BRENDA - Enzyme Database show
show all sequences of 6.4.1.3

Propionic acidemia: analysis of mutant propionyl-CoA carboxylase enzymes expressed in Escherichia coli

Chloupkova, M.; Maclean, K.N.; Alkhateeb, A.; Kraus, J.P.; Hum. Mutat. 19, 629-640 (2002)

Data extracted from this reference:

Cloned(Commentary)
Commentary
Organism
expressed in Escherichia coli
Homo sapiens
Engineering
Amino acid exchange
Commentary
Organism
A497V
significantly increased activity
Homo sapiens
A513_R514insP
insertion, severe degradation of the subunit
Homo sapiens
D178H
increased alpha/beta subunit ratio, very low activity
Homo sapiens
DELTA408
catalytically inactive
Homo sapiens
DELTA499
severe degradation of the subunit
Homo sapiens
DELTA514
severe degradation of the subunit
Homo sapiens
E168K
increased alpha/beta subunit ratio, very low activity
Homo sapiens
G131R
catalytically inactive
Homo sapiens
G198D
catalytically inactive
Homo sapiens
L519P
complete degradation of the subunit
Homo sapiens
M442T
catalytically inactive
Homo sapiens
N536D
complete degradation of the subunit
Homo sapiens
P228L
increased alpha/beta subunit ratio, very low activity
Homo sapiens
R165W
increased alpha/beta subunit ratio, very low activity
Homo sapiens
R410W
very low activity
Homo sapiens
R44P
catalytically inactive
Homo sapiens
R512C
severe degradation of the subunit
Homo sapiens
S106R
catalytically inactive
Homo sapiens
V205D
catalytically inactive; increased alpha/beta subunit ratio
Homo sapiens
Localization
Localization
Commentary
Organism
GeneOntology No.
Textmining
mitochondrion
-
Homo sapiens
5739
-
Metals/Ions
Metals/Ions
Commentary
Organism
Structure
Mg2+
required to form a complex with ATP
Homo sapiens
Molecular Weight [Da]
Molecular Weight [Da]
Molecular Weight Maximum [Da]
Commentary
Organism
800000
-
-
Homo sapiens
Natural Substrates/ Products (Substrates)
Natural Substrates
Organism
Commentary (Nat. Sub.)
Natural Products
Commentary (Nat. Pro.)
Organism (Nat. Pro.)
Reversibility
ATP + propanoyl-CoA + HCO3-
Homo sapiens
key enzyme in catabolic pathways for odd-chain fatty acids, cholesterol and branched chain amino acids
ADP + phosphate + (S)-methylmalonyl-CoA
-
Homo sapiens
?
Organism
Organism
Primary Accession No. (UniProt)
Commentary
Textmining
Homo sapiens
-
cDNA encoding for the alpha subunit
-
Homo sapiens
P05166
cDNA encoding for the beta subunit
-
Purification (Commentary)
Commentary
Organism
recombinant enzymes from Escherichia coli
Homo sapiens
Substrates and Products (Substrate)
Substrates
Commentary Substrates
Literature (Substrates)
Organism
Products
Commentary (Products)
Literature (Products)
Organism (Products)
Reversibility
ATP + propanoyl-CoA + HCO3-
-
651459
Homo sapiens
ADP + phosphate + (S)-methylmalonyl-CoA
-
651459
Homo sapiens
?
ATP + propanoyl-CoA + HCO3-
key enzyme in catabolic pathways for odd-chain fatty acids, cholesterol and branched chain amino acids
651459
Homo sapiens
ADP + phosphate + (S)-methylmalonyl-CoA
-
651459
Homo sapiens
?
Subunits
Subunits
Commentary
Organism
dodecamer
6 alpha and 6 beta subunits
Homo sapiens
Cofactor
Cofactor
Commentary
Organism
Structure
biotin
bound to enzyme
Homo sapiens
Cloned(Commentary) (protein specific)
Commentary
Organism
expressed in Escherichia coli
Homo sapiens
Cofactor (protein specific)
Cofactor
Commentary
Organism
Structure
biotin
bound to enzyme
Homo sapiens
Engineering (protein specific)
Amino acid exchange
Commentary
Organism
A497V
significantly increased activity
Homo sapiens
A513_R514insP
insertion, severe degradation of the subunit
Homo sapiens
D178H
increased alpha/beta subunit ratio, very low activity
Homo sapiens
DELTA408
catalytically inactive
Homo sapiens
DELTA499
severe degradation of the subunit
Homo sapiens
DELTA514
severe degradation of the subunit
Homo sapiens
E168K
increased alpha/beta subunit ratio, very low activity
Homo sapiens
G131R
catalytically inactive
Homo sapiens
G198D
catalytically inactive
Homo sapiens
L519P
complete degradation of the subunit
Homo sapiens
M442T
catalytically inactive
Homo sapiens
N536D
complete degradation of the subunit
Homo sapiens
P228L
increased alpha/beta subunit ratio, very low activity
Homo sapiens
R165W
increased alpha/beta subunit ratio, very low activity
Homo sapiens
R410W
very low activity
Homo sapiens
R44P
catalytically inactive
Homo sapiens
R512C
severe degradation of the subunit
Homo sapiens
S106R
catalytically inactive
Homo sapiens
V205D
catalytically inactive; increased alpha/beta subunit ratio
Homo sapiens
Localization (protein specific)
Localization
Commentary
Organism
GeneOntology No.
