<unk>  is a form of metabolic disorder involving failures of carboxylation enzymes. The deficiency can be in biotinidase or holocarboxylase synthetase. These conditions respond to biotin. Forms include: * Holocarboxylase synthetase deficiency - neonatal; * Biotinidase deficiency - late onset; If left untreated, the symptoms can include feeding problems, decreased body tone, generalized red rash with skin exfoliation and baldness, failure to thrive, seizure, coma, developmental delay, foul smelling urine, lactic acidosis, and high levels of ketones and ammonia in the blood.
Multiple carboxylase deficiency
3-Methylcrotonyl-CoA carboxylase deficiency
Glutaminase deficiency
Pyruvate carboxylase deficiency
2,4 Dienoyl-CoA reductase deficiency
Acyl-CoA oxidase deficiency
Pyruvate kinase deficiency
Malonyl-CoA decarboxylase deficiency