DBT transfers BCAA to CoA

Stable Identifier
R-HSA-9859163
Type
Reaction [transition]
Species
Homo sapiens
Compartment
mitochondrial matrix
ReviewStatus
5/5
Locations in the PathwayBrowser
Expand all
General
SVG | 
PNG
Low
Medium
High
 | PPTX  | SBGN
DBT transfers BCAA to CoA
Click the image above or here to open this reaction in the Pathway Browser
The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout
Lipoamide acyltransferase E2 component (DBT) of branched-chain alpha-keto acid dehydrogenase complex (BCKDH) transfers the isovaleryl, alpha-methylbutyryl, or isobutyryl groups bound to one of its N6-dihydrolipoyllysine residues onto the substrate CoA (Billington et al., 2022). Due to its homology with other E2 subunits like DLST and DLAT, DBT is likely to form the BCKDH complex core of eight E2 trimers.
Literature References
PubMed ID Title Journal Year
35799415 Genomic and biochemical analysis of repeatedly observed variants in DBT in individuals with maple syrup urine disease of Central American ancestry

Billington, CJ, Chapman, KA, Leon, E, Meltzer, BW, Berger, SI, Olson, M, Figler, RA, Hoang, SA, Wanxing, C, Wamhoff, BR, Collado, MS, Cusmano-Ozog, K

Am J Med Genet A 2022
Participants
Input
Output
Participates
as an event of
Catalyst Activity

acyltransferase activity, transferring groups other than amino-acyl groups of BCKDH [mitochondrial matrix]

Physical Entity
BCKDH [mitochondrial matrix] (Homo sapiens)
Activity
acyltransferase activity, transferring groups other than amino-acyl groups (GO:0016747)
Orthologous Events
DBT transfers BCAA to CoA (Bos taurus)
DBT transfers BCAA to CoA (Caenorhabditis elegans)
DBT transfers BCAA to CoA (Canis familiaris)
DBT transfers BCAA to CoA (Danio rerio)
DBT transfers BCAA to CoA (Dictyostelium discoideum)
DBT transfers BCAA to CoA (Drosophila melanogaster)
DBT transfers BCAA to CoA (Gallus gallus)
DBT transfers BCAA to CoA (Mus musculus)
DBT transfers BCAA to CoA (Plasmodium falciparum)
DBT transfers BCAA to CoA (Rattus norvegicus)
DBT transfers BCAA to CoA (Sus scrofa)
DBT transfers BCAA to CoA (Xenopus tropicalis)
Cross References
RHEA
Authored
Stephan, R  (2024-01-16)
Reviewed
D'Eustachio, P  (2024-04-17)
Created
Stephan, R  (2024-01-16)
Cite Us!