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Property "Has-20abstract" (as page type) with input value "Alterations-20to-20branched-2Dchain-20keto-20acid-20(BCKA)-20oxidation-20have-20been-20implicated-20in-20a-20wide-20variety-20of-20human-20diseases,-20ranging-20from-20diabetes-20to-20cancer.-20Although-20global-20shifts-20in-20BCKA-20metabolism-2Devident-20by-20gene-20transcription,-20metabolite-20profiling,-20and-20-27-27in-20vivo-27-27-20flux-20analyses-20have-20been-20documented-20across-20various-20pathological-20conditions,-20the-20underlying-20biochemical-20mechanism(s)-20within-20the-20mitochondrion-20remain-20largely-20unknown.-20-27-27In-20vitro-27-27-20experiments-20using-20isolated-20mitochondria-20represent-20a-20powerful-20biochemical-20tool-20for-20elucidating-20the-20role-20of-20the-20mitochondrion-20in-20driving-20disease.-20Such-20analyses-20have-20routinely-20been-20utilized-20across-20disciplines-20to-20shed-20valuable-20insight-20into-20mitochondrial-2Dlinked-20pathologies.-20That-20said,-20few-20studies-20have-20attempted-20to-20model-20-27-27in-20vitro-27-27-20BCKA-20oxidation-20in-20isolated-20organelles.-20The-20impetus-20for-20the-20present-20study-20stemmed-20from-20the-20knowledge-20that-20complete-20oxidation-20of-20each-20of-20the-20three-20BCKAs-20involves-20a-20reaction-20dependent-20upon-20bicarbonate-20and-20ATP,-20both-20of-20which-20are-20not-20typically-20included-20in-20respiration-20experiments.-20Based-20on-20this,-20it-20was-20hypothesized-20that-20the-20inclusion-20of-20exogenous-20bicarbonate-20and-20stimulation-20of-20respiration-20using-20physiological-20shifts-20in-20ATP-2Dfree-20energy,-20rather-20than-20excess-20ADP,-20would-20allow-20for-20maximal-20BCKA-2Dsupported-20respiratory-20flux-20in-20isolated-20mitochondria.-20This-20hypothesis-20was-20confirmed-20in-20mitochondria-20from-20several-20mouse-20tissues,-20including-20heart,-20liver-20and-20skeletal-20muscle.-20What-20follows-20is-20a-20thorough-20characterization-20and-20validation-20of-20a-20novel-20biochemical-20tool-20for-20investigating-20BCKA-20metabolism-20in-20isolated-20mitochondria.-0A-0A-3Csmall-3E©-202019-20The-20Author(s).-20Published-20by-20Portland-20Press-20Limited-20on-20behalf-20of-20the-20Biochemical-20Society.-3C-2Fsmall-3E" contains invalid characters or is incomplete and therefore can cause unexpected results during a query or annotation process.

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