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Literature summary extracted from

  • Chuang, D.T.; Fisher, C.W.; Lau, K.S.; Griffin, T.A.; Wynn, R.M.; Cox, R.P.
    Maple syrup urine disease: domain structure, mutations and exon skipping in the dihydrolipoyl transacylase (E2) component of the branched-chain alpha-keto acid dehydrogenase complex (1991), Mol. Biol. Med., 8, 49-63.
    View publication on PubMed

Cloned(Commentary)

EC Number Cloned (Comment) Organism
2.3.1.168 DNA sequence determination, overexpression of wild-type, lipol-free apo-enzyme, and mutants in Escherichia coli Bos taurus
2.3.1.168 gene E2, DNA sequence determination and analysis Homo sapiens
2.3.1.168 gene E2, mapping to chromosome 1p31 Homo sapiens

Protein Variants

EC Number Protein Variants Comment Organism
2.3.1.168 F214C natural splicing mutant from WG-34 cells, related to the maple syrup urine disease, phenotype of homo- and heterozygous mutations in humans Homo sapiens
2.3.1.168 H391N enzymatically inactive Bos taurus
2.3.1.168 H391Q enzymatically inactive Bos taurus

Molecular Weight [Da]

EC Number Molecular Weight [Da] Molecular Weight Maximum [Da] Comment Organism
2.3.1.168 52000
-
 24 * 52000, SDS-PAGE Bos taurus
2.3.1.168 1116000
-
 amino acid determination and sedimentation equilibrium analysis Bos taurus

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
2.3.1.168 2-methylpropanoyl-CoA + enzyme N6-(dihydrolipoyl)lysine Homo sapiens
-
 CoA + enzyme N6-(S-[2-methylpropanoyl]dihydrolipoyl)lysine
-
 ?
2.3.1.168 2-methylpropanoyl-CoA + enzyme N6-(dihydrolipoyl)lysine Bos taurus multienzyme complex, scheme of the reaction steps CoA + enzyme N6-(S-[2-methylpropanoyl]dihydrolipoyl)lysine
-
 ?

Organism

EC Number Organism UniProt Comment Textmining
2.3.1.168 Bos taurus
-
-
-
2.3.1.168 Homo sapiens P11182
-
-

Purification (Commentary)

EC Number Purification (Comment) Organism
2.3.1.168 recombinant wild-type, apo-E2 enzyme, and mutants from Escherichia coli Bos taurus

Reaction

EC Number Reaction Comment Organism Reaction ID
2.3.1.168 2-methylpropanoyl-CoA + enzyme N6-(dihydrolipoyl)lysine = CoA + enzyme N6-(S-[2-methylpropanoyl]dihydrolipoyl)lysine His391 is involved in the catalytic reaction Bos taurus
a

Source Tissue

EC Number Source Tissue Comment Organism Textmining
2.3.1.168 liver
-
 Bos taurus
-
2.3.1.168 liver
-
 Homo sapiens
-

Specific Activity [micromol/min/mg]

EC Number Specific Activity Minimum [µmol/min/mg] Specific Activity Maximum [µmol/min/mg] Comment Organism
2.3.1.168 additional information
-
 recombinant enzyme Bos taurus

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
2.3.1.168 2-methylpropanoyl-CoA + enzyme N6-(dihydrolipoyl)lysine
-
 Bos taurus CoA + enzyme N6-(S-[2-methylpropanoyl]dihydrolipoyl)lysine
-
 ?
2.3.1.168 2-methylpropanoyl-CoA + enzyme N6-(dihydrolipoyl)lysine
-
 Homo sapiens CoA + enzyme N6-(S-[2-methylpropanoyl]dihydrolipoyl)lysine
-
 ?
2.3.1.168 2-methylpropanoyl-CoA + enzyme N6-(dihydrolipoyl)lysine multienzyme complex, scheme of the reaction steps Bos taurus CoA + enzyme N6-(S-[2-methylpropanoyl]dihydrolipoyl)lysine
-
 ?
2.3.1.168 isovaleryl-CoA + dihydrolipoamide recombinant enzyme, i.e. E2 domain, overexpressed in Escherichia coli Bos taurus CoA + S-isovaleryldihydrolipoamide
-
 r

Subunits

EC Number Subunits Comment Organism
2.3.1.168 24-mer 24 * 52000, SDS-PAGE Bos taurus
2.3.1.168 More enzyme shows the E2 structure of 3 folded domains: lipoyl-bearing, E3-binding, and inner core, typical for all E2 protein of alpha-keto acid dehydrogenases Bos taurus
2.3.1.168 More assembly of 24 E2 subunits into a cubic structure, forming the core of the mammalian branched-chain alpha-keto acid dehydrogenase multienzyme complex Bos taurus
2.3.1.168 More assembly of 24 E2 subunits into a cubic structure, forming the core of the mammalian branched-chain alpha-keto acid dehydrogenase multienzyme complex Homo sapiens

Synonyms

EC Number Synonyms Comment Organism
2.3.1.168 More enzyme is the E2 component of the multienzyme complex branched-chain alpha-keto acid dehydrogenase, i.e. branched-chain 2-oxo acid dehydrogenase Bos taurus
2.3.1.168 More enzyme is the E2 component of the multienzyme complex branched-chain alpha-keto acid dehydrogenase, i.e. branched-chain 2-oxo acid dehydrogenase Homo sapiens