The Mitochondrial Calcium Uniporter (MCU): Molecular Identity and Role in Human Diseases

Calcium (Ca<sup>2+</sup>) ions act as a second messenger, regulating several cell functions. Mitochondria are critical organelles for the regulation of intracellular Ca<sup>2+</sup>. Mitochondrial calcium (mtCa<sup>2+</sup>) uptake is ensured by the presence in th...

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Main Authors: Donato D’Angelo, Rosario Rizzuto
Format: Article
Language:English
Published: MDPI AG 2023-08-01
Series:Biomolecules
Subjects:
Online Access:https://www.mdpi.com/2218-273X/13/9/1304
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author Donato D’Angelo
Rosario Rizzuto
author_facet Donato D’Angelo
Rosario Rizzuto
author_sort Donato D’Angelo
collection DOAJ
description Calcium (Ca<sup>2+</sup>) ions act as a second messenger, regulating several cell functions. Mitochondria are critical organelles for the regulation of intracellular Ca<sup>2+</sup>. Mitochondrial calcium (mtCa<sup>2+</sup>) uptake is ensured by the presence in the inner mitochondrial membrane (IMM) of the mitochondrial calcium uniporter (MCU) complex, a macromolecular structure composed of pore-forming and regulatory subunits. MtCa<sup>2+</sup> uptake plays a crucial role in the regulation of oxidative metabolism and cell death. A lot of evidence demonstrates that the dysregulation of mtCa<sup>2+</sup> homeostasis can have serious pathological outcomes. In this review, we briefly discuss the molecular structure and the function of the MCU complex and then we focus our attention on human diseases in which a dysfunction in mtCa<sup>2+</sup> has been shown.
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spelling doaj.art-7251f7219c8c4497aef3566a2f3ae3812023-11-19T09:45:05ZengMDPI AGBiomolecules2218-273X2023-08-01139130410.3390/biom13091304The Mitochondrial Calcium Uniporter (MCU): Molecular Identity and Role in Human DiseasesDonato D’Angelo0Rosario Rizzuto1Department of Biomedical Sciences, University of Padua, 35131 Padua, ItalyDepartment of Biomedical Sciences, University of Padua, 35131 Padua, ItalyCalcium (Ca<sup>2+</sup>) ions act as a second messenger, regulating several cell functions. Mitochondria are critical organelles for the regulation of intracellular Ca<sup>2+</sup>. Mitochondrial calcium (mtCa<sup>2+</sup>) uptake is ensured by the presence in the inner mitochondrial membrane (IMM) of the mitochondrial calcium uniporter (MCU) complex, a macromolecular structure composed of pore-forming and regulatory subunits. MtCa<sup>2+</sup> uptake plays a crucial role in the regulation of oxidative metabolism and cell death. A lot of evidence demonstrates that the dysregulation of mtCa<sup>2+</sup> homeostasis can have serious pathological outcomes. In this review, we briefly discuss the molecular structure and the function of the MCU complex and then we focus our attention on human diseases in which a dysfunction in mtCa<sup>2+</sup> has been shown.https://www.mdpi.com/2218-273X/13/9/1304MCUmitochondrial Ca<sup>2+</sup> signalingcancercardiovascular diseasesmetabolic diseasesskeletal muscle diseases
spellingShingle Donato D’Angelo
Rosario Rizzuto
The Mitochondrial Calcium Uniporter (MCU): Molecular Identity and Role in Human Diseases
Biomolecules
MCU
mitochondrial Ca<sup>2+</sup> signaling
cancer
cardiovascular diseases
metabolic diseases
skeletal muscle diseases
title The Mitochondrial Calcium Uniporter (MCU): Molecular Identity and Role in Human Diseases
title_full The Mitochondrial Calcium Uniporter (MCU): Molecular Identity and Role in Human Diseases
title_fullStr The Mitochondrial Calcium Uniporter (MCU): Molecular Identity and Role in Human Diseases
title_full_unstemmed The Mitochondrial Calcium Uniporter (MCU): Molecular Identity and Role in Human Diseases
title_short The Mitochondrial Calcium Uniporter (MCU): Molecular Identity and Role in Human Diseases
title_sort mitochondrial calcium uniporter mcu molecular identity and role in human diseases
topic MCU
mitochondrial Ca<sup>2+</sup> signaling
cancer
cardiovascular diseases
metabolic diseases
skeletal muscle diseases
url https://www.mdpi.com/2218-273X/13/9/1304
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