Decreased mitochondrial respiration in cardiac fibers isolated from a mouse model of Friedreich’s ataxia

PT Pierce, X Wang, A Rivera, A Cooper, H Van Remmen, J Brown, PF Vitiello, The American Journal of the Medical Sciences, Volume 365, Supplement 1, 2023, Pages S194-S195, ISSN 0002-9629, doi:10.1016/S0002-9629(23)00372-5. 

Since mitochondrial complex activities of cardiac fibers from FRDAkD mice were impaired without changes in mitochondrial mass, we hypothesize that bioenergetic changes could be attributable to altered fragmentation of the mitochondrial network. This is the first set of data providing ex vivo evidence of mitochondrial respiratory defects during FXN knockdown associated with FRDA. Furthermore, these findings highlight that there are tissue-specific mechanisms during FXN loss since cardiac and skeletal muscle fibers had differential bioenergetic responses.

Foot and Ankle Biomechanics

Morgan E. Leslie, Joseph M. Iaquinto, Chapter 31 - Neurological Foot Pathology, Editor(s): William R. Ledoux, Scott Telfer, Academic Press, 2023, Pages 489-506, ISBN 9780128154496, doi:10.1016/B978-0-12-815449-6.00047-0. 

This chapter provides an overview of pathologies involving neurological mechanisms affecting foot and ankle biomechanics including stroke, cerebral palsy, toe walking, peripheral neuropathy, foot drop, tarsal tunnel syndrome, Morton’s neuroma, Charcot foot, Charcot-Marie-Tooth disease, Friedreich’s ataxia, and poliomyelitis.

Interactions of reactive sulfur species with metalloproteins

ndrea Domán, Éva Dóka, Dorottya Garai, Virág Bogdándi, György Balla, József Balla, Péter Nagy; Redox Biology, 2023, 102617, ISSN 2213-2317, doi:10.1016/j.redox.2023.102617. 

The current review summarizes the interactions of RSS with protein metal centers and their biological implications with special emphasis on mechanistic aspects, sulfide-mediated signaling, and pathophysiological consequences. A deeper understanding of the biological actions of reactive sulfur species on a molecular level is primordial in H2S-related drug development and the advancement of redox medicine.

Peroxisome proliferator-activated receptor (PPAR) agonists as a potential therapy for inherited metabolic disorders

Bianca Seminotti, Mateus Grings, Nícolas Manzke Glänzel, Jerry Vockley, Guilhian Leipnitz; Biochemical Pharmacology, 2023, 115433, ISSN 0006-2952, doi:10.1016/j.bcp.2023.115433. 

Peroxisomal proliferator-activated receptors (PPARs) consist of a group of nuclear hormone receptors (PPARα, PPARβ/δ, and PPARγ) that regulate multiple cellular functions and processes, including response to oxidative stress, inflammation, lipid metabolism, and mitochondrial bioenergetics and biogenesis. In this context, the activation of PPARs has been shown to stimulate oxidative phosphorylation and reduce reactive species levels. Thus, pharmacological treatment with PPAR activators, such as fibrates, has gained much attention in the last 15 years. This review summarizes preclinical (animal models and patient-derived cells) and clinical data on the effect of PPARs in IMDs.