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Literature summary for 6.4.1.4 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

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

Natural Substrates/ Products (Substrates)

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

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Substrates and Products (Substrate)

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

Synonyms

Synonyms Comment Organism
3-methylcrotonyl-CoA carboxylase
-
Homo sapiens
MCC
-
Homo sapiens

Cofactor

Cofactor Comment Organism Structure
ATP
-
Homo sapiens
biotin dependent on Homo sapiens

General Information

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