Next Article in Journal
Role of Autoantibodies in the Diagnosis of Connective-Tissue Disease ILD (CTD-ILD) and Interstitial Pneumonia with Autoimmune Features (IPAF)
Next Article in Special Issue
Riboflavin Responsive Mitochondrial Dysfunction in Neurodegenerative Diseases
Previous Article in Journal
Serum Profiles of Cytokines in Behcet’s Disease
Previous Article in Special Issue
The Value of Coenzyme Q10 Determination in Mitochondrial Patients
Article Menu
Issue 5 (May) cover image

Export Article

Open AccessReview
J. Clin. Med. 2017, 6(5), 50; doi:10.3390/jcm6050050

Glutathione as a Redox Biomarker in Mitochondrial Disease—Implications for Therapy

Departments of Pediatrics and Pathology, Stanford University, 300 Pasteur Drive, H-315, Stanford, CA 94005–5208, USA
*
Author to whom correspondence should be addressed.
Academic Editor: Iain P. Hargreaves
Received: 17 February 2017 / Revised: 24 April 2017 / Accepted: 27 April 2017 / Published: 3 May 2017
View Full-Text   |   Download PDF [250 KB, uploaded 3 May 2017]

Abstract

Technical advances in the ability to measure mitochondrial dysfunction are providing new insights into mitochondrial disease pathogenesis, along with new tools to objectively evaluate the clinical status of mitochondrial disease patients. Glutathione (l-ϒ-glutamyl-l-cysteinylglycine) is the most abundant intracellular thiol, and the intracellular redox state, as reflected by levels of oxidized (GSSG) and reduced (GSH) glutathione, as well as the GSH/GSSG ratio, is considered to be an important indication of cellular health. The ability to quantify mitochondrial dysfunction in an affected patient will not only help with routine care, but also improve rational clinical trial design aimed at developing new therapies. Indeed, because multiple disorders have been associated with either primary or secondary deficiency of the mitochondrial electron transport chain and redox imbalance, developing mitochondrial therapies that have the potential to improve the intracellular glutathione status has been a focus of several clinical trials over the past few years. This review will also discuss potential therapies to increase intracellular glutathione with a focus on EPI-743 (α-tocotrienol quinone), a compound that appears to have the ability to modulate the activity of oxidoreductases, in particular NAD(P)H:quinone oxidoreductase 1. View Full-Text
Keywords: mitochondrial disease; glutathione; redox imbalance; EPI-743; N-acetylcysteine; RP103; cysteamine mitochondrial disease; glutathione; redox imbalance; EPI-743; N-acetylcysteine; RP103; cysteamine
This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. (CC BY 4.0).

Scifeed alert for new publications

Never miss any articles matching your research from any publisher
  • Get alerts for new papers matching your research
  • Find out the new papers from selected authors
  • Updated daily for 49'000+ journals and 6000+ publishers
  • Define your Scifeed now

SciFeed Share & Cite This Article

MDPI and ACS Style

Enns, G.M.; Cowan, T.M. Glutathione as a Redox Biomarker in Mitochondrial Disease—Implications for Therapy. J. Clin. Med. 2017, 6, 50.

Show more citation formats Show less citations formats

Note that from the first issue of 2016, MDPI journals use article numbers instead of page numbers. See further details here.

Related Articles

Article Metrics

Article Access Statistics

1

Comments

[Return to top]
J. Clin. Med. EISSN 2077-0383 Published by MDPI AG, Basel, Switzerland RSS E-Mail Table of Contents Alert
Back to Top