SUPV3L1:PNPT1 hydrolyzes mitochondrial RNA to yield 4-5 nucleotide oligoribonucleotides

Stable Identifier
R-HSA-9836585
Type
Reaction [transition]
Species
Homo sapiens
Compartment
mitochondrial matrix
ReviewStatus
5/5
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SUPV3L1:PNPT1 hydrolyzes mitochondrial RNA to yield 4-5 nucleotide oligoribonucleotides
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A dimer of SUPV3L1 (SUV3, hSUV3) and three molecules of PNPT1 (PNPase) form a complex, the degradosome, that is localized with RNA adjacent to nucleoids (Borowski et al. 2013) and that unwinds double-stranded RNA (Jain et al. 2022) and exonucleolytically degrades the resulting single-stranded RNA (Wang et al. 2009, Szczesny et al. 2010, Borowski et al. 2013). PNPT1 is a 3'-5' exonuclease that produces 4-5 nucleotide RNAs called nanoRNAs (Lin et al. 2012, Szewczyk et al. 2020) which are then degraded by the REXO2 dimer (Szewczyk et al. 2020).
GRSF1 binds regions of particular RNAs that contain quadruplex G structures, interacts with SUPV3L1:PNPT1 via the RNA, and unwinds the quadruplex G to yield a single-stranded RNA that is a substrate for SUPV3L1:PNPT1 (Pietras et al. 2018). Silencing of PNPT1 (Silva et al. 2018) or a mutation that prematurely truncates the C-terminus of SUPV3L1 (van Esveld et al. 2022) causes accumulation of double-stranded RNA in mitochondria.
Literature References
PubMed ID Title Journal Year
29967381 Dedicated surveillance mechanism controls G-quadruplex forming non-coding RNAs in human mitochondria

Dziembowski, A, Stepien, PP, Szczesny, RJ, Szewczyk, M, Wojcik, MA, Kulinski, TM, Borowski, LS, Cysewski, D, Pietras, Z

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

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

Protein Sci 2022
22210891 Crystal structure of human polynucleotide phosphorylase: insights into its domain function in RNA binding and degradation

Yang, WZ, Hsiao, YY, Lin, CL, Yuan, HS, Wang, YT

Nucleic Acids Res 2012
19864255 Human mitochondrial RNA turnover caught in flagranti: involvement of hSuv3p helicase in RNA surveillance

Brzezniak, LK, Gewartowski, K, Stepien, PP, Szczesny, RJ, Dmochowska, A, Borowski, LS, Bartnik, E

Nucleic Acids Res 2010
23221631 Human mitochondrial RNA decay mediated by PNPase-hSuv3 complex takes place in distinct foci

Dziembowski, A, Stepien, PP, Szczesny, RJ, Borowski, LS, Hejnowicz, MS

Nucleic Acids Res 2013
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, Chen, PL, Shu, Z, Lieser, SA, Lee, WH

J Biol Chem 2009
32365187 Human REXO2 controls short mitochondrial RNAs generated by mtRNA processing and decay machinery to prevent accumulation of double-stranded RNA

Kotrys, AV, Nowotny, M, Malik, D, Szczesny, RJ, Szewczyk, M, Czarnomska, SD, Borowski, LS, Klosowska-Kosicka, K

Nucleic Acids Res 2020
Participants
Input
Output
Participates
as an event of
Catalyst Activity

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

Physical Entity
SUPV3L1:PNPT1 [mitochondrial matrix] (Homo sapiens)
Activity
3'-5'-RNA exonuclease activity (GO:0000175)
This event is regulated
Negatively by
Regulator
SLIRP:LRPPRC [mitochondrial matrix] (Homo sapiens)
Authored
May, B  (2023-05-28)
Reviewed
Anonymous, R  (2023-08-09)
Created
May, B  (2023-05-30)
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