Functional delivery of a cytosolic tRNA into mutant mitochondria of human cells

被引:80
作者
Mahata, Bidesh [1 ]
Mukherjee, Saikat [1 ]
Mishra, Sumita [1 ]
Bandyopadhyay, Arun [1 ]
Adhya, Samit [1 ]
机构
[1] Indian Inst Chem Biol, Kolkata 700032, W Bengal, India
关键词
D O I
10.1126/science.1129754
中图分类号
O [数理科学和化学]; P [天文学、地球科学]; Q [生物科学]; N [自然科学总论];
学科分类号
07 ; 0710 ; 09 ;
摘要
Many maternally inherited and incurable neuromyopathies are caused by mutations in mitochondrial (mt) transfer RNA ( tRNA) genes. Kinetoplastid protozoa, including Leishmania, have evolved specialized systems for importing nucleus-encoded tRNAs into mitochondria. We found that the Leishmania RNA import complex (RIC) could enter human cells by a caveolin-1-dependent pathway, where it induced import of endogenous cytosolic tRNAs, including tRNALys, and restored mitochondrial function in a cybrid harboring a mutant mt tRNALys (MT-TK) gene. The use of protein complexes to modulate mitochondrial function may help in the management of such genetic disorders.
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收藏
页码:471 / 474
页数:4
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