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RNA-Mediated Restoration of Mitochondrial Function in Cells Harboring a Kearns Sayre Syndrome Mutation.

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Title RNA-Mediated Restoration of Mitochondrial Function in Cells Harboring a Kearns Sayre Syndrome Mutation.
 
Creator Mahato, Biraj
Jash, Sukanta
Adhya, Samit
 
Subject Molecular & Human Genetics
 
Description Mutations in mitochondrial DNA (mtDNA) generate multi-system disorders due to failure of ATP production.
A cybrid containing a 1.9-kb mtDNA deletion from a patient with Kearns Sayre Syndrome is respirationdefective
and grows glycolytically. When treated with a ribonucleoprotein (RNP) complex of polycistronic
RNA 1 (pcRNA1) containing mtDNA-encoded genes and a multi-subunit carrier complex R8, full-length
pcRNA1 was transported to mitochondria. Translation of the pcRNA1-encoded mRNAs was observed in
mitochondria from RNP-treated cells. Respiration of the cybrid was rescued to ~90% of normal within hours,
switching the cells to aerobic growth. These findings have implications for the development of effective
mitochondrial gene therapy
 
Publisher Elsevier
 
Date 2011
 
Type Article
PeerReviewed
 
Format application/pdf
 
Identifier http://www.eprints.iicb.res.in/1308/1/MITOCHONDRION__11(_4)_564%2D574;2011[67].pdf
Mahato, Biraj and Jash, Sukanta and Adhya, Samit (2011) RNA-Mediated Restoration of Mitochondrial Function in Cells Harboring a Kearns Sayre Syndrome Mutation. Mitochondrion, 11 (4). pp. 564-574.
 
Relation http://dx.doi.org/10.1016/j.mito.2011.03.006
http://www.eprints.iicb.res.in/1308/