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Literature summary extracted from

  • Horvath, T.; Stratton, S.; Bogusiewicz, A.; Owen, S.; Mock, D.; Moran, J.
    Quantitative measurement of urinary excretion of 3-hydroxyisovaleryl carnitine by LC-MS/MS as an indicator of biotin status in humans (2010), Anal. Chem., 82, 9543-9548.
    View publication on PubMedView publication on EuropePMC

Localization

EC Number Localization Comment Organism GeneOntology No. Textmining
6.4.1.4 mitochondrion
-
 Homo sapiens 5739
-

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
6.4.1.4 ATP + 3-methylcrotonoyl-CoA + HCO3- Homo sapiens
-
 ADP + phosphate + 3-methylglutaconyl-CoA
-
 ?

Organism

EC Number Organism UniProt Comment Textmining
6.4.1.4 Homo sapiens
-
-
-

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
6.4.1.4 ATP + 3-methylcrotonoyl-CoA + HCO3-
-
 Homo sapiens ADP + phosphate + 3-methylglutaconyl-CoA
-
 ?

Synonyms

EC Number Synonyms Comment Organism
6.4.1.4 3-methylcrotonyl-CoA carboxylase
-
 Homo sapiens
6.4.1.4 MCC
-
 Homo sapiens

Cofactor

EC Number Cofactor Comment Organism Structure
6.4.1.4 ATP
-
 Homo sapiens
6.4.1.4 biotin dependent on Homo sapiens

General Information

EC Number General Information Comment Organism
6.4.1.4 malfunction marginal biotin deficiency reduces the enzymatic activity of MCC, thereby causing impairment in leucine catabolism Homo sapiens