Impaired Biotinidase Activity Disrupts Holocarboxylase Synthetase Expression in Late Onset Multiple Carboxylase Deficiency

Biotinidase catalyzes the hydrolysis of the vitamin biotin from proteolytically degraded biotin-dependent carboxylases. This key reaction makes the biotin available for reutilization in the biotinylation of newly synthesized apocarboxylases. This latter reaction is catalyzed by holocarboxylase synth...

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Hauptverfasser: Pérez-Monjaras, Anylu, Cervantes-Roldán, Rafael, Meneses-Morales, Iván, Gravel, Roy A., Reyes-Carmona, Sandra, Solórzano-Vargas, Sergio, González-Noriega, Alfonso, León-Del-Río, Alfonso
Format: Artigo
Sprache:Inglês
Veröffentlicht: American Society for Biochemistry and Molecular Biology 2008
Schlagworte:
Metabolism and Bioenergetics
Online Zugang:https://ncbi.nlm.nih.gov/pmc/articles/PMC2662232/
https://ncbi.nlm.nih.gov/pubmed/18845537
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.M806985200
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