
Species
Homo sapiens
Compartment
mitochondrial matrix
ReviewStatus
5/5
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SUPV3L1 unwinds double-stranded mitochondiral RNA
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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).
Literature References
PubMed ID Title Journal Year
30046113 Mitochondrial double-stranded RNA triggers antiviral signalling in humans

Dhir, A, Dhir, S, Borowski, LS, Jimenez, L, Teitell, M, Rötig, A, Crow, YJ, Rice, GI, Duffy, D, Tamby, C, Nojima, T, Munnich, A, Schiff, M, de Almeida, CR, Rehwinkel, J, Dziembowski, A, Szczesny, RJ, Proudfoot, NJ

Nature 2018
35481630 Dimeric assembly of human Suv3 helicase promotes its RNA unwinding function in mitochondrial RNA degradosome for RNA decay

Jain, M, Golzarroshan, B, Lin, CL, Agrawal, S, Tang, WH, Wu, CJ, Yuan, HS

Protein Sci 2022
15096047 Purified human SUV3p exhibits multiple-substrate unwinding activity upon conformational change

Shu, Z, Vijayakumar, S, Chen, CF, Chen, PL, Lee, WH

Biochemistry 2004
19509288 Human mitochondrial SUV3 and polynucleotide phosphorylase form a 330-kDa heteropentamer to cooperatively degrade double-stranded RNA with a 3'-to-5' directionality

Wang, DD, Shu, Z, Lieser, SA, Chen, PL, Lee, WH

J Biol Chem 2009
Participants
Input
Output
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as an event of
Catalyst Activity

3'-5' RNA helicase activity of SUPV3L1:PNPT1 [mitochondrial matrix]

Physical Entity
SUPV3L1:PNPT1 [mitochondrial matrix] (Homo sapiens)
Activity
3'-5' RNA helicase activity (GO:0034458)
Authored
May, B  (2023-06-06)
Reviewed
Anonymous, R  (2023-08-09)
Created
May, B  (2023-06-06)
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