Textmining
mitochondrion
-
Homo sapiens
5739
-
Metals/Ions (protein specific)
Metals/Ions
Commentary
Organism
Structure
Mg2+
required to form a complex with ATP
Homo sapiens
Molecular Weight [Da] (protein specific)
Molecular Weight [Da]
Molecular Weight Maximum [Da]
Commentary
Organism
800000
-
-
Homo sapiens
Natural Substrates/ Products (Substrates) (protein specific)
Natural Substrates
Organism
Commentary (Nat. Sub.)
Natural Products
Commentary (Nat. Pro.)
Organism (Nat. Pro.)
Reversibility
ATP + propanoyl-CoA + HCO3-
Homo sapiens
key enzyme in catabolic pathways for odd-chain fatty acids, cholesterol and branched chain amino acids
ADP + phosphate + (S)-methylmalonyl-CoA
-
Homo sapiens
?
Purification (Commentary) (protein specific)
Commentary
Organism
recombinant enzymes from Escherichia coli
Homo sapiens
Substrates and Products (Substrate) (protein specific)
Substrates
Commentary Substrates
Literature (Substrates)
Organism
Products
Commentary (Products)
Literature (Products)
Organism (Products)
Reversibility
ATP + propanoyl-CoA + HCO3-
-
651459
Homo sapiens
ADP + phosphate + (S)-methylmalonyl-CoA
-
651459
Homo sapiens
?
ATP + propanoyl-CoA + HCO3-
key enzyme in catabolic pathways for odd-chain fatty acids, cholesterol and branched chain amino acids
651459
Homo sapiens
ADP + phosphate + (S)-methylmalonyl-CoA
-
651459
Homo sapiens
?
Subunits (protein specific)
Subunits
Commentary
Organism
dodecamer
6 alpha and 6 beta subunits
Homo sapiens
Other publictions for EC 6.4.1.3
No.
1st author
Pub Med
title
organims
journal
volume
pages
year
Activating Compound
Application
Cloned(Commentary)
Crystallization (Commentary)
Engineering
General Stability
Inhibitors
KM Value [mM]
Localization
Metals/Ions
Molecular Weight [Da]
Natural Substrates/ Products (Substrates)
Organic Solvent Stability
Organism
Oxidation Stability
Posttranslational Modification
Purification (Commentary)
Reaction
Renatured (Commentary)
Source Tissue
Specific Activity [micromol/min/mg]
Storage Stability
Substrates and Products (Substrate)
Subunits
Temperature Optimum [C]
Temperature Range [C]
Temperature Stability [C]
Turnover Number [1/s]
pH Optimum
pH Range
pH Stability
Cofactor
Ki Value [mM]
pI Value
IC50 Value
Activating Compound (protein specific)
Application (protein specific)
Cloned(Commentary) (protein specific)
Cofactor (protein specific)
Crystallization (Commentary) (protein specific)
Engineering (protein specific)
General Stability (protein specific)
IC50 Value (protein specific)
Inhibitors (protein specific)
Ki Value [mM] (protein specific)
KM Value [mM] (protein specific)
Localization (protein specific)
Metals/Ions (protein specific)
Molecular Weight [Da] (protein specific)
Natural Substrates/ Products (Substrates) (protein specific)
Organic Solvent Stability (protein specific)
Oxidation Stability (protein specific)
Posttranslational Modification (protein specific)
Purification (Commentary) (protein specific)
Renatured (Commentary) (protein specific)
Source Tissue (protein specific)
Specific Activity [micromol/min/mg] (protein specific)
Storage Stability (protein specific)
Substrates and Products (Substrate) (protein specific)
Subunits (protein specific)
Temperature Optimum [C] (protein specific)
Temperature Range [C] (protein specific)
Temperature Stability [C] (protein specific)
Turnover Number [1/s] (protein specific)
pH Optimum (protein specific)
pH Range (protein specific)
pH Stability (protein specific)
pI Value (protein specific)
Expression
General Information
General Information (protein specific)
Expression (protein specific)
KCat/KM [mM/s]
KCat/KM [mM/s] (protein specific)
745766
Collard
Import of TAT-conjugated prop ...
Homo sapiens
Mol. Cell. Biol.
