OSGEPL1 threonylcarbamoylates adenosine-37 in tRNA

Stable Identifier
R-HSA-9611197
Type
Reaction [transition]
Species
Homo sapiens
Compartment
mitochondrial matrix
ReviewStatus
5/5
Locations in the PathwayBrowser
Expand all
General
SVG | 
PNG
Low
Medium
High
 | PPTX  | SBGN
OSGEPL1 threonylcarbamoylates adenosine-37 in tRNA
Click the image above or here to open this reaction in the Pathway Browser
The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout
In vitro, YRDC and OSGEPL1 catalyze the threonylcarbamoylation of adenosine-37 of 5 mitochondrial tRNAs: tRNA(Asn), tRNA(Ile), tRNA(Lys), tRNA(Ser)(AGY), tRNA(Thr) (Lin et al. 2018, Zhou et al. 2020, reviewed in Su et al. 2022). YRDC and OSGEPL1 are also required for the reaction in vivo and loss of function of OSGEPL1 causes reduced translation efficiency, disrupted translation fidelity, and respiratory defects in human cells (Lin et al. 2018, Zhang et al. 2024). Deletion of Osgepl1 in mice also impairs mitochondrial translation (Zhang et al. 2024). OSGEPL1 threonylcarbamoylates the N6 position of adenosine-37 in 5 mitochondrial tRNAs using threonylcarbamoyladenosine produced by YRDC (Zhou et al. 2020).
Literature References
PubMed ID Title Journal Year
29760464 CO2-sensitive tRNA modification associated with human mitochondrial disease

Lin, H, Miyauchi, K, Harada, T, Okita, R, Takeshita, E, Komaki, H, Fujioka, K, Yagasaki, H, Goto, YI, Yanaka, K, Nakagawa, S, Sakaguchi, Y, Suzuki, T

Nat Commun 2018
38227555 Multifaceted roles of t6A biogenesis in efficiency and fidelity of mitochondrial gene expression

Zhang, Y, Zhou, JB, Yin, Y, Wang, ED, Zhou, XL

Nucleic Acids Res 2024
32047918 Molecular basis for t6A modification in human mitochondria

Zhou, JB, Wang, Y, Zeng, QY, Meng, SX, Wang, ED, Zhou, XL

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

tRNA N(6)-L-threonylcarbamoyladenine synthase activity of OSGEPL1 [mitochondrial matrix]

Physical Entity
OSGEPL1 [mitochondrial matrix] (Homo sapiens)
Activity
tRNA N(6)-L-threonylcarbamoyladenine synthase activity (GO:0061711)
Authored
May, B  (2018-06-17)
Reviewed
Zhou, XL  (2025-02-27)
Created
May, B  (2018-06-18)
Cite Us!