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1.3.8.1: short-chain acyl-CoA dehydrogenase

This is an abbreviated version!
For detailed information about short-chain acyl-CoA dehydrogenase, go to the full flat file.

Word Map on EC 1.3.8.1

Reaction

a short-chain acyl-CoA
+
electron-transfer flavoprotein
=
a short-chain trans-2,3-dehydroacyl-CoA
 +
reduced electron-transfer flavoprotein

Synonyms

3-hydroxyacyl CoA reductase, ACADS, Acfer_0556, Acfer_1477, acyl-CoA dehydrogenase short chain, BCAD, BCD, bcd2, BcdAf, bSCAD, butyryl CoA dehydrogenase, butyryl coenzyme A dehydrogenase, butyryl-CoA dehydrogenase, butyryl-CoA dehydrogenase complex, butyryl-CoA dehydrogenase/Etf complex, CD1054, dehydrogenase, butyryl coenzyme A, EC 1.3.2.1, EC 1.3.99.2, enoyl-coenzyme A reductase, ethylene reductase, HADHSC, non-dissociating bifurcating Etf-Bcd complex, SCAD, ScdA, short chain acyl-CoA dehydrogenase, short-chain 3-hydroxyacyl-CoA dehydrogenase, short-chain acyl CoA dehydrogenase, short-chain acyl-CoA dehydrogenase, short-chain acyl-coenzyme A dehydrogenase, short-chain acylCoA dehydrogenase, unsaturated acyl coenzyme A reductase

ECTree

     1 Oxidoreductases
         1.3 Acting on the CH-CH group of donors
             1.3.8 With a flavin as acceptor
                1.3.8.1 short-chain acyl-CoA dehydrogenase

Engineering

Engineering on EC 1.3.8.1 - short-chain acyl-CoA dehydrogenase

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PROTEIN VARIANTS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
C1147T/G625A
-
naturally occuring mutation, genotype, mutant shows increased acylglycines and organic acid levels in the urine
C319G/G625A
-
naturally occuring mutation, genotype, mutant shows increased acylglycines and organic acid levels in the urine and upper reaspiratory infections
C319T
-
naturally occuring mutation, genotype, mutant shows increased acylglycines and organic acid levels in the urine
C319T/G1095T
-
naturally occuring mutation, genotype, mutant shows increased acylglycines and organic acid levels in the urine
C488A/C988T
-
naturally occuring mutation, genotype, mutant shows increased acylglycines and organic acid levels in the urine and reactive airway disease
C527A/T1164/G1165del/G625A
-
naturally occuring mutation, genotype, mutant shows increased acylglycines and organic acid levels in the urine
C867A
-
naturally occuring homozygote mutation, genotype, mutant shows increased isobutyrylcarnitine levels in the urine
E344G
site-directed mutagenesis, the SCAD mutant shows reduced activity compared to the wild-typ enzyme, but does not influence the wild-type SCAD activity when co-transfected in HEK-293 cells
E368G
-
is unable to form a charge-transfer complex with substrate/product, does not efficiently compete with the wild-type enzyme for the physiological electron acceptor
E368Q
-
inactivates the reductive and oxidative pathways
G108D
site-directed mutagenesis, the SCAD mutant shows reduced activity compared to the wild-typ enzyme, but does not influence the wild-type SCAD activity when co-transfected in HEK-293 cells
G1095T/G625A
-
naturally occuring mutation, genotype, mutant shows increased acylglycines and organic acid levels in the urine
G1153T/G625A
-
naturally occuring mutation, genotype, mutant shows increased acylglycines and organic acid levels in the urine
G185S
G209S
-
mutant shows a temperature-dependent production of SCAD tetramers with reduced amounts compared to the wild type enzyme
G268A/1C147T/G625A
-
naturally occuring mutation, genotype, mutant shows increased acylglycines and organic acid levels in the urine
G268A/C1147T/G625A
-
naturally occuring mutation, genotype, mutant shows increased acylglycines and organic acid levels in the urine
G320A/G417C
-
naturally occuring mutation, genotype, mutant shows highly increased acylglycines and organic acid levels in the urine and eczema
G625A
-
naturally occuring mutation, genotype, mutant shows increased acylglycines and organic acid levels in the urine
G682/A683del/C988T
-
naturally occuring mutation, genotype, mutant shows increased acylglycines and organic acid levels in the urine
P55L
site-directed mutagenesis, the SCAD mutant shows reduced activity compared to the wild-typ enzyme, but does not influence the wild-type SCAD activity when co-transfected in HEK-293 cells
R107C
-
naturally occuring mutation, development of a cell model system, stably expressing either the SCAD wild-type protein or the misfolding SCAD variant protein, R107C, genotype C319T. The model system is used for investigation of SCAD with respect to expression, degree of misfolding, and enzymatic SCAD activity
R147W
R171W
-
mutant shows a temperature-dependent production of SCAD tetramers with reduced amounts compared to the wild type enzyme
T455C/T443T
-
naturally occuring mutation, genotype, mutant shows increased isobutyrylcarnitine levels in the urine, and pyelonephritisand emesis
T529C
-
naturally occuring mutation, genotype, mutant shows increased acylglycines and organic acid levels in the urine
T529C/G625A
-
naturally occuring mutation, genotype, mutant shows increased acylglycines and organic acid levels in the urine and atrial septal defect
E367Q
-
mutant enzyme shows less than 0.03% of the activity seen in normal cloned enzyme, E367 is responsible for catalytic activity
E368D
-
mutant exhibits enzyme activity
E368G
-
inactive enzyme, E368 is responsible for catalytic activity
E368G/G247E
-
mutant exhibits enzyme activity
E368L
-
inactive enzyme, E368 is responsible for catalytic activity
E368Q
-
inactive enzyme, E368 is responsible for catalytic activity
E368R
-
inactive enzyme, E368 is responsible for catalytic activity
additional information