期刊
MOLECULAR ASPECTS OF MEDICINE
卷 34, 期 2-3, 页码 465-484出版社
ELSEVIER
DOI: 10.1016/j.mam.2012.05.005
关键词
Membrane transport; Mitochondrial carrier; Mitochondrial carrier disease; Mitochondrial carrier family; Mitochondrial transporter; SLC25
资金
- MIUR
- CNR
- CEGBA
- EC
- Italian Human ProteomeNet
- Comitato Telethon Fondazione Onlus [GGP11139]
- Swiss National Science Foundation through the National Center of Competence in Research (NCCR) TransCure, University of Bern, Switzerland
SLC25 is a large family of nuclear-encoded transporters embedded in the inner mitochondrial membrane and in a few cases other organelle membranes. The members of this superfamily are widespread in eukaryotes and involved in numerous metabolic pathways and cell functions. They can be easily recognized by their striking sequence features, i.e., a tripartite structure, six transmembrane alpha-helices and a 3-fold repeated signature motifs. SLC25 members vary greatly in the nature and size of their transported substrates, modes of transport (i.e., uniport, symport or antiport) and driving forces, although the molecular mechanism of substrate translocation may be basically the same. Based on substrate specificity, 24 subfamilies, well conserved throughout evolution, have been functionally characterized mainly by transport assays upon heterologous gene expression, purification and reconstitution into liposomes. Several other SLC25 family members remain to be characterized. In recent years mutations in the SLC25 genes have been shown to be responsible for 11 diseases, highlighting the important role of SLC25 in metabolism. (C) 2012 Elsevier Ltd. All rights reserved.
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