期刊
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH
卷 1863, 期 10, 页码 2362-2378出版社
ELSEVIER SCIENCE BV
DOI: 10.1016/j.bbamcr.2016.03.007
关键词
Mitochondrial carrier; Mitochondrial transporter; SLC25; Mitochondria; Transport; Mitochondrial carrier-associated diseases
资金
- Ministero dell'Istruzione, dell'Universita e della Ricerca (MIUR)
- Center of Excellence on Comparative Genomics (CEGBA)
- European Social Fund
- Italian Human ProteomeNet [RBRN07BMCT_009]
- Comitato Telethon Fondazione Onlus [GGP11139]
Mitochondrial carriers (MCs) are a superfamily of nuclear-encoded proteins that are mostly localized in the inner mitochondrial membrane and transport numerous metabolites, nucleotides, cofactors and inorganic anions. Their unique sequence features, i.e., a tripartite structure, six transmembrane a-helices and a three-fold repeated signature motif, allow MCs to be easily recognized. This review describes how the functions of MCs from Saccharomyces cerevisiae, Homo sapiens and Arabidopsis thaliana (listed in the first table) were discovered after the genome sequence of S. cerevisiae was determined in 1996. In the genomic era, more than 50 previously unknown MCs from these organisms have been identified and characterized biochemically using a method consisting of gene expression, purification of the recombinant proteins, their reconstitution into liposomes and transport assays (EPRA). Information derived from studies with intact mitochondria, genetic and metabolic evidence, sequence similarity, phylogenetic analysis and complementation of knockout phenotypes have guided the choice of substrates that were tested in the transport assays. In addition, the diseases associated to defects of human MCs have been briefly reviewed. This article is part of a Special Issue entitled: Mitochondrial Channels edited by Pierre Sonveaux, Pierre Maechler and Jean-Claude Martinou. (C) 2016 Elsevier B.V. All rights reserved.
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