38
e00491
2018
-
1
1
-
-
-
-
-
1
-
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1
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1
1
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1
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-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
745187
Vandova
Heterologous expression of di ...
Streptomyces coelicolor
J. Antibiot.
70
859-863
2017
-
1
-
-
-
-
-
-
-
-
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1
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745501
Chapman
Propionyl-CoA carboxylase pcc ...
Caenorhabditis elegans
J. Inherit. Metab. Dis.
41
157-168
2017
-
-
-
-
-
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2
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5
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1
1
-
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-
744741
Liu
Measuring propionyl-CoA carbo ...
Homo sapiens
Clin. Chim. Acta
453
13-20
2016
-
1
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-
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2
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1
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1
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1
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-
-
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-
729050
Hou
Propionyl-CoA carboxylase in H ...
Haloferax mediterranei
Appl. Environ. Microbiol.
81
794-804
2015
-
-
-
-
-
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-
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2
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1
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-
1
-
-
-
-
-
-
-
-
-
2
2
-
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-
726557
Peng
Characterization of acetyl-CoA ...
Leptospira interrogans
Acta Biochim. Biophys. Sin.
44
692-702
2012
-
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1
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5
-
-
6
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1
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5
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1
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2
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1
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6
1
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1
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5
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6
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1
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1
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-
-
2
-
1
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6
1
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6
6
728270
Lee
The identification of antigeni ...
Clonorchis sinensis
Mol. Biochem. Parasitol.
182
1-6
2012
-
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1
-
-
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-
-
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1
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5
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1
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1
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713913
Jung
A combined approach of classic ...
Streptomyces hygroscopicus, Streptomyces hygroscopicus ATCC 29253
Appl. Microbiol. Biotechnol.
91
1389-1397
2011
-
-
1
-
-
-
-
-
-
-
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-
-
4
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2
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2
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1
1
-
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715895
Demirev
Biochemical characteristizatio ...
Streptomyces toxytricini
J. Microbiol.
49
407-412
2011
-
-
1
-
-
-
-
-
-
-
-
-
-
2
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1
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2
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1
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1
1
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1
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2
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702794
Zhang
Investigating the role of nati ...
Streptomyces coelicolor
Biotechnol. Bioeng.
105
567-573
2010
-
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1
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1
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1
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1
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714212
Arabolaza
Crystal structures and mutatio ...
Streptomyces coelicolor
Biochemistry
49
7367-7376
2010
-
-
1
1
6
-
-
13
-
-
-
-
-
2
-
-
1
-
-
-
-
-
4
-
-
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-
1
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1
1
1
6
-
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13
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1
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4
-
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716328
Huang
Crystal structure of the alpha ...
Ruegeria pomeroyi
Nature
466
1001-1005
2010
-
-
1
1
5
-
-
-
1
-
1
-
-
3
-
-
1
-
-
-
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-
4
1
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2
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2
3
2
5
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1
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2
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2
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4
2
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1
1
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692572
Hofherr
Short-term rescue of neonatal ...
Mus musculus
Hum. Gene Ther.
20
169-180
2009
-
1
-
-
1
-
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1
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704957
Mo
Enhanced FK506 production in S ...
Streptomyces coelicolor, Streptomyces coelicolor A3(2)
J. Ind. Microbiol. Biotechnol.
36
1473-1482
2009
-
-
1
-
-
-
-
-
-
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-
-
-
44
-
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2
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2
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704970
Lee
Unusual presentation of propio ...
Homo sapiens
J. Inherit. Metab. Dis.
23
S97-101
2009
-
-
-
-
-
-
-
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2
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1
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1
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705936
Ballhausen
Evidence for catabolic pathway ...
Rattus norvegicus
Neuroscience
164
578-587
2009
-
-
-
-
-
-
-
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1
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3
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19
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1
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19
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1
1
1
1
-
-
706079
Sato
Liver transplantation in a pat ...
Homo sapiens
Pediatr. Transplant.
13
790-793
2009
-
1
-
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2
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1
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1
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-
690723
Lin
Neurofibromin interacts with C ...
Rattus norvegicus
Biochem. Biophys. Res. Commun.
369
747-752
2008
-
-
-
-
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1
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2
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1
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2
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671196
Stratton
Lymphocyte propionyl-CoA carbo ...
Homo sapiens
Am. J. Clin. Nutr.
84
384-388
2006
-
1
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-
-
-
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3
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2
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2
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679922
Mutka
Metabolic pathway engineering ...
Streptomyces coelicolor
FEMS Yeast Res.
6
40-47
2006
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-
1
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-
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1
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Crystal structure of the beta- ...
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651169
Hugler
Characterization of acetyl-CoA ...
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Lau
Bovine kidney 3-methylcrotonyl ...
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Hector
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Erfle
Acetyl-CoA and propionyl-CoA c ...
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1973
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Kaziro
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