SLIRP:LRPPRC binds mitochondrial RNAs

Stable Identifier
R-HSA-9837034
Type
Reaction [binding]
Species
Homo sapiens
Compartment
mitochondrial matrix
ReviewStatus
5/5
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SLIRP:LRPPRC binds mitochondrial RNAs
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The SLIRP:LRPPRC complex (Sasarman et al. 2010, inferred from mouse homologs in Ruzzenente et al. 2012) binds throughout the mitochondrial transcriptome (Siira et al. 2017) including 12S rRNA, 16S rRNA, 13 mRNAs, and 5 precursor RNAs, and inhibits their degradation (Sasarman et al. 2010, Chujo et al. 2012, Siira et al. 2017). SLIRP:LRPPRC reduces secondary structure of RNAs and may act to expose sites for translation, stabilization, and polyadenylation (Siira et al. 2017). Mutations in LRPPRC cause French Canadian Leigh Syndrome which is characterized by neurodegeneration caused by a deficiency of cytochrome c oxidase (Sasarman et al. 2010).
Literature References
PubMed ID Title Journal Year
20200222 LRPPRC and SLIRP interact in a ribonucleoprotein complex that regulates posttranscriptional gene expression in mitochondria

Antonicka, H, Consortium, LSFC, Sasarman, F, Shoubridge, EA, Wai, T, Brunel-Guitton, C

Mol Biol Cell 2010
29146908 LRPPRC-mediated folding of the mitochondrial transcriptome

Shearwood, AJ, Ruzzenente, B, Filipovska, A, Larsson, NG, Spåhr, H, Siira, SJ, Rackham, O

Nat Commun 2017
22661577 LRPPRC/SLIRP suppresses PNPase-mediated mRNA decay and promotes polyadenylation in human mitochondria

Suzuki, T, Chujo, T, Ohira, T, Nomura, N, Sakaguchi, Y, Nagao, A, Goshima, N

Nucleic Acids Res 2012
Participants
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Output
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Authored
May, B  (2023-06-07)
Reviewed
Anonymous, R  (2023-08-09)
Created
May, B  (2023-06-08)
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