Altered threshold of the mitochondrial permeability transition pore in Ullrich congenital muscular dystrophy.

Biochimica Et Biophysica Acta
A AngelinPaolo Bernardi

Abstract

We have studied the effects of rotenone in myoblasts from healthy donors and from patients with Ullrich congenital muscular dystrophy (UCMD), a severe muscle disease due to mutations in the genes encoding the extracellular matrix protein collagen VI. Addition of rotenone to normal myoblasts caused a very limited mitochondrial depolarization because the membrane potential was maintained by the F1FO synthase, as indicated by full depolarization following the subsequent addition of oligomycin. In UCMD myoblasts rotenone instead caused complete mitochondrial depolarization, which was followed by faster ATP depletion than in healthy myoblasts. Mitochondrial depolarization could be prevented by treatment with cyclosporin A and intracellular Ca(2+) chelators, while it was worsened by depleting Ca(2+) stores with thapsigargin. Thus, in UCMD myoblasts rotenone-induced depolarization is due to opening of the permeability transition pore rather than to inhibition of electron flux as such. These findings indicate that in UCMD myoblasts the threshold for pore opening is very close to the resting membrane potential, so that even a small depolarization causes permeability transition pore opening and precipitates ATP depletion.

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Citations

Dec 18, 2013·Seminars in Pediatric Neurology·Christos D KatsetosJoseph J Melvin
May 24, 2011·Cell Calcium·Andrea Rasola, Paolo Bernardi
Feb 24, 2016·Biochimica Et Biophysica Acta·Lucia BiasuttoMario Zoratti
Oct 27, 2011·Oxidative Medicine and Cellular Longevity·Luciano MerliniPaolo Bernardi
Nov 22, 2012·Journal of Cellular Physiology·Camilla PellegriniNadir M Maraldi
Nov 15, 2008·Biochimica Et Biophysica Acta·Karin NowikovskyPaolo Bernardi
Mar 19, 2009·Brain : a Journal of Neurology·Paolo BernardiPatrizia Sabatelli
Jun 24, 2009·Arquivos de neuro-psiquiatria·Umbertina Conti Reed
Jun 22, 2011·Nature Reviews. Neurology·Carsten G Bönnemann
Apr 3, 2014·Physiological Reviews·Ildiko Szabo, Mario Zoratti
Jan 17, 2020·Circulation Research·Tyler M Bauer, Elizabeth Murphy
Mar 19, 2014·EMBO Reports·Caterina Da-RéPaolo Bernardi
Sep 24, 2020·Cell Biochemistry and Biophysics·Adeola Oluwakemi OlowofolahanOlufunso Olabode Olorunsogo

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