Gene Ontology annotation through association of InterPro records with GO terms
Manual transfer of experimentally-verified manual GO annotation data to orthologs by curator judgment of sequence similarity
Annotation inferences using phylogenetic trees
Gene Ontology annotation based on curation of immunofluorescence data
Automatic transfer of experimentally verified manual GO annotation data to orthologs using Ensembl Compara
Electronic Gene Ontology annotations created by ARBA machine learning models
Combined Automated Annotation using Multiple IEA Methods
The layered structure of human mitochondrial DNA nucleoids.
Proteomic analysis of increased Parkin expression and its interactants provides evidence for a role in modulation of mitochondrial function.
Structural and biochemical characterization of human mitochondrial branched-chain α-ketoacid dehydrogenase phosphatase.
Architecture of the human interactome defines protein communities and disease networks.
Dual proteome-scale networks reveal cell-specific remodeling of the human interactome.
Quantitative high-confidence human mitochondrial proteome and its dynamics in cellular context.
Purification and characterization of human liver branched-chain alpha-keto acid dehydrogenase complex.
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Purified human liver BCKDH oxidizes all three corresponding ketoacids; these kinetics are for the complete complex with E3 supplementation.
"The BCKADH effectively oxidized all of KIV, KIC, and KMV, yielding apparent Km values in the range of 14-17 microM for those alpha-keto acids."
Multimodal cell maps as a foundation for structural and functional genomics.
BCKDK phosphorylates BCKDH
PPM1K dephosphorylates p-BCKDH
LIPT1 transfers lipoyl group from lipoyl-GCSH to DBT/DLST
Branched-chain amino acid catabolism
LONP1 degrades mitochondrial matrix proteins
LONP1 binds mitochondrial matrix proteins
BCKDHA:BCKDHB tetramer decarboxylates KIC, KMVA, KIV
DBT transfers BCAA to CoA
DLD dimer dehydrogenates dihydrolipoyl
DBT loss-of-function mutants don't synthesize BCAA-CoA
BCKDHA or BCKDHB loss-of-function mutants don't synthesize BCAA-CoA
Loss-of-function DLD mutants don't dehydrogenate dihydrolipoyl DBT
BCKDK loss-of-function mutations do not phosphorylate BCKDH
H139Hfs13* PPM1K does not dephosphorylate BCKDH
Solution structure and dynamics of the lipoic acid-bearing domain of human mitochondrial branched-chain alpha-keto acid dehydrogenase complex.
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Complete abstract and ROOT selected author-original Methods/Results support the lipoyl-domain structure. Official 1K8M mapping reconstructs native residues 62–145 and lipoyl Lys105; the tagged 93-residue construct is not full-length E2.
"The lipoyl Lys(44) residue resides at the tip of a beta-hairpin"
Differential processing of human and rat E1 alpha precursors of the branched-chain alpha-keto acid dehydrogenase complex caused by an N-terminal proline in the rat sequence.
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Complete abstract explicitly reports human E2 microsequencing and an N-terminal glycine. Full original unavailable. Current UniProt reference positions 47–81 conflict with Tyr47; no alternative mature chain is invented.
"E1 beta and E2 subunits (Val and Gly,
respectively) are identical to those of the corresponding rat and bovine
subunits."
Small molecule branched-chain ketoacid dehydrogenase kinase (BDK) inhibitors with opposing effects on BDK protein levels.
UniProtKB P11182: human DBT
DBT source and product evidence guide
DBT selected original assay and table notes
UniProtKB entry P11182 (ODB2_HUMAN)
Falcon deep research report for DBT