Sea squirt alternative oxidase bypasses fatal mitochondrial heart disease

Sea squirt alternative oxidase bypasses fatal mitochondrial heart disease

Mitochondrial diseases are a diverse group of inborn disorders affecting cellular energy production by oxidative phosphorylation (OXPHOS) via the five (CI‐CV) mitochondrial respiratory chain (MRC) complexes. The sea squirt alternative oxidase (AOX) is able to bypass the distal part of the MRC and wa...

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Main Author: Ann Saada
Format: Article
Language:English
Published: Wiley 2019-01-01
Series:EMBO Molecular Medicine
Online Access:https://doi.org/10.15252/emmm.201809962
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spelling doaj-1e642ba4a8ad4473ae150b1b34f7dd4a2021-08-02T16:46:08ZengWileyEMBO Molecular Medicine1757-46761757-46842019-01-01111n/an/a10.15252/emmm.201809962Sea squirt alternative oxidase bypasses fatal mitochondrial heart diseaseAnn Saada0The Monique and Jacques Roboh Department of Genetic Research Department of Genetics and Metabolic Diseases Hadassah Medical Center Jerusalem IsraelMitochondrial diseases are a diverse group of inborn disorders affecting cellular energy production by oxidative phosphorylation (OXPHOS) via the five (CI‐CV) mitochondrial respiratory chain (MRC) complexes. The sea squirt alternative oxidase (AOX) is able to bypass the distal part of the MRC and was shown to alleviate the consequences of CIII and CIV defects in several cellular and Drosophila models. In this issue of EMBO Molecular Medicine, Rajendran et al () demonstrate the first proof of concept in mammals, by showing that AOX is capable to extend lifespan and prevent heart failure in a CIII deficient mouse model, raising the possibility of future human AOX bypass treatment.https://doi.org/10.15252/emmm.201809962
collection DOAJ
language English
format Article
sources DOAJ
author Ann Saada
spellingShingle Ann Saada
Sea squirt alternative oxidase bypasses fatal mitochondrial heart disease
EMBO Molecular Medicine
author_facet Ann Saada
author_sort Ann Saada
title Sea squirt alternative oxidase bypasses fatal mitochondrial heart disease
title_short Sea squirt alternative oxidase bypasses fatal mitochondrial heart disease
title_full Sea squirt alternative oxidase bypasses fatal mitochondrial heart disease
title_fullStr Sea squirt alternative oxidase bypasses fatal mitochondrial heart disease
title_full_unstemmed Sea squirt alternative oxidase bypasses fatal mitochondrial heart disease
title_sort sea squirt alternative oxidase bypasses fatal mitochondrial heart disease
publisher Wiley
series EMBO Molecular Medicine
issn 1757-4676
1757-4684
publishDate 2019-01-01
description Mitochondrial diseases are a diverse group of inborn disorders affecting cellular energy production by oxidative phosphorylation (OXPHOS) via the five (CI‐CV) mitochondrial respiratory chain (MRC) complexes. The sea squirt alternative oxidase (AOX) is able to bypass the distal part of the MRC and was shown to alleviate the consequences of CIII and CIV defects in several cellular and Drosophila models. In this issue of EMBO Molecular Medicine, Rajendran et al () demonstrate the first proof of concept in mammals, by showing that AOX is capable to extend lifespan and prevent heart failure in a CIII deficient mouse model, raising the possibility of future human AOX bypass treatment.
url https://doi.org/10.15252/emmm.201809962
work_keys_str_mv AT annsaada seasquirtalternativeoxidasebypassesfatalmitochondrialheartdisease
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