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Information on EC 1.2.4.4 - 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) and Organism(s) Mus musculus

for references in articles please use BRENDA:EC1.2.4.4
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IUBMB Comments
Contains thiamine diphosphate. It acts not only on 3-methyl-2-oxobutanaoate, but also on 4-methyl-2-oxopentanoate and (S)-3-methyl-2-oxopentanoate, so that it acts on the 2-oxo acids that derive from the action of transaminases on valine, leucine and isoleucine. It is a component of the multienzyme 3-methyl-2-oxobutanoate dehydrogenase complex in which multiple copies of it are bound to a core of molecules of EC 2.3.1.168, dihydrolipoyllysine-residue (2-methylpropanoyl)transferase, which also binds multiple copies of EC 1.8.1.4, dihydrolipoyl dehydrogenase. It does not act on free lipoamide or lipoyllysine, but only on the lipoyllysine residue in EC 2.3.1.168.
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Mus musculus
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The taxonomic range for the selected organisms is: Mus musculus
The expected taxonomic range for this enzyme is: Bacteria, Eukaryota, Archaea
Synonyms
bckdh, bckdhb, bckdha, branched-chain 2-oxo acid dehydrogenase, branched-chain alpha-keto acid dehydrogenase, alpha-ketoacid dehydrogenase, branched-chain alpha-ketoacid dehydrogenase, bckdc, branched-chain alpha-ketoacid dehydrogenase complex, bcka dehydrogenase, more
SYNONYM
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
2-oxoisocaproate dehydrogenase
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2-oxoisovalerate dehydrogenase (lipoate)
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alpha-keto-beta-methylvalerate dehydrogenase
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alpha-ketoisocaproate dehydrogenase
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alpha-ketoisocaproic dehydrogenase
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alpha-ketoisocaproic-alpha-keto-beta-methylvaleric dehydrogenase
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alpha-ketoisovalerate dehydrogenase
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alpha-oxoisocaproate dehydrogenase
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BCDH
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BCKADC
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BCKDC
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BCKDH
BCKDH E1-alpha
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BCKDH E1-beta
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BCOAD
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branched chain alpha-ketoacid dehydrogenase complex
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branched chain keto acid dehydrogenase
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branched-chain (-2-oxoacid BCD) dehydrogenase
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branched-chain 2-keto acid dehydrogenase
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branched-chain 2-oxo acid dehydrogenase
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branched-chain alpha-keto acid dehydrogenase
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branched-chain alpha-keto acid dehydrogenase complex
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branched-chain alpha-ketoacid dehydrogenase
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branched-chain alpha-oxo acid dehydrogenase
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branched-chain keto acid dehydrogenase
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branched-chain keto acid dehydrogenase complex
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branched-chain ketoacid dehydrogenase
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dehydrogenase, 2-oxoisocaproate
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dehydrogenase, 2-oxoisovalerate (lipoate)
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dehydrogenase, branched chain alpha-keto acid
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REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
oxidation
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oxidative decarboxylation
redox reaction
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reduction
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SYSTEMATIC NAME
IUBMB Comments
3-methyl-2-oxobutanoate:[dihydrolipoyllysine-residue (2-methylpropanoyl)transferase]-lipoyllysine 2-oxidoreductase (decarboxylating, acceptor-2-methylpropanoylating)
Contains thiamine diphosphate. It acts not only on 3-methyl-2-oxobutanaoate, but also on 4-methyl-2-oxopentanoate and (S)-3-methyl-2-oxopentanoate, so that it acts on the 2-oxo acids that derive from the action of transaminases on valine, leucine and isoleucine. It is a component of the multienzyme 3-methyl-2-oxobutanoate dehydrogenase complex in which multiple copies of it are bound to a core of molecules of EC 2.3.1.168, dihydrolipoyllysine-residue (2-methylpropanoyl)transferase, which also binds multiple copies of EC 1.8.1.4, dihydrolipoyl dehydrogenase. It does not act on free lipoamide or lipoyllysine, but only on the lipoyllysine residue in EC 2.3.1.168.
CAS REGISTRY NUMBER
COMMENTARY hide
9031-05-4
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9082-72-8
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SUBSTRATE
PRODUCT                       
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
2-oxoisocaproate + NAD+ + CoA
3-methylbutanoyl-CoA + CO2 + NADH + H+
show the reaction diagram
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?
