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Literature summary for 1.2.4.4 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

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

Natural Substrates/ Products (Substrates)

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

Organism

Organism UniProt Comment Textmining
Mus musculus
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
liver
-
Mus musculus
-

Substrates and Products (Substrate)

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

Synonyms

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

Cofactor

Cofactor Comment Organism Structure
CoA
-
Mus musculus
NAD+
-
Mus musculus

Expression

Organism Comment Expression
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

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