Format

Send to:

Choose Destination
See comment in PubMed Commons below
Mitochondrion. 2009 Jun;9(3):196-203. doi: 10.1016/j.mito.2009.01.012. Epub 2009 Feb 4.

Recombinant mitochondrial transcription factor A with N-terminal mitochondrial transduction domain increases respiration and mitochondrial gene expression.

Author information

  • 1Center for the Study of Neurodegenerative Diseases and The Morris K. Udall Parkinson's Disease Research Center of Excellence, University of Virginia, PO Box 800394, Charlottesville, VA 22908, United States.

Abstract

We developed a scalable procedure to produce human mitochondrial transcription factor A (TFAM) modified with an N-terminal protein transduction domain (PTD) and mitochondrial localization signal (MLS) that allow it to cross membranes and enter mitochondria through its "mitochondrial transduction domain" (MTD=PTD+MLS). Alexa488-labeled MTD-TFAM rapidly entered the mitochondrial compartment of cybrid cells carrying the G11778A LHON mutation. MTD-TFAM reversibly increased respiration and levels of respiratory proteins. In vivo treatment of mice with MTD-TFAM increased motor endurance and complex I-driven respiration in mitochondria from brain and skeletal muscle. MTD-TFAM increases mitochondrial bioenergetics and holds promise for treatment of mitochondrial diseases involving deficiencies of energy production.

PMID:
19460293
[PubMed - indexed for MEDLINE]
PMCID:
PMC2783715
Free PMC Article
PubMed Commons home

PubMed Commons

0 comments
How to join PubMed Commons

    Supplemental Content

    Full text links

    Icon for Elsevier Science Icon for PubMed Central
    Loading ...
    Write to the Help Desk