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Literature summary extracted from

  • Shafqat, N.; Kavanagh, K.L.; Sass, J.O.; Christensen, E.; Fukao, T.; Lee, W.H.; Oppermann, U.; Yue, W.W.
    A structural mapping of mutations causing succinyl-CoA:3-ketoacid CoA transferase (SCOT) deficiency (2013), J. Inherit. Metab. Dis., 36, 983-987.
    View publication on PubMedView publication on EuropePMC

Cloned(Commentary)

EC Number Cloned (Comment) Organism
2.8.3.5 expressed in Escherichia coli BL21(DE3) cells Homo sapiens

Crystallization (Commentary)

EC Number Crystallization (Comment) Organism
2.8.3.5 sitting drop vapor diffusion method, using 0.20 M sodium chloride, 0.1 M Tris pH 9.0 and 25% (w/v) polyethylene glycol 3350 Homo sapiens

Protein Variants

EC Number Protein Variants Comment Organism
2.8.3.5 A215V the mutation is associated with succinyl-CoA:3-ketoacid CoA transferase deficiency Homo sapiens
2.8.3.5 C456F the mutation is associated with succinyl-CoA:3-ketoacid CoA transferase deficiency Homo sapiens
2.8.3.5 E273X the mutation is associated with succinyl-CoA:3-ketoacid CoA transferase deficiency Homo sapiens
2.8.3.5 G219E the mutation is associated with succinyl-CoA:3-ketoacid CoA transferase deficiency Homo sapiens
2.8.3.5 G324E the mutation is associated with succinyl-CoA:3-ketoacid CoA transferase deficiency Homo sapiens
2.8.3.5 L327P the mutation is associated with succinyl-CoA:3-ketoacid CoA transferase deficiency Homo sapiens
2.8.3.5 L429F the mutation is associated with succinyl-CoA:3-ketoacid CoA transferase deficiency Homo sapiens
2.8.3.5 M388V the mutation is associated with succinyl-CoA:3-ketoacid CoA transferase deficiency Homo sapiens
2.8.3.5 P262R the mutation is associated with succinyl-CoA:3-ketoacid CoA transferase deficiency Homo sapiens
2.8.3.5 R217X the mutation is associated with succinyl-CoA:3-ketoacid CoA transferase deficiency Homo sapiens
2.8.3.5 R268C the mutation is associated with succinyl-CoA:3-ketoacid CoA transferase deficiency Homo sapiens
2.8.3.5 R268H the mutation is associated with succinyl-CoA:3-ketoacid CoA transferase deficiency Homo sapiens
2.8.3.5 R38C the mutation is associated with succinyl-CoA:3-ketoacid CoA transferase deficiency Homo sapiens
2.8.3.5 R468C the mutation is associated with succinyl-CoA:3-ketoacid CoA transferase deficiency Homo sapiens
2.8.3.5 S226N the mutation is associated with succinyl-CoA:3-ketoacid CoA transferase deficiency Homo sapiens
2.8.3.5 S283X the mutation is associated with succinyl-CoA:3-ketoacid CoA transferase deficiency Homo sapiens
2.8.3.5 S405P the mutation is associated with succinyl-CoA:3-ketoacid CoA transferase deficiency Homo sapiens
2.8.3.5 T435N the mutation is associated with succinyl-CoA:3-ketoacid CoA transferase deficiency Homo sapiens
2.8.3.5 V112D the mutation is associated with succinyl-CoA:3-ketoacid CoA transferase deficiency Homo sapiens
2.8.3.5 V133E the mutation is associated with succinyl-CoA:3-ketoacid CoA transferase deficiency Homo sapiens
2.8.3.5 V221M the mutation is associated with succinyl-CoA:3-ketoacid CoA transferase deficiency Homo sapiens
2.8.3.5 V404F the mutation is associated with succinyl-CoA:3-ketoacid CoA transferase deficiency Homo sapiens

Localization

EC Number Localization Comment Organism GeneOntology No. Textmining
2.8.3.5 mitochondrion
-
 Homo sapiens 5739
-

Organism

EC Number Organism UniProt Comment Textmining
2.8.3.5 Homo sapiens P55809
-
-

Purification (Commentary)

EC Number Purification (Comment) Organism
2.8.3.5 HisTrap column chromatography, and Superdex 200 gel filtration Homo sapiens

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
2.8.3.5 succinyl-CoA + acetoacetate
-
 Homo sapiens succinate + acetoacetyl-CoA
-
 ?

Subunits

EC Number Subunits Comment Organism
2.8.3.5 homodimer x-ray crystallography Homo sapiens

Synonyms

EC Number Synonyms Comment Organism
2.8.3.5 3-oxoacid CoA transferase 1
-
 Homo sapiens
2.8.3.5 OXCT1
-
 Homo sapiens
2.8.3.5 SCOT
-
 Homo sapiens
2.8.3.5 succinyl-CoA:3-ketoacid CoA transferase
-
 Homo sapiens