TBRG4 (FASTKD4) binds mitochondrial rRNAs and mRNAs

Stable Identifier
R-HSA-9837149
Type
Reaction [binding]
Species
Homo sapiens
Compartment
Synonyms
TBRG4 (FASTKD4) binds 12S rRNA,16S rRNA, MT-ATP6 mRNA, MTCO1,2,3 mRNA,MT-CYB mRNA,MT-ND1,2,3,4,5,6 mRNA
ReviewStatus
5/5
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TBRG4 (also called FASTKD4) binds most RNAs encoded by the heavy strand of mitochondrial DNA and enhances their steady state levels (Wolf and Mootha 2014, Boehm et al. 2017, Ohkubo et al. 2021). Large mitochondrial precursor RNAs are processed by cleavage at the tRNA sequences that flank most protein coding sequences; however, TBRG4 promotes expression of RNAs that are not flanked by tRNA genes (MT-ND5, MT-CYB, MT-CO1, MT-CO3 and MT-ATP8/6) and, therefore plays a role in processing the mRNAs or protecting the processed, products from degradation by SUPV3L1:PNPT1 (Boehm et al. 2017). Absence of TBGR4 causes increased degradation of MT-CO1, MT-ND3, and MT-CO2 mRNAs and decreased processing of MT-ND5 and MT-CYB mRNAs (Boehm et al. 2014). TBRG4 also binds tRNAE, which is complementary to the MT-ND5/MT-CYB processing site (Ohkubo et al. 2021).
Literature References
PubMed ID Title Journal Year
24746820 Functional genomic analysis of human mitochondrial RNA processing

Wolf, AR, Mootha, VK

Cell Rep 2014
28335001 FASTKD1 and FASTKD4 have opposite effects on expression of specific mitochondrial RNAs, depending upon their endonuclease-like RAP domain

Zaganelli, S, Boehm, E, Maundrell, K, Thore, S, Martinou, JC, Jourdain, AA

Nucleic Acids Res 2017
34748562 The FASTK family proteins fine-tune mitochondrial RNA processing

Minczuk, M, Stentenbach, M, Filipovska, A, Van Haute, L, Martinou, JC, Rudler, DL, Steiner, FA, Rackham, O, Ohkubo, A

PLoS Genet 2021
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