biostudies-literatureFahrenbach JPAmerican Heart AssociationNCATS NIH HHSNational Heart, Lung, and Blood InstituteNHLBI NIH HHSNIAMS NIH HHS2804-15https://www.ebi.ac.uk/biostudies/studies/S-EPMC4062826biostudies-literatureUnknown28(7)The newborn heart adapts to postnatal life by shifting from a fetal glycolytic metabolism to a mitochondrial oxidative metabolism. Abcc9, an ATP-binding cassette family member, increases expression concomitant with this metabolic shift. Abcc9 encodes a membrane-associated receptor that partners with a potassium channel to become the major potassium-sensitive ATP channel in the heart. Abcc9 also encodes a smaller protein enriched in the mitochondria. We now deleted exon 5 of Abcc9 to ablate expression of both plasma membrane and mitochondria-associated Abcc9-encoded proteins, and found that the myocardium failed to acquire normal mature metabolism, resulting in neonatal cardiomyopathy. Unlike wild-type neonatal cardiomyocytes, mitochondria from Ex5 cardiomyocytes were unresponsive to the KAFASEB journal : official publication of the Federation of American Societies for Experimental BiologyAbcc9 is required for the transition to oxidative metabolism in the newborn heart.PMC4062826F32‐HL097587R01 HL078926R21 HL093626F32-HL09758711GRNT7600070UL1 TR000430K08 HL098565U54 AR052646R01‐HL57414R21‐HL936260630268NR21-HL93626R01 HL057414F32 HL097587K08-HL098565R01‐HL078926R01-HL078926R01-HL57414K08‐HL098565Yerokun BMakielski JCMcNally EMShi NQKim GStoller DAggarwal NHadhazy MFahrenbach JPEarley JUfalseAbcc9 is required for the transition to oxidative metabolism in the newborn heart.The newborn heart adapts to postnatal life by shifting from a fetal glycolytic metabolism to a mitochondrial oxidative metabolism. Abcc9, an ATP-binding cassette family member, increases expression concomitant with this metabolic shift. Abcc9 encodes a membrane-associated receptor that partners with a potassium channel to become the major potassium-sensitive ATP channel in the heart. Abcc9 also encodes a smaller protein enriched in the mitochondria. We now deleted exon 5 of Abcc9 to ablate expression of both plasma membrane and mitochondria-associated Abcc9-encoded proteins, and found that the myocardium failed to acquire normal mature metabolism, resulting in neonatal cardiomyopathy. Unlike wild-type neonatal cardiomyocytes, mitochondria from Ex5 cardiomyocytes were unresponsive to the KA2014-01-01T00:00:00Z2014 Jul2025-04-22T17:39:49.671Z2019-03-27T01:30:26ZS-EPMC40628262464854510.1096/fj.13-244459