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Putative adjunct therapies to target mitochondrial dysfunction and oxidative stress in phenylketonuria, lysosomal storage disorders and peroxisomal disorders

Turton, N, Rutherford, T, Thijssen, DHJ and Hargreaves, IP (2020) Putative adjunct therapies to target mitochondrial dysfunction and oxidative stress in phenylketonuria, lysosomal storage disorders and peroxisomal disorders. Expert Opinion on Orphan Drugs, 8 (11). pp. 431-444. ISSN 2167-8707

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Putative adjunct therapies to target mitochondria dysfunction and oxidative stress in Phenylketonuria, Lysosomal storage disorders and Peroxisomal disorders..pdf - Accepted Version
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Abstract

Introduction: Oxidative stress (OS) and mitochondrial dysfunction are implicated in the pathogenesis of a number of metabolic diseases. OS occurs when there is an imbalance between the pro-oxidant/antioxidant homeostasis, leading to an increased generation of reactive oxidant species (ROS) with resultant cellular dysfunction. It is becoming apparent that increased ROS generation may be attributable to secondary mitochondrial dysfunction as a consequence of disease pathophysiology. Mitochondrial dysfunction occurs as a result of oxidative damage from enhanced ROS generation as well as the accumulation of toxic metabolites in some metabolic diseases.
Areas covered: The present review will discuss evidence of OS and mitochondrial dysfunction in phenylketonuria (PKU), lysosomal storage disorders (LSDs), and peroxisomal disorders. In addition, potential adjunct therapies which have the potential to enhance mitochondrial functioning and mitigate OS will be explored. The databases utilized for this review were Pubmed and the Wed of science, with inclusive dates, 1988–2020.
Expert opinion: There is an un-unified approach in the treatment of metabolic diseases. Agents including augmenters of mitochondrial function, antioxidants, and activators of mitochondrial biogenesis, may be beneficial. However, although successful in some cases, these adjunct therapies have yet to be incorporated into the clinical-management of metabolic diseases.

Item Type: Article
Additional Information: This is an Accepted Manuscript of an article published by Taylor & Francis in Expert Opinion on Orphan Drugs on 26/11/2020, available online: http://www.tandfonline.com/10.1080/21678707.2020.1850254
Subjects: Q Science > QR Microbiology
R Medicine > RC Internal medicine > RC1200 Sports Medicine
R Medicine > RM Therapeutics. Pharmacology
Divisions: Pharmacy & Biomolecular Sciences
Sport & Exercise Sciences
Publisher: Taylor & Francis
Date Deposited: 15 Feb 2021 10:32
Last Modified: 15 Feb 2021 10:32
DOI or Identification number: 10.1080/21678707.2020.1850254
URI: https://researchonline.ljmu.ac.uk/id/eprint/14096

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