Pathological mutations promote proteolysis of mitochondrial tRNA-specific 2-thiouridylase 1 (MTU1) via mitochondrial caseinolytic peptidase (CLPP)

Ahmad, RNR; Zhang, LT; Morita, R; Tani, H; Wu, Y; Chujo, T; Ogawa, A; Harada, R; Shigeta, Y; Tomizawa, K; Wei, FY

Wei, FY (通讯作者)，Tohoku Univ, Dept Mod Biol & Med, Inst Dev Aging & Canc, Sendai, Miyagi 9808575, Japan.

NUCLEIC ACIDS RESEARCH, 2023; ():

Abstract

MTU1 controls intramitochondrial protein synthesis by catalyzing the 2-thiouridine modification of mitochondrial transfer RNAs (mt-tRNAs). Missense mu......

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