RT Book, Section A1 Goodman, Stephen I. A2 Rudolph, Colin D. A2 Rudolph, Abraham M. A2 Lister, George E. A2 First, Lewis R. A2 Gershon, Anne A. SR Print(0) ID 6725833 T1 Chapter 148. Biotin Responsive Disorders T2 Rudolph's Pediatrics, 22e YR 2011 FD 2011 PB The McGraw-Hill Companies PP New York, NY SN 978-0-07-149723-7 LK accesspediatrics.mhmedical.com/content.aspx?aid=6725833 RD 2024/03/28 AB Biotinidase releases biotin from the peptide form in which it usually exists in nature, and the free biotin is then available for attachment to apocarboxylases that act on propionyl-CoA, 3-methylcrotonyl-CoA, acetyl-CoA, and pyruvate by holocarboxylase synthetase. Deficiency of biotinidase or holocarboxylase synthetase produces multiple carboxylase deficiency; both conditions are characterized by the triad of alopecia, skin rash, and encephalopathy, and both are inherited as autosomal recessive traits.