EC Number | Application | Comment | Organism |
---|---|---|---|
6.3.4.10 | medicine | HCS deficiency results in biotin-responsive multiple carboxylase deficiency | Homo sapiens |
EC Number | Cloned (Comment) | Organism |
---|---|---|
6.3.4.10 | - |
Homo sapiens |
EC Number | Protein Variants | Comment | Organism |
---|---|---|---|
6.3.4.10 | D571N | mutation within the biotin-binding region, HCS deficiency, biotin-responsive multiple carboxylase deficiency | Homo sapiens |
6.3.4.10 | G518E | mutation within the biotin-binding region, HCS deficiency, biotin-responsive multiple carboxylase deficiency | Homo sapiens |
6.3.4.10 | L216R | mutation outside the biotin-binding region, HCS deficiency, biotin-responsive multiple carboxylase deficiency | Homo sapiens |
6.3.4.10 | R508W | mutation within the biotin-binding region, HCS deficiency, biotin-responsive multiple carboxylase deficiency | Homo sapiens |
6.3.4.10 | V363D | mutation outside the biotin-binding region, HCS deficiency, biotin-responsive multiple carboxylase deficiency | Homo sapiens |
6.3.4.10 | V550M | mutation within the biotin-binding region, HCS deficiency, biotin-responsive multiple carboxylase deficiency | Homo sapiens |
EC Number | KM Value [mM] | KM Value Maximum [mM] | Substrate | Comment | Organism | Structure |
---|---|---|---|---|---|---|
6.3.4.10 | additional information | - |
additional information | HCS from patients with neonatal biotin-responsive multiple carboxylase deficiency has a higher Km for biotin than normal HCS | Homo sapiens |
EC Number | Organism | UniProt | Comment | Textmining |
---|---|---|---|---|
6.3.4.10 | Homo sapiens | - |
- |
- |
6.3.4.10 | Homo sapiens | - |
holocarboxylase synthetase catalyzes the biotinylation of the four biotin-dependent carboxylases found in human, mitochondrial propionyl-CoA carboxylase, pyruvate carboxylase, beta-methylcrotonyl-CoA carboxylase, and cytosolic acetyl-CoA carboxylase | - |
6.3.4.15 | Homo sapiens | - |
holocarboxylase synthetase catalyzes the biotinylation of the four biotin-dependent carboxylases found in human, mitochondrial propionyl-CoA carboxylase, pyruvate carboxylase, beta-methylcrotonyl-CoA carboxylase, and cytosolic acetyl-CoA carboxylase | - |
EC Number | Source Tissue | Comment | Organism | Textmining |
---|---|---|---|---|
6.3.4.10 | fibroblast | - |
Homo sapiens | - |
EC Number | Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|---|
6.3.4.10 | ATP + biotin + apo-beta-methylcrotonyl-CoA carboxylase | - |
Homo sapiens | AMP + diphosphate + holo-beta-methylcrotonyl-CoA carboxylase | - |
? | |
6.3.4.10 | ATP + biotin + apo-[propanoyl-CoA:carbon-dioxide ligase (ADP-forming)] | - |
Homo sapiens | AMP + diphosphate + [propanoyl-CoA:carbon-dioxide ligase (ADP-forming)] | - |
? | |
6.3.4.10 | ATP + biotin + apoacetyl-CoA carboxylase | biotinylation of cytosolic ACC | Homo sapiens | AMP + diphosphate + holoacetyl-CoA carboxylase | - |
? | |
6.3.4.10 | ATP + biotin + apopropionyl-CoA carboxylase | biotinylation of mitochondrial PCC | Homo sapiens | AMP + diphosphate + holopropionyl-CoA carboxylase | - |
? | |
6.3.4.10 | ATP + biotin + apopyruvate carboxylase | - |
Homo sapiens | AMP + diphosphate + holopyruvate carboxylase | - |
? | |
6.3.4.10 | additional information | HCS catalyzes the ATP-dependent biotinylation of the four biotin-dependent carboxylases found in humans | Homo sapiens | ? | - |
? | |
6.3.4.15 | ATP + biotin + apo-[acetyl-CoA:carbon-dioxide ligase (ADP-forming)] | - |
Homo sapiens | AMP + diphosphate + [acetyl-CoA:carbon-dioxide ligase (ADP-forming)] | - |
? |