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MCU mitochondria calcium emre micu1

Julia C Liu, Jie Liu, Kira M Holmström, Sara Menazza, Randi J Parks, Maria M Fergusson, Zu-Xi Yu, Danielle A Springer, Charles Halsey, Chengyu Liu, Elizabeth Murphy, Toren Finkel
MICU1 is a component of the mitochondrial calcium uniporter, a multiprotein complex that also includes MICU2, MCU, and EMRE. Here, we describe a mouse model of MICU1 deficiency. MICU1(-/-) mitochondria demonstrate altered calcium uptake, and deletion of MICU1 results in significant, but not complete, perinatal mortality. Similar to afflicted patients, viable MICU1(-/-) mice manifest marked ataxia and muscle weakness. Early in life, these animals display a range of biochemical abnormalities, including increased resting mitochondrial calcium levels, altered mitochondrial morphology, and reduced ATP...
August 9, 2016: Cell Reports
Alexander I Bondarenko, Warisara Parichatikanond, Corina T Madreiter, Rene Rost, Markus Waldeck-Weiermair, Roland Malli, Wolfgang F Graier
The mitochondrial Ca(2+) uniporter is a highly Ca(2+)-selective protein complex that consists of the pore-forming mitochondrial Ca(2+) uniporter protein (MCU), the scaffolding essential MCU regulator (EMRE), and mitochondrial calcium uptake 1 and 2 (MICU1/2), which negatively regulate mitochondrial Ca(2+) uptake. We have previously reported that uncoupling proteins 2 and 3 (UCP2/3) are also engaged in the activity of mitochondrial Ca(2+) uptake under certain conditions, while the mechanism by which UCP2/3 facilitates mitochondrial Ca(2+) uniport remains elusive...
December 2015: Pflügers Archiv: European Journal of Physiology
Erika Kovács-Bogdán, Yasemin Sancak, Kimberli J Kamer, Molly Plovanich, Ashwini Jambhekar, Robert J Huber, Michael A Myre, Michael D Blower, Vamsi K Mootha
The mitochondrial calcium uniporter is a highly selective calcium channel distributed broadly across eukaryotes but absent in the yeast Saccharomyces cerevisiae. The molecular components of the human uniporter holocomplex (uniplex) have been identified recently. The uniplex consists of three membrane-spanning subunits--mitochondrial calcium uniporter (MCU), its paralog MCUb, and essential MCU regulator (EMRE)--and two soluble regulatory components--MICU1 and its paralog MICU2. The minimal components sufficient for in vivo uniporter activity are unknown...
June 17, 2014: Proceedings of the National Academy of Sciences of the United States of America
Maria Patron, Vanessa Checchetto, Anna Raffaello, Enrico Teardo, Denis Vecellio Reane, Maura Mantoan, Veronica Granatiero, Ildikò Szabò, Diego De Stefani, Rosario Rizzuto
Mitochondrial calcium accumulation was recently shown to depend on a complex composed of an inner-membrane channel (MCU and MCUb) and regulatory subunits (MICU1, MCUR1, and EMRE). A fundamental property of MCU is low activity at resting cytosolic Ca(2+) concentrations, preventing deleterious Ca(2+) cycling and organelle overload. Here we demonstrate that these properties are ensured by a regulatory heterodimer composed of two proteins with opposite effects, MICU1 and MICU2, which, both in purified lipid bilayers and in intact cells, stimulate and inhibit MCU activity, respectively...
March 6, 2014: Molecular Cell
Saverio Marchi, Paolo Pinton
Although it has long been known that mitochondria take up Ca2+, the molecular identities of the channels and transporters involved in this process were revealed only recently. Here, we discuss the recent work that has led to the characterization of the mitochondrial calcium uniporter complex, which includes the channel-forming subunit MCU (mitochondrial calcium uniporter) and its regulators MICU1, MICU2, MCUb, EMRE, MCUR1 and miR-25. We review not only the biochemical identities and structures of the proteins required for mitochondrial Ca2+ uptake but also their implications in different physiopathological contexts...
March 1, 2014: Journal of Physiology
Yasemin Sancak, Andrew L Markhard, Toshimori Kitami, Erika Kovács-Bogdán, Kimberli J Kamer, Namrata D Udeshi, Steven A Carr, Dipayan Chaudhuri, David E Clapham, Andrew A Li, Sarah E Calvo, Olga Goldberger, Vamsi K Mootha
The mitochondrial uniporter is a highly selective calcium channel in the organelle's inner membrane. Its molecular components include the EF-hand-containing calcium-binding proteins mitochondrial calcium uptake 1 (MICU1) and MICU2 and the pore-forming subunit mitochondrial calcium uniporter (MCU). We sought to achieve a full molecular characterization of the uniporter holocomplex (uniplex). Quantitative mass spectrometry of affinity-purified uniplex recovered MICU1 and MICU2, MCU and its paralog MCUb, and essential MCU regulator (EMRE), a previously uncharacterized protein...
December 13, 2013: Science
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