Kinetics and Mechanism of Mammalian Mitochondrial Ribosome Assembly

被引:99
作者
Bogenhagen, Daniel F. [1 ]
Ostermeyer-Fay, Anne G. [1 ]
Haley, John D. [2 ,3 ]
Garcia-Diaz, Miguel [1 ]
机构
[1] SUNY Stony Brook, Dept Pharmacol Sci, Stony Brook, NY 11794 USA
[2] SUNY Stony Brook, Dept Pathol, Stony Brook, NY 11794 USA
[3] SUNY Stony Brook, Prote Ctr, Stony Brook, NY 11794 USA
关键词
MUTATION; SUBUNIT; DISEASE; MRPL44;
D O I
10.1016/j.celrep.2018.01.066
中图分类号
Q2 [细胞生物学];
学科分类号
071013 [干细胞生物学];
摘要
Mammalian mtDNA encodes only 13 proteins, all essential components of respiratory complexes, synthesized by mitochondrial ribosomes. Mitoribosomes contain greatly truncated RNAs transcribed from mtDNA, including a structural tRNA in place of 5S RNA as a scaffold for binding 82 nucleus-encoded proteins, mitoribosomal proteins (MRPs). Cryoelectron microscopy (cryo-EM) studies have determined the structure of the mitoribosome, but its mechanism of assembly is unknown. Our SILAC pulse-labeling experiments determine the rates of mitochondrial import of MRPs and their assembly into intact mitoribosomes, providing a basis for distinguishing MRPs that bind at early and late stages in mitoribosome assembly to generate a working model for mitoribosome assembly. Mitoribosome assembly is a slow process initiated at the mtDNA nucleoid driven by excess synthesis of individual MRPs. MRPs that are tightly associated in the structure frequently join the complex in a coordinated manner. Clinically significant MRP mutations reported to date affect proteins that bind early on during assembly.
引用
收藏
页码:1935 / 1944
页数:10
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