Correction of Translational Defects in Patient-derived Mutant Mitochondria by Complex-mediated Import of a Cytoplasmic tRNA
IR@IICB: CSIR-Indian Institute of Chemical Biology, Kolkata
View Archive Info| Field | Value | |
| Title |
Correction of Translational
Defects in Patient-derived
Mutant Mitochondria by
Complex-mediated Import of a
Cytoplasmic tRNA
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| Creator |
Mahata, Bidesh
Bhattacharyya, Suvendra Nath Mukherjee, Saikat Adhya, Samit |
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| Subject |
Molecular & Human Genetics
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| Description |
A variety of clinical disorders result from mutations in
mitochondrial tRNA genes, leading to translational defects.
We show here that a protein complex from the
kinetoplastid protozoon Leishmania induces specific,
ATP-dependent import of human cytoplasmic tRNA1
Lys
into human mitochondria in vitro. The imported tRNA
undergoes efficient aminoacylation within the organelle
and supports organellar protein synthesis. Moreover,
translation in mitochondria from patients with myclonic
epilepsy with ragged red fibers (MERRF) and
Kearns-Sayre syndrome (KSS), containing mutant
tRNALys genes, is stimulated to near-wild-type levels
and the formation of aberrant polypeptides suppressed
by complex-mediated import. These results
suggest a novel way to introduce RNAs for the modulation
of mitochondrial gene expression.
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| Publisher |
American Society for Biochemistry and Molecular Biology
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| Date |
2005
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| Type |
Article
PeerReviewed |
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| Format |
application/pdf
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| Identifier |
http://www.eprints.iicb.res.in/550/1/JOURNAL_OF_BIOLOGICAL_CHEMISTRY__280_(7_)5141%2D5144;2005[94].pdf
Mahata, Bidesh and Bhattacharyya, Suvendra Nath and Mukherjee, Saikat and Adhya, Samit (2005) Correction of Translational Defects in Patient-derived Mutant Mitochondria by Complex-mediated Import of a Cytoplasmic tRNA. The Journal of Biological Chemistry, 280 (7). pp. 5141-5144. |
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| Relation |
http://dx.doi.org/10.1074/jbc.C400572200
http://www.eprints.iicb.res.in/550/ |
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