The SLC25 Carrier Family: Important Transport Proteins in Mitochondrial Physiology and Pathology

Physiology (Bethesda). 2020 Sep 1;35(5):302-327. doi: 10.1152/physiol.00009.2020.

Abstract

Members of the mitochondrial carrier family (SLC25) transport a variety of compounds across the inner membrane of mitochondria. These transport steps provide building blocks for the cell and link the pathways of the mitochondrial matrix and cytosol. An increasing number of diseases and pathologies has been associated with their dysfunction. In this review, the molecular basis of these diseases is explained based on our current understanding of their transport mechanism.

Keywords: bioenergetics; impaired transport mechanism; mitochondrial disease; mitochondrial physiology; pathological mutations.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Biological Transport
  • Energy Metabolism*
  • Humans
  • Mitochondria / genetics
  • Mitochondria / metabolism*
  • Mitochondria / pathology
  • Mitochondrial Diseases / genetics
  • Mitochondrial Diseases / metabolism*
  • Mitochondrial Diseases / pathology
  • Mitochondrial Membranes / metabolism*
  • Mitochondrial Membranes / pathology
  • Mitochondrial Proteins / genetics
  • Mitochondrial Proteins / metabolism*
  • Mutation, Missense
  • Organic Anion Transporters / genetics
  • Organic Anion Transporters / metabolism*

Substances

  • Mitochondrial Proteins
  • Organic Anion Transporters