Luigi Palmieri
Medical Research Council
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Featured researches published by Luigi Palmieri.
Journal of Biological Chemistry | 1999
Luigi Palmieri; A. Vozza; Gennaro Agrimi; V. De Marco; Michael J. Runswick; Ferdinando Palmieri; John E. Walker
Saccharomyces cerevisiae encodes 35 members of the mitochondrial carrier family, including the OAC protein. The transport specificities of some family members are known, but most are not. The function of the OAC has been revealed by overproduction inEscherichia coli, reconstitution into liposomes, and demonstration that the proteoliposomes transport malonate, oxaloacetate, sulfate, and thiosulfate. Reconstituted OAC catalyzes both unidirectional transport and exchange of substrates. In S. cerevisiae, OAC is in inner mitochondrial membranes, and deletion of its gene greatly reduces transport of oxaloacetate sulfate, thiosulfate, and malonate. Mitochondria from wild-type cells swelled in isoosmotic solutions of ammonium salts of oxaloacetate, sulfate, thiosulfate, and malonate, indicating that these anions are cotransported with protons. Overexpression of OAC in the deletion strain increased greatly the [35S]sulfate/sulfate and [35S]sulfate/oxaloacetate exchanges in proteoliposomes reconstituted with digitonin extracts of mitochondria. The main physiological role of OAC appears to be to use the proton-motive force to take up into mitochondria oxaloacetate produced from pyruvate by cytoplasmic pyruvate carboxylase.
Journal of Biological Chemistry | 2001
Giuseppe Fiermonte; Vincenza Dolce; Luigi Palmieri; Mario Ventura; Michael J. Runswick; Ferdinando Palmieri; John E. Walker
In Saccharomyces cerevisiae, the genes ODC1 and ODC2 encode isoforms of the oxodicarboxylate carrier. They both transport C5-C7 oxodicarboxylates across the inner membranes of mitochondria and are members of the family of mitochondrial carrier proteins. Orthologs are encoded in the genomes of Caenorhabditis elegans and Drosophila melanogaster, and a human expressed sequence tag (EST) encodes part of a closely related protein. Information from the EST has been used to complete the human cDNA sequence. This sequence has been used to map the gene to chromosome 14q11.2 and to show that the gene is expressed in all tissues that were examined. The human protein was produced by overexpression in Escherichia coli, purified, and reconstituted into phospholipid vesicles. It has similar transport characteristics to the yeast oxodicarboxylate carrier proteins (ODCs). Both the human and yeast ODCs catalyzed the transport of the oxodicarboxylates 2-oxoadipate and 2-oxoglutarate by a counter-exchange mechanism. Adipate, glutarate, and to a lesser extent, pimelate, 2-oxopimelate, 2-aminoadipate, oxaloacetate, and citrate were also transported by the human ODC. The main differences between the human and yeast ODCs are that 2-aminoadipate is transported by the former but not by the latter, whereas malate is transported by the yeast ODCs but not by the human ortholog. In mammals, 2-oxoadipate is a common intermediate in the catabolism of lysine, tryptophan, and hydroxylysine. It is transported from the cytoplasm into mitochondria where it is converted into acetyl-CoA. Defects in human ODC are likely to be a cause of 2-oxoadipate acidemia, an inborn error of metabolism of lysine, tryptophan, and hydroxylysine.
Journal of Biological Chemistry | 2002
Giuseppe Fiermonte; Luigi Palmieri; Simona Todisco; Gennaro Agrimi; Ferdinando Palmieri; John E. Walker
Journal of Biological Chemistry | 2001
Luigi Palmieri; Gennaro Agrimi; Michael J. Runswick; Ian M. Fearnley; Ferdinando Palmieri; John E. Walker
Archive | 2002
John E. Walker; Michael J. Runswick; Ferdinando Palmieri; Luigi Palmieri; Gennaro Agrimi
Archive | 2017
Pasquale Scarcia; Zampi Giuseppina; Vito Porcelli; Ruggiero Gorgoglione; Saada Ann; Elpeleg Orly; Chaouch Amina; Lochmuller Hanns; Di Schiavi Elia; Luigi Palmieri
Archive | 2015
Gennaro Agrimi; Isabella Pisano; Maria Antonietta Ricci; Luigi Palmieri
Archive | 2012
Gennaro Agrimi; Isabella Pisano; Luigi Palmieri
Archive | 2010
Alessandra Castegna; Pasquale Scarcia; Gennaro Agrimi; Luigi Palmieri; Hanspeter Rottensteiner; Iolanda Spera; Lucrezia Germinario; Ferdinando Palmieri
Archive | 2009
Carlo M.T. Marobbio; Luigi Palmieri; Ferdinando Palmieri; Antonella Santoro