Publication: Mutant MRPS5 affects mitoribosomal accuracy and confers stress-related behavioral alterations
Mutant MRPS5 affects mitoribosomal accuracy and confers stress-related behavioral alterations
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Akbergenov, R., Duscha, S., Fritz, A.-K., Juskeviciene, R., Oishi, N., Schmitt, K., Shcherbakov, D., Teo, Y., Boukari, H., Freihofer, P., Isnard-Petit, P., Oettinghaus, B., Frank, S., Thiam, K., Rehrauer, H., Westhof, E., Schacht, J., Eckert, A., Wolfer, D., & Böttger, E. C. (2018). Mutant MRPS5 affects mitoribosomal accuracy and confers stress-related behavioral alterations. EMBO Reports, 19, e46193. https://doi.org/10.15252/embr.201846193
Abstract
Abstract
Abstract
The 1555 A to G substitution in mitochondrial 12S A-site rRNA is associated with maternally transmitted deafness of variable penetrance in the absence of otherwise overt disease. Here, we recapitulate the suggested A1555G-mediated pathomechanism in an experimental model of mitoribosomal mistranslation by directed mutagenesis of mitoribosomal protein MRPS5. We first establish that the ratio of cysteine/methionine incorporation and read-through of mtDNA-encoded MT-CO1 protein constitute reliable measures of mitoribosomal misreading. Nex
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Creators (Authors)
- Akbergenov, Rashid
- Duscha, Stefan
- Fritz, Ann-Kristina
- Juskeviciene, Reda
- Oishi, Naoki
- Schmitt, Karen
- Shcherbakov, Dimitri
- Teo, Youjin
- Boukari, Heithem
- Freihofer, Pietro
- Isnard-Petit, Patricia
- Oettinghaus, Björn
- Frank, Stephan
- Thiam, Kader
- Rehrauer, Hubert
- Westhof, Eric
- Schacht, Jochen
- Eckert, Anne
- Wolfer, David
- Böttger, Erik C
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Akbergenov, R., Duscha, S., Fritz, A.-K., Juskeviciene, R., Oishi, N., Schmitt, K., Shcherbakov, D., Teo, Y., Boukari, H., Freihofer, P., Isnard-Petit, P., Oettinghaus, B., Frank, S., Thiam, K., Rehrauer, H., Westhof, E., Schacht, J., Eckert, A., Wolfer, D., & Böttger, E. C. (2018). Mutant MRPS5 affects mitoribosomal accuracy and confers stress-related behavioral alterations. EMBO Reports, 19, e46193. https://doi.org/10.15252/embr.201846193
