Augmenter of liver regeneration, a protective factor against ROS-induced oxidative damage in muscle tissue of mitochondrial myopathy affected patients

Lorenzo Polimeno, Roberta Rossi, Maria Mastrodonato, Monica Montagnani, Domenico Piscitelli, Barbara Pesetti, Leonarda De Benedictis, Bruna Girardi, Leonardo Resta, Anna Napoli, Antonio Francavilla

Research output: Contribution to journalArticlepeer-review

Abstract

Mitochondria-related myopathies (MM) are a group of different diseases defined by a varying degree of dysfunctions of the mitochondrial respiratory chain which leads to reactive oxygen species (ROS) generation followed by oxidative stress and cellular damage. In mitochondrial myopathy muscle tissue an overexpression of antioxidant enzymes has been documented probably as an attempt to counteract the free radical generation. We previously documented, in human non-pathological muscle fibres, the expression of the augmenter of liver regeneration (ALR), a sulfhydryl oxidase enzyme, whose presence is related to the mitochondria; indeed it has been demonstrated that ALR mainly localizes in the mitochondrial inter-membrane space. Furthermore we reported, in different experimental models, in vivo and in vitro, the anti-apoptotic and anti-oxidative capacities of ALR, achieved by up-regulating Bcl-2 anti-apoptotic family factors and the anti-apoptotic/anti-oxidative secretory isoform of clusterin (sClu). With the present study we aimed to determine ALR, Bcl-2 protein, clusterin and ROS expression in muscle tissue biopsies from MM-affected patients. Non-pathological muscle tissue was used as control. Enzymatic, histochemical, immunohistochemical and immune electron microscopy techniques were performed. The data obtained revealed in MM-derived muscle tissue, compared to non-pathological tissue, the over-expression of ROS, ALR and Bcl-2 and the induction of the nuclear, pro-apoptotic, isoform of clusterin (nCLU).

Original languageEnglish
Pages (from-to)2410-2419
Number of pages10
JournalInternational Journal of Biochemistry and Cell Biology
Volume45
Issue number11
DOIs
Publication statusPublished - 2013

Keywords

  • ALR
  • Apoptosis
  • Myopathies
  • ROS

ASJC Scopus subject areas

  • Biochemistry
  • Cell Biology
  • Medicine(all)

Fingerprint Dive into the research topics of 'Augmenter of liver regeneration, a protective factor against ROS-induced oxidative damage in muscle tissue of mitochondrial myopathy affected patients'. Together they form a unique fingerprint.

Cite this