Molecular structure and pathophysiological roles of the Mitochondrial Calcium Uniporter.

TitleMolecular structure and pathophysiological roles of the Mitochondrial Calcium Uniporter.
Publication TypeJournal Article
Year of Publication2016
AuthorsMammucari, C, Raffaello, A, Reane, DVecellio, Rizzuto, R
JournalBiochim Biophys Acta
Volume1863
Issue10
Pagination2457-64
Date Published2016 10
ISSN0006-3002
KeywordsAnimals, Calcium, Calcium Channels, Calcium Signaling, Calcium-Binding Proteins, Disease Models, Animal, Humans, Ion Transport, Mice, Mice, Knockout, Mice, Transgenic, Mitochondria, Heart, Mitochondria, Muscle, Mitochondrial Diseases, Mitochondrial Membrane Transport Proteins, Mitochondrial Membranes, Muscle Proteins, Organ Specificity, Protein Conformation
Abstract

Mitochondrial Ca(2+) uptake regulates a wide array of cell functions, from stimulation of aerobic metabolism and ATP production in physiological settings, to induction of cell death in pathological conditions. The molecular identity of the Mitochondrial Calcium Uniporter (MCU), the highly selective channel responsible for Ca(2+) entry through the IMM, has been described less than five years ago. Since then, research has been conducted to clarify the modulation of its activity, which relies on the dynamic interaction with regulatory proteins, and its contribution to the pathophysiology of organs and tissues. Particular attention has been placed on characterizing the role of MCU in cardiac and skeletal muscles. In this review we summarize the molecular structure and regulation of the MCU complex in addition to its pathophysiological role, with particular attention to striated muscle tissues. This article is part of a Special Issue entitled: Mitochondrial Channels edited by Pierre Sonveaux, Pierre Maechler and Jean-Claude Martinou.

DOI10.1016/j.bbamcr.2016.03.006
Alternate JournalBiochim. Biophys. Acta
Citation Key10.1016/j.bbamcr.2016.03.006
PubMed ID26968367
Grant List1P01AG025532-01A1 / NH / NIH HHS / United States