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Literature summary for 2.8.3.5 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)

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

Crystallization (Commentary)

Crystallization (Comment) Organism
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

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

Localization

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

Organism

Organism UniProt Comment Textmining
Homo sapiens P55809
-
-

Purification (Commentary)

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

Substrates and Products (Substrate)

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

Subunits

Subunits Comment Organism
homodimer x-ray crystallography Homo sapiens

Synonyms

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