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Literature summary for 1.2.1.105 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
Search for more literature for 1.2.1.105

Application

Application Comment Organism
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

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

Localization

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

Organism

Organism UniProt Comment Textmining
Rattus norvegicus
-
-
-

Purification (Commentary)

Purification (Comment) Organism
by gel filtration Rattus norvegicus

Source Tissue

Source Tissue Comment Organism Textmining
brain
-
 Rattus norvegicus
-

Specific Activity [micromol/min/mg]

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

Substrates and Products (Substrate)

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

Synonyms

Synonyms Comment Organism
2-oxoglutarate dehydrogenase
-
 Rattus norvegicus
2-oxoglutarate dehydrogenase complex
-
 Rattus norvegicus
E1o
-
 Rattus norvegicus