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
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The molecular mechanism of mitochondrial fusion.
Suzanne Hoppins,Jodi Nunnari +1 more
TL;DR: Observations from both yeast and mammalian cells are presented that have provided insights into the mechanism of mitochondrial fusion and speculate on how the key players, which are dynamin-related GTPases do the work of membrane tethering and fusion.
Journal ArticleDOI
Role of the mitochondrial contact site and cristae organizing system in membrane architecture and dynamics
TL;DR: The mitochondrial contact site and cristae organizing system (MICOS) is located at crista junctions where it stabilizes membrane curvature and forms contact sites between the mitochondrial inner and outer membranes.
Journal ArticleDOI
Mitochondrial fusion but not fission regulates larval growth and synaptic development through steroid hormone production
Hector Sandoval,Chi-Kuang Yao,Kuchuan Chen,Manish Jaiswal,Taraka R. Donti,Yong Qi Lin,Vafa Bayat,Bo Xiong,Ke Zhang,Gabriela David,Wu Lin Charng,Shinya Yamamoto,Lita Duraine,Brett H. Graham,Hugo J. Bellen +14 more
TL;DR: The data show that Marf and Mitofusins share an evolutionarily conserved role in mitochondrial transport, cholesterol ester storage and steroid-hormone synthesis.
Journal ArticleDOI
The Parkinson Disease Mitochondrial Hypothesis Where Are We at
TL;DR: Current understanding of Parkinson’s disease–related mitochondrial dysfunction, including bioenergetic defects, mitochondrial DNA alterations, altered mitochondrial dynamics, activation of mitochondrial-dependent programmed cell death, and perturbations in mitochondrial tethering to the endoplasmic reticulum are discussed.
Journal ArticleDOI
OPA1-associated disorders: phenotypes and pathophysiology.
Patrizia Amati-Bonneau,D. Milea,Dominique Bonneau,Dominique Bonneau,Arnaud Chevrollier,Marc Ferré,Virginie Guillet,Virginie Guillet,Naïg Gueguen,Dominique Loiseau,Dominique Loiseau,Marie-Anne Pou de Crescenzo,Marie-Anne Pou de Crescenzo,Christophe Verny,Vincent Procaccio,Guy Lenaers,Pascal Reynier,Pascal Reynier +17 more
TL;DR: The study of the various clinical presentations of ADOA in conjunction with the investigation of OPA1 mutations in fibroblasts from patients with optic atrophy provides new insights into the pathophysiological mechanisms of the disease while underscoring the multiple physiological roles played by OPA 1 in energetic metabolism, mitochondrial structure and maintenance, and cell death.
References
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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.
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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.