6.3.4.10 ATP + biotin + apo-[propanoyl-CoA:carbon-dioxide ligase (ADP-forming)] - 6.3.4.10 ATP + biotin + apo-[propanoyl-CoA:carbon-dioxide ligase (ADP-forming)] plays an essential role in biotin utilization 6.3.4.10 ATP + biotin + apo-[propanoyl-CoA:carbon-dioxide ligase (ADP-forming)] its deficiency causes biotin-responsive multiple carboxylase deficiency in humans 6.3.4.10 ATP + biotin + apo-[propionyl CoA carboxylase] - 6.3.4.10 ATP + biotin + apo-[propionyl-CoA carboxylase] - 6.3.4.10 ATP + biotin + apo-[propionyl-CoA:carbon-dioxide ligase (ADP-forming)] - 6.3.4.10 ATP + biotin + apocarboxylase - 6.3.4.10 ATP + biotin + histone H4 biotinylation at Lys12 especially in telomeric repeats, overview 6.3.4.10 additional information the enzyme is critical to utilization of exogenous or dietary biotin and responds to biotin deprivation by reduction in the steady state level of their mRNAs. The free biotin is directed to brain because the reduction in holocarboxylase synthetase, sodium-dependent multivitamin-transporter and carboxylase mRNAs in liver reduces their capacity to utilize biotin while leaving free biotin in circulation 6.3.4.10 additional information although mammals have multiple biotin-dependent enzymes there is only a single gene encoding holocarboxylase synthetase, HCS, responsible for all cellular biotinylation. The enzyme catalyzes the reactions of EC 6.3.4.10, EC 6.3.4.11, and EC 6.3.4.15, overview 6.3.4.10 additional information holocarboxylase synthetase catalyzes the attachment of biotin to the epsilon-amino group of a specific lysine residue in apocarboxylases, biotinylation of biotin-dependent carboxylases requires ATP and proceeds in the following two steps 6.3.4.10 additional information holocarboxylase synthetase, HCS, catalyzes the binding of biotin to lysines in carboxylases and histones in two steps. First, HCS catalyzes the synthesis of biotinyl-5'-AMP, and second, the biotinyl moiety is ligated to lysine residues, binding structure, modelling, overview 6.3.4.10 additional information the enzyme catalyzes the reactions of EC 6.3.4.10, EC 6.3.4.11, and EC 6.3.4.15, overview