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

来源：

SCIENCE | 2006年 / 314卷 / 5798期

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.

引用

页码：471 / 474

页数：4

共 23 条

[1] Ping-Pong interactions between mitochondrial tRNA import receptors within a multiprotein complex [J].

Bhattacharyya, SN ;

Chatterjee, S ;

Goswami, S ;

Tripathi, G ;

Dey, SN ;

Adhya, S .

MOLECULAR AND CELLULAR BIOLOGY, 2003, 23 (15) :5217-5224

[2]

Bhattacharyya Suvendra Nath, 2004, RNA Biol, V1, P84, DOI 10.4161/rna.1.2.1180

[3]

CHEN LB, 1988, ANNU REV CELL BIOL, V4, P155, DOI 10.1146/annurev.cellbio.4.1.155

[4] MTDNA MUTATION IN MERRF-SYNDROME CAUSES DEFECTIVE AMINOACYLATION OF TRNA(LYS) AND PREMATURE TRANSLATION TERMINATION [J].

ENRIQUEZ, JA ;

CHOMYN, A ;

ATTARDI, G .

NATURE GENETICS, 1995, 10 (01) :47-55

[5] MITOCHONDRIAL DIABETES-MELLITUS - A REVIEW [J].

GERBITZ, KD ;

VANDENOUWELAND, JMW ;

MAASSEN, JA ;

JAKSCH, M .

BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE, 1995, 1271 (01) :253-260

[6] Allosteric regulation of tRNA import:: interactions between tRNA domains at the inner membrane of Leishmania mitochondria [J].

Goswami, S ;

Chatterjee, S ;

Bhattacharyya, SN ;

Basu, S ;

Adhya, S .

NUCLEIC ACIDS RESEARCH, 2003, 31 (19) :5552-5559

[7] A bifunctional tRNA import receptor from Leishmania mitochondria [J].

Goswami, Srikanta ;

Dhar, Gunjan ;

Mukherjee, Saikat ;

Mahata, Bidesh ;

Chatterjee, Saibal ;

Home, Pratik ;

Adhya, Samit .

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 2006, 103 (22) :8354-8359

[8] A MUTATION IN THE TRANSFER RNALEU(UUR) GENE ASSOCIATED WITH THE MELAS SUBGROUP OF MITOCHONDRIAL ENCEPHALOMYOPATHIES [J].

GOTO, Y ;

NONAKA, I ;

HORAI, S .

NATURE, 1990, 348 (6302) :651-653

[9]

GOTO Y, 1995, MUSCLE NERVE, pS107

[10] TRANSFER-RNAS ARE IMPORTED INTO MITOCHONDRIA OF TRYPANOSOMA-BRUCEI INDEPENDENTLY OF THEIR GENOMIC CONTEXT AND GENETIC-ORIGIN [J].

HAUSER, R ;

SCHNEIDER, A .

EMBO JOURNAL, 1995, 14 (17) :4212-4220

← 1 2 3 →