Defects in biotin (Btn) metabolism

Proteins (6)

Protein	Synonyms	Taxonomy
Acetyl-CoA carboxylase 1	ACC1; EC 6.4.1.2; Acetyl-Coenzyme A carboxylase alpha; ACC-alpha	Homo sapiens (human)
Propionyl-CoA carboxylase alpha chain, mitochondrial	PCCase subunit alpha; EC 6.4.1.3; Propanoyl-CoA:carbon dioxide ligase subunit alpha	Homo sapiens (human)
Propionyl-CoA carboxylase beta chain, mitochondrial	PCCase subunit beta; EC 6.4.1.3; Propanoyl-CoA:carbon dioxide ligase subunit beta	Homo sapiens (human)
Pyruvate carboxylase, mitochondrial	EC 6.4.1.1; Pyruvic carboxylase; PCB	Homo sapiens (human)
Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial	MCCase subunit alpha; EC 6.4.1.4; 3-methylcrotonyl-CoA carboxylase 1; 3-methylcrotonyl-CoA carboxylase biotin-containing subunit; 3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha	Homo sapiens (human)
Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial	MCCase subunit beta; EC 6.4.1.4; 3-methylcrotonyl-CoA carboxylase 2; 3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit; 3-methylcrotonyl-CoA:carbon dioxide ligase subunit beta	Homo sapiens (human)

Compound	Description
biotin	A water-soluble, enzyme co-factor present in minute amounts in every living cell. It occurs mainly bound to proteins or polypeptides and is abundant in liver, kidney, pancreas, yeast, and milk.
Manganese	A trace element with atomic symbol Mn, atomic number 25, and atomic weight 54.94. It is concentrated in cell mitochondria, mostly in the pituitary gland, liver, pancreas, kidney, and bone, influences the synthesis of mucopolysaccharides, stimulates hepatic synthesis of cholesterol and fatty acids, and is a cofactor in many enzymes, including arginase and alkaline phosphatase in the liver. (From AMA Drug Evaluations Annual 1992, p2035)
adenosine triphosphate	An adenine nucleotide containing three phosphate groups esterified to the sugar moiety. In addition to its crucial roles in metabolism adenosine triphosphate is a neurotransmitter.
biocytin