Record Details

Correction of Translational Defects in Patient-derived Mutant Mitochondria by Complex-mediated Import of a Cytoplasmic tRNA

EPrints@IICB

View Archive Info
 
 
Field Value
 
Title Correction of Translational
Defects in Patient-derived
Mutant Mitochondria by
Complex-mediated Import of a
Cytoplasmic tRNA
 
Creator Mahata, Bidesh
Bhattacharyya, Suvendra Nath
Mukherjee, Saikat
Adhya, Samit
 
Subject Molecular & Human Genetics
 
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.
 
Publisher American Society for Biochemistry and Molecular Biology
 
Date 2005
 
Type Article
PeerReviewed
 
Format application/pdf
 
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.
 
Relation http://dx.doi.org/10.1074/jbc.C400572200
http://www.eprints.iicb.res.in/550/