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Literature summary extracted from

  • 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 (2009), Mol. Ther., 17, 1266-1273.
    View publication on PubMedView publication on EuropePMC

Localization

EC Number Localization Comment Organism GeneOntology No. Textmining
1.2.4.4 mitochondrion
-
Mus musculus 5739
-

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
1.2.4.4 3-methyl-2-oxobutanoate + [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] lipoyllysine Mus musculus
-
[dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(2-methylpropanoyl)dihydrolipoyllysine + CO2
-
?

Organism

EC Number Organism UniProt Comment Textmining
1.2.4.4 Mus musculus
-
-
-

Source Tissue

EC Number Source Tissue Comment Organism Textmining
1.2.4.4 liver
-
Mus musculus
-

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
1.2.4.4 3-methyl-2-oxobutanoate + [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] lipoyllysine
-
Mus musculus [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(2-methylpropanoyl)dihydrolipoyllysine + CO2
-
?

Synonyms

EC Number Synonyms Comment Organism
1.2.4.4 BCKDH
-
Mus musculus
1.2.4.4 branched chain alpha-ketoacid dehydrogenase complex
-
Mus musculus

Cofactor

EC Number Cofactor Comment Organism Structure
1.2.4.4 CoA
-
Mus musculus
1.2.4.4 NAD+
-
Mus musculus

Expression

EC Number Organism Comment Expression
1.2.4.4 Mus musculus 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 up

General Information

EC Number General Information Comment Organism
1.2.4.4 malfunction maple syrup urine disease is an inborn error of metabolism of the branched chain alpha-ketoacid dehydrogenase complex Mus musculus