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

Author:

D’Angelo Donato1ORCID,Rizzuto Rosario12

Affiliation:

1. Department of Biomedical Sciences, University of Padua, 35131 Padua, Italy

2. National Center on Gene Therapy and RNA-Based Drugs, 35131 Padua, Italy

Abstract

Calcium (Ca2+) ions act as a second messenger, regulating several cell functions. Mitochondria are critical organelles for the regulation of intracellular Ca2+. Mitochondrial calcium (mtCa2+) 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. MtCa2+ uptake plays a crucial role in the regulation of oxidative metabolism and cell death. A lot of evidence demonstrates that the dysregulation of mtCa2+ 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 mtCa2+ has been shown.

Funder

Italian Ministry of University and Research (PRIN, PNRR MUR Mission 4, Component 2) and of Health

Cariparo Foundation

Italian Association for Cancer Research

Telethon-Italy

Publisher

MDPI AG

Subject

Molecular Biology,Biochemistry

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