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

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.

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    Abstract

    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

    Item Type: Article
    URI: http://www.eprints.iicb.res.in/id/eprint/1308
    Subjects: Molecular & Human Genetics
    Divisions: Indian Institute of Chemical Biology
    Depositing User: Ms Sutapa Ganguly
    Date Deposited: 24 Jan 2012 12:12
    Last Modified: 25 Jan 2012 11:30
    Official URL: http://dx.doi.org/10.1016/j.mito.2011.03.006
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