RecName: Full=2-oxoisovalerate dehydrogenase subunit alpha, mitochondr - Protein

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Neonatal gene therapy achieves sustained disease rescue of maple syrup urine disease in mice. [Nat Commun. 2022]
Neonatal gene therapy achieves sustained disease rescue of maple syrup urine disease in mice.
Pontoizeau C, Simon-Sola M, Gaborit C, Nguyen V, Rotaru I, Tual N, Colella P, Girard M, Biferi MG, Arnoux JB, et al. Nat Commun. 2022 Jun 7; 13(1):3278. Epub 2022 Jun 7.
Effects of insulin on the regulation of branched-chain alpha-keto acid dehydrogenase E1 alpha subunit gene expression. [Biochem J. 1996]
Effects of insulin on the regulation of branched-chain alpha-keto acid dehydrogenase E1 alpha subunit gene expression.
Costeas PA, Chinsky JM. Biochem J. 1996 Aug 15; 318 ( Pt 1)(Pt 1):85-92.
BCKDH kinase promotes hepatic gluconeogenesis independent of BCKDHA. [Cell Death Dis. 2024]
BCKDH kinase promotes hepatic gluconeogenesis independent of BCKDHA.
Zhou F, Sheng C, Ma X, Li T, Ming X, Wang S, Tan J, Yang Y, Sun H, Lu J, et al. Cell Death Dis. 2024 Oct 10; 15(10):736. Epub 2024 Oct 10.

RefSeq protein isoforms
See 5 reference sequence protein isoforms for the Bckdha gene.

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MODBASE, Database of Comparative Protein Structure Models (Sali Lab/UCSF) [MODBASE, Database of Comparat...]
MODBASE, Database of Comparative Protein Structure Models (Sali Lab/UCSF)
Mouse Genome Informatics (MGI) [Mouse Genome Informatics (MGI)]
Mouse Genome Informatics (MGI)
Transcript/Protein Information [PANTHER Classification System]
Transcript/Protein Information
PANTHER Classification System
reagent reviews [ExactAntigen/Labome]
reagent reviews
ExactAntigen/Labome
reagents [ExactAntigen/Labome]
reagents
ExactAntigen/Labome

RecName: Full=2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial; AltNa...
RecName: Full=2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial; AltName: Full=Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain; Short=BCKDE1A; Short=BCKDH E1-alpha; Flags: Precursor
gi|1709439|sp|P50136.1|ODBA_MOUSE

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