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
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Journal ArticleDOI
OPA1 functions in mitochondria and dysfunctions in optic nerve
Guy Lenaers,Pascal Reynier,Ghizlane Elachouri,Chadi Soukkarieh,Aurélien Olichon,Pascale Belenguer,Laurent Baricault,Bernard Ducommun,Christian P. Hamel,Cécile Delettre +9 more
TL;DR: The fundamental knowledge on OPA1 functions and regulations are reviewed, highlighting their involvements in mitochondrial respiration, membrane dynamic and apoptosis, and the remarkable RGC mitochondrial network physiology is described.
Journal ArticleDOI
OPA1-related auditory neuropathy: Site of lesion and outcome of cochlear implantation
Rosamaria Santarelli,Roberta Rossi,Pietro Scimemi,Elona Cama,Maria Lucia Valentino,Chiara La Morgia,Leonardo Caporali,Rocco Liguori,Vincenzo Magnavita,Anna Monteleone,Ariella Biscaro,Edoardo Arslan,Valerio Carelli +12 more
TL;DR: Cochlear implantation improves speech perception and synchronous activation of auditory pathways in patients carrying OPA1 missense mutations by bypassing the lesion site.
Journal ArticleDOI
Mitochondrial dysfunction in glaucoma: Understanding genetic influences
Gerassimos Lascaratos,David F. Garway-Heath,Colin E. Willoughby,Kai-Yin Chau,Anthony H.V. Schapira +4 more
TL;DR: It is proposed that a variety of genes may each confer a background susceptibility to POAG in different populations having one common link: mitochondrial dysfunction, which contributes to the pathogenesis of POAG.
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Restoration of L-OPA1 alleviates acute ischemic stroke injury in rats via inhibiting neuronal apoptosis and preserving mitochondrial function.
Yongxing Lai,Peiqiang Lin,Manli Chen,Yixian Zhang,Jianhao Chen,Mouwei Zheng,Ji Liu,Houwei Du,Ronghua Chen,Xiaodong Pan,Nan Liu,Hongbin Chen +11 more
TL;DR: The results demonstrate that the stabilization of L-OPA1 protects ischemic brains by reducing neuronal apoptosis and preserving mitochondrial function, suggesting its significance as a promising therapeutic target for stroke prevention and treatment.
Journal ArticleDOI
Mitochondrial dynamics in health and disease.
TL;DR: In this article, a review of the machinery involved in mitochondrial dynamics and their dysfunction in disease is discussed, and the major components of the fission and fusion machineries including dynamin-related protein 1 (DRP1), mitofusins 1 and 2 (MFN1, MFN2) and optic atrophy protein 1(OPA1) and ensuing imbalance of mitochondrial dynamics can lead to neurodegenerative disease.
References
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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.
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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.