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Literature summary extracted from

  • Bunik, V.I.; Raddatz, G.; Wanders, R.J.; Reiser, G.
    Brain pyruvate and 2-oxoglutarate dehydrogenase complexes are mitochondrial targets of the CoA ester of the Refsum disease marker phytanic acid (2006), FEBS Lett., 580, 3551-3557.
    View publication on PubMed

Application

EC Number Application Comment Organism
1.2.1.105 medicine key regulatory branch points of mitochondrial metabolism are targets of a cellular derivative of phytanic acid, decreased activity of the complexes may contribute to neurological symptoms upon accumulation of phytanic acid in Refsum disease Rattus norvegicus

Inhibitors

EC Number Inhibitors Comment Organism Structure
1.2.1.105 palmitoyl-CoA is 10-fold less potent than phytanoyl-CoA, no inhibitory effect up to 0.3 mM Rattus norvegicus
1.2.1.105 phytanoyl-CoA is 10-fold more potent than palmitoyl-CoA, no inhibitory effect up to 0.3 mM Rattus norvegicus

Localization

EC Number Localization Comment Organism GeneOntology No. Textmining
1.2.1.105 mitochondrion
-
 Rattus norvegicus 5739
-

Organism

EC Number Organism UniProt Comment Textmining
1.2.1.105 Rattus norvegicus
-
-
-

Purification (Commentary)

EC Number Purification (Comment) Organism
1.2.1.105 by gel filtration Rattus norvegicus

Source Tissue

EC Number Source Tissue Comment Organism Textmining
1.2.1.105 brain
-
 Rattus norvegicus
-

Specific Activity [micromol/min/mg]

EC Number Specific Activity Minimum [µmol/min/mg] Specific Activity Maximum [µmol/min/mg] Comment Organism
1.2.1.105 0.7
-
-
 Rattus norvegicus

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
1.2.1.105 2-oxoglutarate + [dihydrolipoyllysine-residue succinyltransferase] lipoyllysine
-
 Rattus norvegicus [dihydrolipoyllysine-residue succinyltransferase] S-succinyldihydrolipoyllysine + CO2
-
 ?

Synonyms

EC Number Synonyms Comment Organism
1.2.1.105 2-oxoglutarate dehydrogenase
-
 Rattus norvegicus
1.2.1.105 2-oxoglutarate dehydrogenase complex
-
 Rattus norvegicus
1.2.1.105 E1o
-
 Rattus norvegicus