OPA1 Controls Apoptotic Cristae Remodeling Independently from Mitochondrial Fusion
Christian Frezza,Sara Cipolat,Olga Martins de Brito,Massimo Micaroni,Galina V. Beznoussenko,Tomasz Rudka,Davide Bartoli,Roman S. Polishuck,Nika N. Danial,Bart De Strooper,Luca Scorrano +10 more
TLDR
Evidence is provided that Optic Atrophy 1 (OPA1), a profusion dynamin-related protein of the inner mitochondrial membrane mutated in dominant optic atrophy, protects from apoptosis by preventing cytochrome c release independently from mitochondrial fusion.About:
This article is published in Cell.The article was published on 2006-07-14 and is currently open access. It has received 1444 citations till now. The article focuses on the topics: Optic Atrophy 1 & Inner mitochondrial membrane.read more
Citations
More filters
Journal ArticleDOI
Mitochondrial dismissal in mammals, from protein degradation to mitophagy.
TL;DR: An overview of mitochondrial stability and quality control mechanisms is provided, highlighting mitophagy, and emphasizing the central role of mitochondrial dynamics in this context.
Journal ArticleDOI
Optic atrophy 1 mediates mitochondria remodeling and dopaminergic neurodegeneration linked to complex I deficiency
David Ramonet,Celine Perier,Ariadna Recasens,Benjamin Dehay,Jordi Bové,Veronica Costa,Luca Scorrano,Miquel Vila,Miquel Vila +8 more
TL;DR: It is shown that complex I inhibition by parkinsonian neurotoxins leads to an oxidative-dependent disruption of OPA1 oligomeric complexes that normally keep mitochondrial cristae junctions tight, and this identifies OPA 1 as molecular link between complex I deficiency and alterations in mitochondrial dynamics machinery.
Journal ArticleDOI
Regulation of Skeletal Muscle Oxidative Capacity and Insulin Signaling by the Mitochondrial Rhomboid Protease PARL
Anthony E. Civitarese,Anthony E. Civitarese,Anthony E. Civitarese,Paul S. MacLean,Stacy Carling,Lyndal Kerr-Bayles,Ryan P. McMillan,Anson Pierce,Thomas C. Becker,Cedric Moro,Jean Finlayson,Natalie Lefort,Christopher B. Newgard,Lawrence J. Mandarino,Lawrence J. Mandarino,William T. Cefalu,Ken Walder,Greg R. Collier,Greg R. Collier,Matthew W. Hulver,Steven R. Smith,Eric Ravussin +21 more
TL;DR: It is proposed that lower PARL expression may contribute to the mitochondrial abnormalities seen in aging and T2DM.
OPA1 mutations cause cytochrome c oxidase deficiency due to loss of wild-type mtDNA
TL;DR: The OPA1 mutations cause autosomal dominant optic atrophy (DOA), a condition characterized by preferential loss of retinal ganglion cells and progressive optic nerve degeneration.
Journal ArticleDOI
Disorders of mitochondrial function
TL;DR: This review highlights recent clinically important developments in diagnostic evaluation and treatment of mitochondrial diseases, a major category of childhood illness that produce a wide variety of symptoms and multisystemic disorders.
References
More filters
Journal ArticleDOI
Proapoptotic BAX and BAK: A Requisite Gateway to Mitochondrial Dysfunction and Death
Michael C. Wei,Michael C. Wei,Wei-Xing Zong,Emily H. Cheng,Tullia Lindsten,Vily Panoutsakopoulou,Andrea J. Ross,Kevin A. Roth,Grant R. MacGregor,Craig B. Thompson,Stanley J. Korsmeyer +10 more
TL;DR: In this article, the authors found that doubly deficient cells are resistant to multiple apoptotic stimuli that act through disruption of mitochondrial function: staurosporine, ultraviolet radiation, growth factor deprivation, etoposide, and the endoplasmic reticulum stress stimuli thapsigargin and tunicamycin.
Journal ArticleDOI
The Pathophysiology of Mitochondrial Cell Death
Douglas R. Green,Guido Kroemer +1 more
TL;DR: The therapeutic induction of MOMP may restore apoptosis in cancer cells in which it is disabled, and the general rules governing the pathophysiology and controversial issues regarding its regulation are discussed.
Journal ArticleDOI
Two CD95 (APO-1/Fas) signaling pathways
Carsten Scaffidi,Simone Fulda,Anu Srinivasan,Claudia Friesen,Feng Li,Kevin J. Tomaselli,Klaus-Michael Debatin,Peter H. Krammer,Marcus E. Peter +8 more
TL;DR: In the presence of caspase‐3 the amount of active casp enzyme‐8 generated at the DISC determines whether a mitochondria‐independent apoptosis pathway is used (type I cells) or not (type II cells).
Journal Article
The expanding role of mitochondria in apoptosis
TL;DR: The complexity of the apoptotic program began to increase with the discovery of Bcl-2, a gene whose product causes resistance to apoptosis in lymphocytes, and the complex role of mitochondria in apoptosis came into focus when biochemical studies identified several mitochondrial proteins that are able to activate cellular apoptotic programs directly.
Journal ArticleDOI
Mitofusins Mfn1 and Mfn2 coordinately regulate mitochondrial fusion and are essential for embryonic development
TL;DR: It is concluded that Mfn1 and Mfn2 have both redundant and distinct functions and act in three separate molecular complexes to promote mitochondrial fusion, and by enabling cooperation between mitochondria, has protective effects on the mitochondrial population.