3-methyl-2-oxobutanoate + [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] lipoyllysine
[dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(2-methylpropanoyl)dihydrolipoyllysine + CO2
show the reaction diagram
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?
NATURAL SUBSTRATE
NATURAL PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
2-oxoisocaproate + NAD+ + CoA
3-methylbutanoyl-CoA + CO2 + NADH + H+
show the reaction diagram
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?
3-methyl-2-oxobutanoate + [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] lipoyllysine
[dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(2-methylpropanoyl)dihydrolipoyllysine + CO2
show the reaction diagram
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?
COFACTOR
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
INHIBITOR
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
(S)-2-chloro-phenylpropionic acid
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additional information
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amount of E2 subunit protein decreases in E2-siRNA transfected cells
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ACTIVATING COMPOUND
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
(S)-2-chlorophenylpropionic acid
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ORGANISM
COMMENTARY hide
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
SOURCE
LOCALIZATION
ORGANISM
UNIPROT
COMMENTARY hide
GeneOntology No.
LITERATURE
SOURCE
GENERAL INFORMATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
malfunction
UNIPROT
ENTRY NAME
ORGANISM
NO. OF AA
NO. OF TRANSM. HELICES
MOLECULAR WEIGHT[Da]
SOURCE
SEQUENCE
LOCALIZATION PREDICTION?
ODBB_MOUSE
390
0
42880
Swiss-Prot
Mitochondrion (Reliability: 1)
ODBA_MOUSE
442
0
50371
Swiss-Prot
Mitochondrion (Reliability: 1)
Q6LC11_MOUSE
17
0
1943
TrEMBL
other Location (Reliability: 3)
Q99L69_MOUSE
442
0
50359
TrEMBL
Mitochondrion (Reliability: 1)
Q3U3J1_MOUSE
446
0
50773
TrEMBL
Mitochondrion (Reliability: 1)
A0A0U1RPK5_MOUSE
99
0
11042
TrEMBL
other Location (Reliability: 2)
EXPRESSION
ORGANISM
UNIPROT
LITERATURE
donor hepatocytes expressing wild type BCKDH engrafted into intermediate maple syrup urine disease recipient liver, increase liver BCKDH activity. Hepatocyte transplantation increases liver BCKDH activity 14.36% of control activity
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APPLICATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
additional information
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activation of the translational regulators by leucine is partly regulated by the activity of BCKDH complex
REF.
AUTHORS
TITLE
JOURNAL
VOL.
PAGES
YEAR
ORGANISM (UNIPROT)
PUBMED ID
SOURCE
Nakai, N.; Shimomura, Y.; Tamura, T.; Tamura, N.; Hamada, K.; Kawano, F.; Ohira, Y.
Leucine-induced activation of translational initiation is partly regulated by the branched-chain alpha-keto acid dehydrogenase complex in C2C12 cells
Biochem. Biophys. Res. Commun.
343
1244-1250
2006
Mus musculus
Manually annotated by BRENDA team
Skvorak, K.J.
Animal models of maple syrup urine disease
J. Inherit. Metab. Dis.
32
229-246
2009
Mus musculus
Manually annotated by BRENDA team
Skvorak, K.J.; Paul, H.S.; Dorko, K.; Marongiu, F.; Ellis, E.; Chace, D.; Ferguson, C.; Gibson, K.M.; Homanics, G.E.; Strom, S.C.
Hepatocyte transplantation improves phenotype and extends survival in a murine model of intermediate maple syrup urine disease
Mol. Ther.
17
1266-1273
2009
Mus musculus
Manually annotated by BRENDA team
Tso, S.C.; Qi, X.; Gui, W.J.; Chuang, J.L.; Morlock, L.K.; Wallace, A.L.; Ahmed, K.; Laxman, S.; Campeau, P.M.; Lee, B.H.; Hutson, S.M.; Tu, B.P.; Williams, N.S.; Tambar, U.K.; Wynn, R.M.; Chuang, D.T.
Structure-based design and mechanisms of allosteric inhibitors for mitochondrial branched-chain alpha-ketoacid dehydrogenase kinase
Proc. Natl. Acad. Sci. USA
110
9728-9733
2013
Mus musculus
Manually annotated by BRENDA team