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Goldberg 2019 Biochem J
Additional label 2019-06  + , Labels  +
Coupling states ET  + , LEAK  +
Enzyme TCA cycle and matrix dehydrogenases  +
Has abstract Alterations to branched-chain keto acid (B
Alterations to branched-chain keto acid (BCKA) oxidation have been implicated in a wide variety of human diseases, ranging from diabetes to cancer. Although global shifts in BCKA metabolism-evident by gene transcription, metabolite profiling, and ''in vivo'' flux analyses have been documented across various pathological conditions, the underlying biochemical mechanism(s) within the mitochondrion remain largely unknown. ''In vitro'' experiments using isolated mitochondria represent a powerful biochemical tool for elucidating the role of the mitochondrion in driving disease. Such analyses have routinely been utilized across disciplines to shed valuable insight into mitochondrial-linked pathologies. That said, few studies have attempted to model ''in vitro'' BCKA oxidation in isolated organelles. The impetus for the present study stemmed from the knowledge that complete oxidation of each of the three BCKAs involves a reaction dependent upon bicarbonate and ATP, both of which are not typically included in respiration experiments. Based on this, it was hypothesized that the inclusion of exogenous bicarbonate and stimulation of respiration using physiological shifts in ATP-free energy, rather than excess ADP, would allow for maximal BCKA-supported respiratory flux in isolated mitochondria. This hypothesis was confirmed in mitochondria from several mouse tissues, including heart, liver and skeletal muscle. What follows is a thorough characterization and validation of a novel biochemical tool for investigating BCKA metabolism in isolated mitochondria. <small>© 2019 The Author(s). Published by Portland Press Limited on behalf of the Biochemical Society.</small>
of the Biochemical Society.</small>  +
Has editor [[Plangger M]]  +
Has info [ PMID: 31092703]  +
Has publicationkeywords Amino acid metabolism  + , Bioenergetics  + , Buffer C  + , Electron transport chain  + , Mitochondria  +
Has title Goldberg EJ, Buddo KA, McLaughlin KL, Fern
Goldberg EJ, Buddo KA, McLaughlin KL, Fernandez RF, Pereyra AS, Psaltis CE, Lin CT, Hagen JT, Boykov IN, Nguyen TK, Gowdy KM, Ellis JM, Neufer PD, McClung JM, Fisher-Wellman KH (2019) Tissue-specific characterization of mitochondrial branched-chain keto acid oxidation using a multiplexed assay platform. Biochem J 476:1521-37.
xed assay platform. Biochem J 476:1521-37.  +
Instrument and method Oxygraph-2k  +
Mammal and model Mouse  +
MiP area Respiration  +
Pathways F  + , N  + , NS  + , Other combinations  + , ROX  + , S  +
Preparation Isolated mitochondria  +
Respiration and regulation ADP  + , ATP  + , PCr;Cr  +
Tissue and cell Heart  +
Was published by MiPNetLab US NC Greenville Neufer PD +
Was published in journal Biochem J +
Was published in year 2019  +
Was written by Goldberg EJ + , Buddo KA + , McLaughlin KL + , Fernandez RF + , Pereyra AS + , Psaltis CE + , Lin CT + , Hagen JT + , Boykov IN + , Nguyen TK + , Gowdy KM + , Ellis JM + , Neufer PD + , McClung JM + , Fisher-Wellman KH +
Categories Publications
Modification date
"Modification date" is a predefined property that corresponds to the date of the last modification of a subject and is provided by Semantic MediaWiki.
10:35:15, 4 June 2019  +
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