6.3.4.10: biotin-[propionyl-CoA-carboxylase (ATP-hydrolysing)] ligase
This is an abbreviated version!
For detailed information about biotin-[propionyl-CoA-carboxylase (ATP-hydrolysing)] ligase, go to the full flat file.
Word Map on EC 6.3.4.10
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6.3.4.10
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biotinylation
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carboxylases
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biotin-dependent
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histone
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biotinidase
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acidosis
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biotin-responsive
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multivitamin
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aciduria
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biotinyl-5\'-amp
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biotin-deficient
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apocarboxylases
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methylcitrate
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3-hydroxyisovaleric
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hyperammonemia
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tokodaii
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3-methylcrotonyl-coa
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biotin-binding
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diagnostics
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medicine
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analysis
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synthesis
- 6.3.4.10
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biotinylation
- carboxylases
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biotin-dependent
- histone
- biotinidase
- acidosis
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biotin-responsive
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multivitamin
- aciduria
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biotinyl-5\'-amp
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biotin-deficient
- apocarboxylases
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methylcitrate
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3-hydroxyisovaleric
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hyperammonemia
- tokodaii
- 3-methylcrotonyl-coa
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biotin-binding
- diagnostics
- medicine
- analysis
- synthesis
Reaction
Synonyms
biotin protein ligase, Biotin-propionyl coenzyme A carboxylase synthetase, Biotin-[propionyl-CoA-carboxylase (ATP-hydrolysing)] synthetase, BPL, HCS, HLCS, Holocarboxylase synthetase, Propionyl coenzyme A holocarboxylase synthetase, Synthetase, biotin-propionyl coenzyme A carboxylase
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Natural Substrates Products
Natural Substrates Products on EC 6.3.4.10 - biotin-[propionyl-CoA-carboxylase (ATP-hydrolysing)] ligase
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REACTION DIAGRAM
ATP + biotin + apo-[propionyl CoA carboxylase]
AMP + diphosphate + propionyl CoA carboxylase
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ATP + biotin + apo-[propionyl-CoA carboxylase]
AMP + diphosphate + propionyl-CoA carboxylase
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ATP + biotin + apo-[propionyl-CoA:carbon-dioxide ligase (ADP-forming)]
AMP + diphosphate + [propionyl-CoA:carbon-dioxide ligase (ADP-forming)]
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ATP + biotin + histone H4
AMP + diphosphate + biotin-histone H4
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biotinylation at Lys12 especially in telomeric repeats, overview
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plays an essential role in biotin utilization
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ATP + biotin + apo-[propanoyl-CoA:carbon-dioxide ligase (ADP-forming)]
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its deficiency causes biotin-responsive multiple carboxylase deficiency in humans
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ATP + biotin + apo-[propanoyl-CoA:carbon-dioxide ligase (ADP-forming)]
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although mammals have multiple biotin-dependent enzymes there is only a single gene encoding holocarboxylase synthetase, HCS, responsible for all cellular biotinylation. The enzyme catalyzes the reactions of EC 6.3.4.10, EC 6.3.4.11, and EC 6.3.4.15, overview
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additional information
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holocarboxylase synthetase catalyzes the attachment of biotin to the epsilon-amino group of a specific lysine residue in apocarboxylases, biotinylation of biotin-dependent carboxylases requires ATP and proceeds in the following two steps
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additional information
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holocarboxylase synthetase, HCS, catalyzes the binding of biotin to lysines in carboxylases and histones in two steps. First, HCS catalyzes the synthesis of biotinyl-5'-AMP, and second, the biotinyl moiety is ligated to lysine residues, binding structure, modelling, overview
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additional information
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the enzyme catalyzes the reactions of EC 6.3.4.10, EC 6.3.4.11, and EC 6.3.4.15, overview
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additional information
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the enzyme is critical to utilization of exogenous or dietary biotin and responds to biotin deprivation by reduction in the steady state level of their mRNAs. The free biotin is directed to brain because the reduction in holocarboxylase synthetase, sodium-dependent multivitamin-transporter and carboxylase mRNAs in liver reduces their capacity to utilize biotin while leaving free biotin in circulation
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