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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A mitochondrial origin for frontotemporal dementia and amyotrophic lateral sclerosis through CHCHD10 involvement
Sylvie Bannwarth,Samira Ait-El-Mkadem,Annabelle Chaussenot,Emmanuelle C. Genin,Sandra Lacas-Gervais,Konstantina Fragaki,Laetitia Berg-Alonso,Yusuke Kageyama,Valérie Serre,David Moore,Annie Verschueren,Cécile Rouzier,Isabelle Le Ber,Gaëlle Augé,Charlotte Cochaud,Françoise Lespinasse,Karine Nguyen,Anne de Septenville,Alexis Brice,Patrick Yu-Wai-Man,Hiromi Sesaki,Jean Pouget,Véronique Paquis-Flucklinger +22 more
TL;DR: A large family with a late-onset phenotype including motor neuron disease, cognitive decline resembling frontotemporal dementia, cerebellar ataxia and myopathy is reported, showing that mitochondrial disease may be at the origin of some of these phenotypes.
Journal ArticleDOI
Cardiolipin, the heart of mitochondrial metabolism.
TL;DR: An overview of cardiolipin metabolism, function and its role in different pathological states is provided.
Journal ArticleDOI
Dopaminergic neuronal loss, reduced neurite complexity and autophagic abnormalities in transgenic mice expressing G2019S mutant LRRK2.
David Ramonet,João Paulo Lima Daher,João Paulo Lima Daher,Brian M. Lin,Klodjan Stafa,Jaekwang Kim,Jaekwang Kim,Rebecca Banerjee,Marie Westerlund,Olga Pletnikova,Liliane Glauser,Lichuan Yang,Ying Liu,Deborah A. Swing,M. Flint Beal,Juan C. Troncoso,J. Michael McCaffery,Nancy A. Jenkins,Nancy A. Jenkins,Neal G. Copeland,Neal G. Copeland,Dagmar Galter,Bobby Thomas,Michael K. Lee,Michael K. Lee,Ted M. Dawson,Valina L. Dawson,Darren J. Moore +27 more
TL;DR: This study demonstrates that expression of G2019S mutant LRRK2 induces the degeneration of nigrostriatal pathway dopaminergic neurons in an age-dependent manner, and will provide important tools for understanding the mechanism(s) through which familial mutations precipitate neuronal degeneration and PD.
Journal ArticleDOI
Imbalanced OPA1 processing and mitochondrial fragmentation cause heart failure in mice.
Timothy Wai,Jaime García-Prieto,Michael J. Baker,Carsten Merkwirth,Paule Bénit,Pierre Rustin,Pierre Rustin,Francisco J. Rupérez,Coral Barbas,Borja Ibanez,Thomas Langer +10 more
TL;DR: The role of OPA1 processing and mitochondrial fragmentation in the heart, a metabolically demanding organ that depends critically on mitochondrial functions, is examined and it is discovered that additional deletion of Oma1 in cardiomyocytes prevented OPA 1 processing altogether and restored normal mitochondrial morphology and cardiac health.
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Dual Role of Mitofilin in Mitochondrial Membrane Organization and Protein Biogenesis
Karina von der Malsburg,Judith M. Müller,Maria Bohnert,Silke Oeljeklaus,Paulina Kwiatkowska,Paulina Kwiatkowska,Thomas Becker,Adrianna Loniewska-Lwowska,Sebastian Wiese,Sanjana Rao,Dusanka Milenkovic,Dana P. Hutu,Ralf M. Zerbes,Agnes Schulze-Specking,Helmut E. Meyer,Jean-Claude Martinou,Sabine Rospert,Peter Rehling,Peter Rehling,Chris Meisinger,Marten Veenhuis,Bettina Warscheid,Ida J. van der Klei,Nikolaus Pfanner,Agnieszka Chacinska,Agnieszka Chacinska,Martin van der Laan +26 more
TL;DR: The findings indicate that mitofilin is a central component of MINOS and functions as a multifunctional regulator of mitochondrial architecture and protein biogenesis.
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.
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.