BRENDA - Enzyme Database show
show all sequences of 6.3.4.10

Positive newborn screen in the biochemically normal infant of a mother with treated holocarboxylase synthetase deficiency

Nyhan, W.L.; Willis, M.; Barshop, B.A.; Gangoiti, J.; J. Inherit. Metab. Dis. 32, S79-82 (2009)

Data extracted from this reference:

Natural Substrates/ Products (Substrates)
Natural Substrates
Organism
Commentary (Nat. Sub.)
Natural Products
Commentary (Nat. Pro.)
Organism (Nat. Pro.)
Reversibility
ATP + biotin + apo-[propionyl-CoA carboxylase]
Homo sapiens
-
AMP + diphosphate + propionyl-CoA carboxylase
-
-
?
Organism
Organism
Primary Accession No. (UniProt)
Commentary
Textmining
Homo sapiens
-
-
-
Substrates and Products (Substrate)
Substrates
Commentary Substrates
Literature (Substrates)
Organism
Products
Commentary (Products)
Literature (Products)
Organism (Products)
Reversibility
ATP + biotin + apo-[propionyl-CoA carboxylase]
-
704972
Homo sapiens
AMP + diphosphate + propionyl-CoA carboxylase
-
-
-
?
Natural Substrates/ Products (Substrates) (protein specific)
Natural Substrates
Organism
Commentary (Nat. Sub.)
Natural Products
Commentary (Nat. Pro.)
Organism (Nat. Pro.)
Reversibility
ATP + biotin + apo-[propionyl-CoA carboxylase]
Homo sapiens
-
AMP + diphosphate + propionyl-CoA carboxylase
-
-
?
Substrates and Products (Substrate) (protein specific)
Substrates
Commentary Substrates
Literature (Substrates)
Organism
Products
Commentary (Products)
Literature (Products)
Organism (Products)
Reversibility
ATP + biotin + apo-[propionyl-CoA carboxylase]
-
704972
Homo sapiens
AMP + diphosphate + propionyl-CoA carboxylase
-
-
-
?
General Information
General Information
Commentary
Organism
malfunction
holocarboxylase synthetase deficiency causes multiple carboxylase deficiency, phenotypes, overview
Homo sapiens
metabolism
the enzyme is part of the biotin metabolism in which it catalyses the attachment of biotin to apoenzyme carboxylases
Homo sapiens
General Information (protein specific)
General Information
Commentary
Organism
malfunction
holocarboxylase synthetase deficiency causes multiple carboxylase deficiency, phenotypes, overview
Homo sapiens
metabolism
the enzyme is part of the biotin metabolism in which it catalyses the attachment of biotin to apoenzyme carboxylases
Homo sapiens
Other publictions for EC 6.3.4.10
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)
745784
Donti
Holocarboxylase synthetase de ...
Homo sapiens
Mol. Genet. Metab. Rep.
7
40-44
2016
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1
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744011
Miyao
Immobilization of immunoglobu ...
Sulfurisphaera tokodaii, Sulfurisphaera tokodaii DSM 16993
Anal. Biochem.
484
113-121
2015
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726655
Xue
Holocarboxylase synthetase cat ...
Homo sapiens
Am. J. Physiol. Cell Physiol.
305
C1240-C1245
2013
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1
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728180
Li
Holocarboxylase synthetase int ...
Homo sapiens
J. Nutr. Biochem.
24
1446-1452
2013
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1
1
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713742
Singh
Identification of holocarboxyl ...
Homo sapiens
Anal. Biochem.
413
55-59
2011
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1
1
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1
1
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714763
Rios-Avila
A 96-well plate assay for high ...
Homo sapiens
Clin. Chim. Acta
412
735-739
2011
-
1
1
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1
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4
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715997
Bao
Holocarboxylase synthetase is ...
Homo sapiens
J. Nutr. Biochem.
22
470-475
2011
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1
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1
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1
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716237
Reyes-Carmona
Trafficking and chromatin dyna ...
Drosophila melanogaster
Mol. Genet. Metab.
103
240-248
2011
-
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1
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1
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726859
Bao
Human holocarboxylase syntheta ...
Homo sapiens
Biochem. Biophys. Res. Commun.
412
115-120
2011
-
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1
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1
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701935
Hassan
The polypeptide Syn67 interact ...
Homo sapiens
Arch. Biochem. Biophys.
495
35-41
2010
-
-
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701936
Bailey
Holocarboxylase synthetase: co ...
Homo sapiens
Arch. Biochem. Biophys.
496
45-52
2010
-
-
-
-
-
-
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5
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2
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1
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2
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2
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703738
Lee
The N-terminal domain of human ...
Homo sapiens
FEBS Lett.
584
675-680
2010
-
-
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1
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1
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3
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2
1
1
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-
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2
1
1
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1
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1
1
-
-
-
705293
Wijeratne
K12-biotinylated histone H4 is ...
Homo sapiens
J. Nutr. Biochem.
21
310-316
2010
-
-
-
-
-
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2
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2
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2
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2
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-
1
1
-
-
-
715999
Bao
Biotin regulates the expressio ...
Homo sapiens
J. Nutr.
140
1546-1551
2010
-
-
1
-
-
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-
-
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3
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4
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4
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1
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1
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-
702499
Zempleni
Biotin ...
Homo sapiens
Biofactors
35
36-46
2009
-
-
-
-
-
-
-
-
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1
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2
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2
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3
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2
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3
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2
2
-
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702952
Yokoi
A case of holocarboxylase synt ...
Homo sapiens
Brain Dev.
31
775-778
2009
-
-
-
-
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-
-
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1
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1
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1
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1
1
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703282
Tammachote
Holocarboxylase synthetase def ...
Homo sapiens
Clin. Genet.
78
88-93
2009
-
-
1
-
1
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1
1
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704561
Ingaramo
Distinct amino termini of two ...
Homo sapiens
J. Biol. Chem.
284
30862-30870
2009
-
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1
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1
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1
1
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3
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1
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1
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704972
Nyhan
Positive newborn screen in the ...
Homo sapiens
J. Inherit. Metab. Dis.
32
S79-82
2009
-
-
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2
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2
2
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705752
Hassan
N- and C-terminal domains in h ...
Homo sapiens
Mol. Genet. Metab.
96
183-188
2009
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1
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706629
Healy
Nonenzymatic biotinylation of ...
Homo sapiens
Protein Sci.
18
314-328
2009
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2
1
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662308
Pacheco-Alvarez
Paradoxical regulation of biot ...
Rattus norvegicus
J. Biol. Chem.
279
52312-52318
2004
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1390
Suzuki
Purification and properties of ...
Bos taurus, Homo sapiens, Oryctolagus cuniculus, Rattus norvegicus
Methods Enzymol.
279
386-393
1997
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2
1
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1
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3
1
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4
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4
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2
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3
2
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15
2
1
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1
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2
1
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1
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3
1
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4
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2
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3
2
-
15
2
1
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1
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-
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1396
Tissot
Evidence for multiple forms of ...
Arabidopsis thaliana, Pisum sativum
Biochem. J.
323
179-188
1997
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1
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4
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9
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1392
Dupuis
Clustering of mutations in the ...
Homo sapiens
Hum. Mol. Genet.
5
1011-1016
1996
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1
1
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6
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1
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3
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1
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6
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1
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6
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