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Sequence of the bovine 2-oxoglutarate carrier prtein: relation to other mitochondrial transport proteins
Clonaggio e struttura primaria del trasportatore mitocondriale del chetoglutarato medinate PCR
Identification in Saccharomyces cerevisiae of two isoforms of a novel mitochondrial transporter for 2-oxoadipate and oxoglutarate
IDENTIFICATION AND METABOLIC SIGNIFICANCE OF YEAST MITOCHONDRIAL TRASNPORTERS VIA BACTERIAL EXPRESSION, FUNCTIONAL RECONSTITUTION INTO PHOSPHOLIPID VESCICLES AND KNOCK-OUT STRAINS ANALYSIS
Identification of the human mitochondrial oxodicarboxylate carrier - Bacterial expression, reconstitution functional characterization, tissue distribution, and chromosomal location RID F-3159-2010 RID E-6420-2011
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
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