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Literature summary extracted from

  • Mock, D.M.
    Marginal biotin deficiency is common in normal human pregnancy and is highly teratogenic in mice (2009), J. Nutr., 139, 154-157.
    View publication on PubMedView publication on EuropePMC

Activating Compound

EC Number Activating Compound Comment Organism Structure
6.4.1.4 biotin dependent on Mus musculus
6.4.1.4 biotin dependent on Homo sapiens

Metals/Ions

EC Number Metals/Ions Comment Organism Structure
6.4.1.4 Mg2+
-
 Mus musculus
6.4.1.4 Mg2+
-
 Homo sapiens

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- Mus musculus
-
 ADP + phosphate + 3-methylglutaconyl-CoA
-
 ?
6.4.1.4 ATP + 3-methylcrotonoyl-CoA + HCO3- Homo sapiens
-
 ADP + phosphate + 3-methylglutaconyl-CoA
-
 ?
6.4.1.4 additional information Homo sapiens induced marginal biotin deficiency increases urinary excretion of 3-hydroxyisovaleric acid caused by decreased activity of the biotin-dependent enzyme beta-methylcrotonyl-CoA carboxylase, and decreased activity of the biotin-dependent enzyme propionyl-CoA carboxylase in peripheral blood lymphocytes, overview. Relation of biotin deficiency during pregnancy to teratogenesis, biotin deficiency does cause human birth defects, overview ?
-
 ?
6.4.1.4 additional information Mus musculus induced marginal biotin deficiency increases urinary excretion of 3-hydroxyisovaleric acid caused by decreased activity of the biotin-dependent enzyme beta-methylcrotonyl-CoA carboxylase, and decreased activity of the biotin-dependent enzyme propionyl-CoA carboxylase in peripheral blood lymphocytes, overview. Relation of biotin deficiency during pregnancy to teratogenesis, in mice a marginal degree of biotin deficiency in the dam causes a severe degree of deficiency in the fetus, overview ?
-
 ?

Organism

EC Number Organism UniProt Comment Textmining
6.4.1.4 Homo sapiens
-
 pregnant women
-
6.4.1.4 Mus musculus
-
 pregnant mice
-

Source Tissue

EC Number Source Tissue Comment Organism Textmining
6.4.1.4 lymphocyte from peripheral blood Mus musculus
-
6.4.1.4 lymphocyte from peripheral blood 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-
-
 Mus musculus ADP + phosphate + 3-methylglutaconyl-CoA
-
 ?
6.4.1.4 ATP + 3-methylcrotonoyl-CoA + HCO3-
-
 Homo sapiens ADP + phosphate + 3-methylglutaconyl-CoA
-
 ?
6.4.1.4 additional information induced marginal biotin deficiency increases urinary excretion of 3-hydroxyisovaleric acid caused by decreased activity of the biotin-dependent enzyme beta-methylcrotonyl-CoA carboxylase, and decreased activity of the biotin-dependent enzyme propionyl-CoA carboxylase in peripheral blood lymphocytes, overview. Relation of biotin deficiency during pregnancy to teratogenesis, biotin deficiency does cause human birth defects, overview Homo sapiens ?
-
 ?
6.4.1.4 additional information induced marginal biotin deficiency increases urinary excretion of 3-hydroxyisovaleric acid caused by decreased activity of the biotin-dependent enzyme beta-methylcrotonyl-CoA carboxylase, and decreased activity of the biotin-dependent enzyme propionyl-CoA carboxylase in peripheral blood lymphocytes, overview. Relation of biotin deficiency during pregnancy to teratogenesis, in mice a marginal degree of biotin deficiency in the dam causes a severe degree of deficiency in the fetus, overview Mus musculus ?
-
 ?

Synonyms

EC Number Synonyms Comment Organism
6.4.1.4 beta-methylcrotonyl-CoA carboxylase
-
 Mus musculus
6.4.1.4 beta-methylcrotonyl-CoA carboxylase
-
 Homo sapiens

Cofactor

EC Number Cofactor Comment Organism Structure
6.4.1.4 ATP
-
 Mus musculus
6.4.1.4 ATP
-
 Homo sapiens